Cardiology Flashcards

Question

Signs of ACS

Answer 1

- Hypotension or hypertension - Reduced 4th heart sound - Signs of heart failure: e.g. increased JVP, oedema; red flag symptom - Systolic murmur: if mitral regurgitation or a ventricular septal defect develops

Answer 2

- Chest pain - Central, ‘heavy’, crushing pain - Radiation to the left arm or neck - Symptoms should continue at rest for more than 20 minutes - Certain patients e.g. diabetics or elderly, have atypical presentation and may not have chest pain (‘silent MI’) - May sometimes feel like indigestion - Shortness of breath - Sweating and clamminess - Nausea and vomiting - Palpitations - Anxiety: often described as a ‘sense of impending doom’

Answer 3

ECG: perform within 10 minutes. Aim to perform serial ECGs every 10 minutes to detect dynamic changes. - **Unstable angina:** non-specific changes - **NSTEMI:** ST-segment depression; T-wave inversion; pathological Q waves; a normal ECG may be seen - **STEMI:** ST-segment elevation; T-wave inversion; new left-bundle branch block Troponin: for a STEMI and NSTEMI, troponin levels will begin to elevate 4-6 hours after injury and will remain elevated for roughly 10 days. In unstable angina, there is no elevation in troponin.

Answer 4

- Perform other tests that you would for stable angina e.g. - Physical Examination (heart sounds, signs of heart failure, BMI) - Lipid profile - Thyroid function tests: check for hypo / hyper thyroid - HbA1C and fasting glucose: check for diabetes - Coronary angiogram: aim to carry out angiography within 90 minutes if required; diagnostic investigation of choice - FBC: Hb and haematocrit may reveal a secondary cause in type 2 MI e.g. anaemia - U&Es: electrolyte imbalances may predispose the patient to cardiac arrhythmias; also done prior to ACEi and other meds - LFTs: done prior to statins - Other biomarkers: less commonly used biomarkers of cardiomyocyte injury include creatine kinase-MB (increases at 3-6 hours), and myoglobin (earliest to rise, usually within 2 hours) - CXR: to exclude other potential causes, if needed - Echocardiogram after the event to assess the functional damage

Answer 5

- Oxygen: only if SpO2 is <94%, and aim for 94-98% - Analgesia: morphine and sublingual glyceryl trinitrate - Dual antiplatelets: - Aspirin - The choice of the second antiplatelet agent depends on if the person is having PCI or not, and will vary based on local guidance: - Prasugrel or ticagrelor or clopidogrel if undergoing PCI - Ticagrelor or clopidogrel if not undergoing PCI - Anticoagulation: - Fondaparinux: offer to all patients unless undergoing immediate coronary angiography - Unfractionated heparin: an alternative to fondaparinux if the patient has renal failure - Beta blockers: e.g. atenolol or metoprolol, unless contraindicated - Remember 'MONA’: Morphine, Oxygen, Nitrates, Aspirin

Answer 6

If the patient is clinically unstable they must be taken for PCI immediately. Otherwise, the remainder can be risk-stratified using the GRACE score (refer to 'other notes' below)

Answer 7

- Oxygen: only if SpO2 is <94%, and aim for 94-98% - Analgesia: morphine and sublingual glyceryl trinitrate - Dual antiplatelets: - Aspirin - The choice of the second antiplatelet agent depends on if the person is having PCI or not, and will vary based on local guidance: - Prasugrel or clopidogrel if undergoing PCI - Ticagrelor or clopidogrel if undergoing fibrinolysis

Answer 8

- PCI: first-line method of revascularisation; insertion of a catheter via the radial or femoral artery to open up the blocked vessels using an inflated balloon (angioplasty), and a stent may also be inserted - Anticoagulation and further antiplatelet therapy - Unfractionated heparin and a glycoprotein IIb/IIIa inhibitor - Bivalirudin may be used as an alternative to unfractionated heparin

Answer 9

- Thrombolysis e.g. alteplase or tenecteplase - IV administration of a fibrinolytic agent - Offered if symptom onset is greater than 12h OR PCI not available within 120 mins - Anticoagulation - An antithrombin agent such as unfractionated heparin is usually given alongside thrombolysis - ECG: if the ECG shows residual ST elevation after 60-90 minutes of thrombolysis, offer immediate angiography and PCI

Answer 10

- Lifestyle changes: exercise, diet change, smoking cessation, reducing alcohol intake - Manage cardiovascular risk factors: lipid, diabetes, hypertension management - Antiplatelet therapy: - Aspirin 75mg OD continued indefinitely - The second antiplatelet depends on the one chosen in the acute setting i.e. prasugrel, ticagrelor, or clopidogrel, and is usually continued for 12 months. - Angiotensin-converting enzyme inhibitors (ACEi) and beta-blocker - Statin: usually atorvastatin 80mg - Cardiac rehabilitation: must be offered following myocardial infarction

Answer 11

- Post-MI pericarditis: inflammation of the pericardium usually occurs a few days post-MI due to irritation of the pericardium; usually benign - Cardiac arrest/tachyarrhythmias: most commonly due to ventricular fibrillation - Can lead to sudden death - Bradyarrhythmias: heart block is more common after an inferior myocardial infarction - Cardiogenic shock: extensive ventricular damage may lead to impaired ejection fraction and the development of cardiogenic shock - Ventricular septal defect: seen within the first week and may present with acute heart failure and a pansystolic murmur - Mitral regurgitation: most commonly due to papillary muscle rupture secondary to an inferior-posterior infarction, results in an early-mid systolic murmur - Left ventricular wall rupture: a rare but fatal complication which presents within a few weeks. Ischaemia leads to a weakened ventricular wall and rupture, presenting as cardiac tamponade and acute heart failure

Answer 12

- Dressler’s syndrome: presents similarly to post-MI pericarditis but occurs 2-6 weeks post-MI, and reflects an autoimmune process against neo-antigens formed by the heart Diagnosis: ECG (global ST elevation and T wave inversion), echocardiogram (pericardial effusion) and raised inflammatory markers (CRP and ESR). Management: NSAIDs (aspirin / ibuprofen) and in more severe cases steroids (prednisolone). May need pericardiocentesis to remove fluid around the heart. - Heart failure: ventricular dysfunction following extensive damage can lead to chronic heart failure - Left ventricular aneurysm: bulge or ballooning of a weakened area of the heart

Answer 13

Unstable angina has a better outcome than an NSTEMI or STEMI. NSTEMIs and STEMIs have similar long-term outcomes. 5-10% of patients with ACS are predisposed to re-infarction. Poor prognostic factors include: increasing age, the magnitude of troponin rise, arrhythmias, left ventricular dysfunction, renal impairment, diabetes, anaemia, cerebrovascular disease

Answer 14

- Type 1: a classic MI and occurs due to atheromatous plaque rupture - Type 2: secondary to ischaemia due to either increased oxygen demand or decreased supply, such as vasospasm, anaemia and sepsis. Management involves treating the underlying cause. - Type 3: Sudden cardiac death or cardiac arrest suggestive of an ischaemic event - Type 4: MI associated with PCI / coronary stenting / CABG

Answer 15

Aspirin - Antiplatelet: predominantly COX-1 inhibition, thus preventing the synthesis of thromboxane A2 Clopidogrel, Prasugrel and Ticagrelor - Antiplatelets: inhibit the binding of ADP to its platelet P2Y12 receptor Tirofiban, Abciximab, eptifibatide - Antiplatelets: glycoprotein IIb/IIIa receptor antagonists Enoxaparin, Fondaparinux - Anticoagulants: activate antithrombin III, thus causing the inhibition of clotting factor Xa Bivalirudin - Anticoagulant: reversible direct thrombin inhibitor

Answer 16

The Global Registry of Acute Coronary Events (GRACE) score is recommended by NICE to risk-stratify patients with unstable angina and non-ST elevation myocardial infarction (NSTEMI). The GRACE score estimates admission to 6-month mortality. Variables: Age, HR, Pulse, Systolic BP, Creatinine, Cardiac arrest at admission, ST-segment deviation on ECG, Abnormal cardiac enzymes, Kilip class (Signs/symptoms) - Rales and/or jugular venous distension, Pul oed, CArdiogenic shock Interpretations: Intermediate- and high-risk patients with unstable angina or NSTEMI require coronary angiography during admission, whilst this can be delayed in low-risk patients. - Clinically unstable: immediate angiogram and percutaneous coronary intervention (PCI) - Intermediate or high risk of cardiovascular event (>3%): coronary angiography and percutaneous coronary intervention (PCI) within 72 hours of admission - Low risk of cardiovascular event (<3%): exclusively medical management

Answer 17

Cardiac failure describes when cardiac output cannot meet metabolic demands. Congestive cardiac failure describes a combination of left and right-sided ventricular failure.

Answer 18

- M>F - More prevalent with increasing age

Answer 19

- Previous myocardial infarction: the single greatest risk factor - Non-modifiable risk factors: male gender, increasing age - Cardiovascular risk factors: ischaemic heart disease, hypertension, hypercholesterolaemia, diabetes - Valvular heart disease - Renal failure: causes ‘high-output’ heart failure due to fluid overload - Atrial fibrillation

Answer 20

In heart failure, cardiac output struggles to meet the metabolic demands of the body. Heart failure can happen one of two ways: systolic failure or diastolic failure: - Systolic heart failure - Cardiac output = stroke volume x heart rate - The ejection fraction is not preserved: an ejection fraction of 40% or less would indicate systolic heart failure. - The low stroke volume is due to the ventricles not pumping enough blood out. Diastolic: - Cardiac output = stroke volume x heart rate - In this case, the stroke volume is low but the ejection fraction is preserved. The reason for the low stroke volume is due to reduced filling of the ventricle (reduced preload) Congestive cardiac failure describes a combination of left and right-sided ventricular failure (biventricular failure). Right-sided heart failure usually occurs as a result of left-sided heart failure. Blood starts backing up into the lungs causing pulmonary oedema and congestion. The pulmonary hypertension puts pressure on the right ventricle (cor pulmonale) and causes right-sided heart failure. The pulmonary congestion is responsible for the respiratory symptoms seen in heart failure.

Answer 21

- Ischaemic heart disease: as less blood and oxygen get to the myocardium, the myocytes start to die - Hypertension: as arterial pressure increases in the systemic circulation, it gets harder for the left ventricle to pump blood out into that hypertensive systemic circulation. - Left ventricular hypertrophy: increased muscle mass requires increased oxygen supply - making it more likely for that the muscle will die - Dilated cardiomyopathy: heart chambers dilate and thin out, leading to weaker contractions.

Answer 22

- Left ventricular hypertrophy: causes the ventricular chamber to decrease in size which means less blood can enter. - Restrictive cardiomyopathy: ventricle can't stretch enough to accommodate the blood - Valvular disease: e.g. aortic stenosis causes LVH or mitral regurgitation means blood doesn't enter the ventricles in the right amount as it leaks back into atria - Arrhythmias e.g. atrial fibrillation

Answer 23

- In a normal heart, increased ventricular filling results in increased contraction via the Frank-Starling law → increased cardiac output - In patients with heart failure, this mechanism fails - As the heart continues to fail → compensatory mechanisms are activated, including an increase in heart rate, catecholamine release and RAAS activation (due to decreased blood flow to kidneys) - These mechanisms are useful in the initial period but are usually overexpressed, thus instigating a vicious cycle. - Compensatory mechanisms are usually responsible for the fluid retention and fluid overload symptoms experienced by the patient

Answer 24

- Tachypnoea and tachycardia - Cool peripheries - Peripheral or central cyanosis - Displaced apex beat - Stony dull percussion: if an effusion is present - Crackles on auscultation: coarse bi-basal crackles due to pulmonary congestion - Third heart sound (S3)

Answer 25

- Dyspnoea: particularly exertional - Orthopnoea (SOB when lying flat) and paroxysmal nocturnal dyspnoea (SOB at night) - Fatigue and weakness - Cough with pink, frothy sputum - Cardiogenic wheeze

Answer 26

- Due to backing up of fluid: - Raised JVP - Peripheral pitting oedema - Hepatosplenomegaly - Ascites

Answer 27

- Fatigue and weakness - Due to backing up of fluid - Swelling in the legs - Distended abdomen

Answer 28

Class I: no limitation on physical activity, ordinary activity doesn't cause fatigue, palpitations or dyspnoea Class II: slight limitation on physical activity, comfortable at rest but physical activity causes symptoms Class III: Marked limitation of physical activity, comfy at rest but not physical activity Class IV: Cant carry out physical activity without discomfort, symptoms of cardiac insufficiency at rest

Answer 29

- NT-proBNP: increased in chronic heart failure - ECG: broad QRS complexes; evidence of left ventricular hypertrophy - CXR: - A -Alveolar oedema (batwing opacities) - B - Kerley B lines - C - Cardiomegaly - D - Dilated upper lobe vessels - E - Pleural effusion - Transthoracic echocardiogram: determine left ventricular ejection fraction (LVEF), diastolic function, and valvular abnormalities

Answer 30

- FBC: anaemia may be a cause of ‘high-output’ heart failure - U&Es: to investigate for renal failure as an underlying cause of heart failure; also U&Es monitored as ACEi's and aldosterone antagonists can cause electrolyte abnormalities - Blood lipids and fasting blood glucose: screen for hypercholesterolaemia and evidence of diabetes

Answer 31

- Beta-blocker e.g. bisoprolol and ACE inhibitor e.g. ramipril: start one drug at a time. - Beta-blockers and ACE inhibitors shown to improve mortality but not in heart failure with preserved ejection fraction - If ACE inhibitor is not tolerated: angiotensin receptor blocker (e.g losartan) or hydralazine with nitrate (particularly in Afro-Caribbean patients)

Answer 32

Aldosterone antagonist (e.g. spironolactone) if symptoms not controlled with 1st line management

Answer 33

- Cardiac resynchronisation therapy (CRT): involves biventricular pacing and forces both ventricles to contract in synchrony, thereby improving cardiac output OR - Implantable cardioverter-defibrillator (ICD): able to perform cardioversion, defibrillation and, in some cases, pacing - Digoxin: an alternative option, particularly for patients with AF and heart failure due to its inotropic effects. - Ivabradine: an alternative option if HR >75 bpm and LVEF <35%; slows the heart rate so the heart can pump more blood through the body each time it beats.

Answer 34

- Smoking cessation, diet changes, exercise - Fluid restriction: usually limited to <1.5L/day - Loop diuretic (e.g. furosemide): symptomatic relief of fluid overload - Annual influenza vaccine and one-off pneumococcal vaccine - Sacubitril-valsartan (Entresto): consider if the patient is symptomatic on an ACE inhibitor or ARB AND has a reduced LVEF; works by relaxing blood vessels so that blood can flow more easily, making it easier for heart to pump blood. - Surgical replacement of valve if valvular disease - Cardiac transplantation: considered for patients with severe refractory symptoms or refractory cardiogenic shock

Answer 35

- Pleural effusion: heart failure causes an elevated pulmonary capillary pressure, usually resulting in bilateral transudative pleural effusions - Acute decompensation of chronic heart failure: patients usually present with acute respiratory distress due to significant pulmonary oedema - Arrhythmias - Acute renal failure: reduced cardiac output and drug overuse (ACE inhibitors, aldosterone antagonists, diuretics) results in poor renal perfusion

Answer 36

In general, the survival of patients with end-stage heart failure is poor. For example, 65% of patients in NYHA class IV are alive at 17-month follow-up.

Answer 37

Cardiac output is not able to meet metabolic demands. This can be new-onset or as an acute decompensation of chronic heart failure.

Answer 38

- In the UK, heart failure is responsible for over 67,000 hospital admissions per year - >65 years of age

Answer 39

Increasing age Coronary artery disease Hypertension Valvular disease Diabetes A Fib Renal insufficiency

Answer 40

Acute decompensated heart failure can occur as either new-onset heart failure without any previous cardiac dysfunction or as an acute decompensation of chronic heart failure. In heart failure, cardiac output is not able to meet the metabolic demands of the body. Common causes of heart failure include coronary artery disease and hypertension. **General pathophysiology:** - In response to reduced cardiac output, the sympathetic nervous system is activated - This results in tachycardia, increased myocardial contractility, peripheral vasoconstriction, and RAAS activation, causing salt and water retention - Patients with heart failure are generally hypervolemic → brain natriuretic peptide (BNP) release by ventricular myocytes in response to stretch - These processes lead to pulmonary and/or venous congestion - Pulmonary oedema presents with shortness of breath, whilst venous congestion causes peripheral oedema

Answer 41

- Cool peripheries - Signs of congestive heart failure: peripheral, pitting oedema and raised JVP - Displaced apex beat - Hypotension - Crackles on auscultation: left-sided failure; usually coarse bi-basal crackles - Third heart sound (S3) - Stony dull percussion: if an effusion is present

Answer 42

- Dyspnoea: due to pulmonary oedema - Often a history of orthopnea and paroxysmal nocturnal dyspnoea - Fatigue and weakness - Cardiogenic wheeze - Symptoms of congestive heart failure: swelling of the peripheries and ascites

Answer 43

- FBC: anaemia can be an underlying cause of heart failure - U&Es: to investigate renal failure as an underlying cause of heart failure. Renal function should be monitored because loop diuretics such as furosemide are nephrotoxic. - Arterial blood gas: demonstrates type 1 respiratory failure; degree of acidosis helps to determine which patients may require non-invasive ventilation - BNP or NT-proBNP: BNP <100 pg/ml or NT‑proBNP <300 pg/ml suggest an alternative diagnosis - ECG: may be AF; left ventricular hypertrophy - CXR: pulmonary congestion features include: - A - Alveolar oedema (batwing opacities) - B - Kerley B lines - C - Cardiomegaly - D - Dilated upper lobe vessels - E - Pleural Effusion - Transthoracic echocardiogram: assess for systolic and diastolic function, ejection fraction and valvular disease (NICE defines a reduced left ventricular ejection fraction as < 40%)

Answer 44

- Stabilise the patient: administer oxygen to maintain a SpO2≥94% - Fluid restriction: fluid intake is usually limited to <1.5L/day - IV diuretic: usually a loop diuretic e.g. furosemide to relieve fluid overload - Inotropes or vasopressors e.g. dobutamine: only offer to patients with heart failure and cardiogenic shock (i.e. haemodynamically unstable) - Non-invasive ventilation (NIV): consider NIV if the patient does not stabilise with initial medical management - Continuous positive airway pressure (CPAP) - Intubation and ventilation: if CPAP is unsuccessful

Answer 45

- If acute heart failure is due to aortic stenosis: offer surgical aortic valve replacement - Mechanical assist device: pump that can temporarily help the pumping action of the heart

Answer 46

- ACE-inhibitor e.g. ramipril and a cardioselective β-blocker e.g. bisoprolol - Improved prognosis by slowing, or even reversing, ventricular remodelling - Fluid restriction: fluid intake is usually limited to <1.5L/day - Loop diuretic (e.g. furosemide) for symptomatic relief of oedema

Answer 47

Arrhythmias: can both precipitate acute heart failure and occur as a result of it. Atrial fibrillation is one of the most common arrhythmias associated with heart failure.

Answer 48

Mortality for acute heart failure ranges from 2-20%. Poor prognostic factors include old age, hypotension, male sex, ischaemic congestive heart failure, renal dysfunction, previous chronic heart failure, a respiratory rate on admission > 30 and an elevated BNP.

Answer 49

Hypertension refers to a persistent elevation of arterial blood pressure. Hypertension is defined as a blood pressure reading of ≥140/90 mmHg (ambulatory blood pressure monitoring ≥135/85 mmHg) and is categorised into primary or secondary hypertension, depending on whether a cause can be identified. Normal blood pressure = 120/80 mmHg

Answer 50

- Hypertension is responsible for 7.5 million deaths annually and is a major contributor to ischaemic heart disease - M>F - Prevalence is in those older than 35

Answer 51

- Non-modifiable risk factors: increasing age, African heritage, family history - Modifiable risk factors: obesity, sedentary lifestyle, alcohol excess, smoking, high sodium intake (>1.5g/day), stress

Answer 52

Any changes in blood pressure, and consequently the development of hypertension, occur due to alterations in cardiac output and peripheral resistance. - Primary (essential) hypertension: has no known underlying cause and is responsible for 90-95% of cases of hypertension. Some contributing factors include: - Genetic susceptibility - Excessive sympathetic nervous system activity - Abnormalities of Na+/K+ membrane transport - High salt intake - Abnormalities in renin-angiotensin-aldosterone system - Secondary hypertension indicates a known underlying cause. Examples include: - Renal disease: glomerulonephritis, polycystic kidney disease, renal artery stenosis, chronic kidney disease - Endocrine disorders: primary hyperaldosteronism, phaeochromocytoma, Cushing’s syndrome, hyperthyroidism, acromegaly - Medication: glucocorticoids, ciclosporin, atypical antipsychotics, the combined oral contraceptive pill - Pregnancy (pre-eclampsia)

Answer 53

Stage 1: 140/90 or above Stage 2: 160/100 or above Stage 3: 180/120 or above - White-coat effect: a discrepancy of ≥20/10mmHg between the clinic reading and average daytime ABPM reading suggests ‘white-coat’ hypertension - Malignant (accelerated) hypertension: a severe increase in blood pressure ≥180/120 mmHg with signs of retinal haemorrhage and/or papilloedema, associated with target organ damage. These patients require emergency assessment!

Answer 54

- Malignant (accelerated) hypertension (≥180/120 mmHg): - Hypertensive retinopathy - Visual disturbance - Cardiac symptoms e.g. chest pain - Oliguria or polyuria - Secondary hypertension: signs of the underlying cause e.g. hyperthyroidism causes weight loss, sweating and palpitations

Answer 55

- Asymptomatic: most common presentation - Headaches: classically occipital and worse in the morning - Dizziness, Blurred vision

Answer 56

- Blood-pressure reading: record the blood pressure on both arms - If the clinic blood pressure is ≥140/90mmHg, take a second reading. Record the lower of the 2 measurements as the clinic blood pressure - If the difference in blood pressure between arms is > 20mmHg, repeat the measurements. If readings remain > 20 mmHg, then subsequent measurements should be from the arm with the higher reading

Answer 57

- Offer to all patients with a clinic blood pressure between 140/90 mmHg and 180/120 mmHg to confirm the diagnosis - Blood pressure is measured over a 24 hour period, with at least 2 measurements per hour during waking hours - An overall of at least 14 measurements are required

Answer 58

- Offered if ABPM is not appropriate; HPBM involves the patient checking their blood pressure manually throughout the day - With the patient seated for each reading, two consecutive measurements are required at least 1 minute apart - Blood pressure should be measured twice daily, usually in the morning and evening, and ideally recorded for 7 days but at least 4 days - Readings on the first day are discarded and the average of the remaining readings are used

Answer 59

- Fundoscopy: assess for hypertensive retinopathy - 12-lead ECG: assess for ischaemic changes and evidence of left ventricular hypertrophy - Albumin:creatinine ratio (ACR) and urinalysis: assessing for renal dysfunction, as evidenced by elevated ACR and proteinuria or haematuria on urinalysis - Bloods: HbA1c, U&Es, total cholesterol and HDL cholesterol - It is also important to formally estimate cardiovascular risk using a tool such as QRisk to discuss prognosis and healthcare options.

Answer 60

- Fundoscopy: assess for hypertensive retinopathy - 12-lead ECG: assess for ischaemic changes and evidence of left ventricular hypertrophy - Albumin:creatinine ratio (ACR) and urinalysis: assessing for renal dysfunction, as evidenced by elevated ACR and proteinuria or haematuria on urinalysis - Bloods: HbA1c, U&Es, total cholesterol and HDL cholesterol - It is also important to formally estimate cardiovascular risk using a tool such as QRisk to discuss prognosis and healthcare options.

Answer 61

Hypertension is confirmed when the following criteria are met: - Clinic blood pressure of 140/90 mmHg or higher and - ABPM daytime average or HBPM average of 135/85 mmHg or higher

Answer 62

- <80 years: <140 systolic; <90 diastolic - >80 years: <150 systolic; <90 diastolic

Answer 63

First line: prevention and lifestyle change (e.g. diet, exercise and weight loss) Second line: ACE-i

Answer 64

Triple therapy: combine ACEi (or ARB) with CCB (amlodipine) and thiazide-like diuretic (indapamide)

Answer 65

Quadruple therapy: dependant on potassium levels. If hypertension is not controlled with 4 drugs, then consider a specialist review. - If K+ >4.5, add an alpha- or beta-blocker - If K+ ≤4.5, add an aldosterone antagonist such as spironolactone (a ‘K+ sparing diuretic’)

Answer 66

- Direct renin inhibitors e.g. aliskiren: a new option - Block the conversion of angiotensinogen to angiotensin I - Generally only considered in patients intolerant to normal antihypertensives

Answer 67

NICE recommend measuring blood pressure every 5 years to screen for hypertension. It should be measured more often in patients that are on the borderline for diagnosis (140/90) and every year in patients with type 2 diabetes.

Answer 68

- Coronary artery disease: for every 20/10 mmHg increase in blood pressure, there is a doubling of mortality related to ischaemic heart disease - Cerebrovascular accident: linear association between increased blood pressure and risk of developing a cerebrovascular accident - Congestive heart failure: hypertensive patients are 3 times more likely to develop congestive heart failure - Chronic kidney disease: increased levels of blood pressure are associated with the development of renal disease - Hypertensive retinopathy

Answer 69

Maintaining a tight control on blood pressure reduces the risk of developing end-organ damage, as well as cardiovascular and cerebrovascular disease, thus reducing the overall morbidity and mortality rate associated with hypertension.

Answer 70

Arrhythmias are abnormal heart rhythms. They result from an interruption to the normal electrical signals that coordinate the contraction of the heart muscle.

Answer 71

These are the four possible rhythms that you will see in a pulseless unresponsive patient. Shockable rhythms: - Ventricular tachycardia - Ventricular fibrillation Non-shockable rhythms: - Pulseless electrical activity (all electrical activity except VF/VT, including sinus rhythm without a pulse) - Asystole (no significant electrical activity)

Answer 72

Atrial fibrillation (AF) is a chaotic irregular atrial arrhythmia and is considered a type of supraventricular tachycardia (SVT)

Answer 73

- Atrial fibrillation is the most common cardiac arrhythmia and is estimated to affect approximately 2.5% of the general population - More common with increasing age - M>F

Answer 74

- Increasing age: AF affects approximately 5% of patients aged 70-75 years, and 10% of patients aged 80-85 years - Diabetes mellitus - Hyperthyroidism - Hypertension - Congestive heart failure - Valvular heart disease - Coronary artery disease - Dietary and lifestyle factors: excessive caffeine intake, alcohol abuse, obesity, smoking, medication use (e.g. thyroxine or beta-agonists)

Answer 75

Normally, the sinoatrial node produces organised electrical activity that coordinates the contraction of the atria of the heart. Atrial fibrillation is where the contraction of the atria is uncoordinated, rapid and irregular. This is due to disorganised electrical activity that overrides the normal, organised activity from the sinoatrial node. This disorganised electrical activity in the atria also leads to irregular conduction of electrical impulses to the ventricles. This results in: - Irregularly irregular ventricular contractions - Tachycardia - Heart failure due to poor filling of the ventricles during diastole - Risk of stroke

Answer 76

- First episode - Paroxysmal: recurrent episodes that stop on their own in less than 7 days - Persistent: recurrent episodes that last more than 7 days - Permanent: continuous atrial fibrillation that is also refractory to treatment. Management is aimed at rate control and anticoagulation, if appropriate.

Answer 77

PIRATES P - Pulmonary Embolism or COPD I - Ischaemic Heart Disease R - Rheumatic heart disease A - Anaemia, Age and Alcohol T - Thyroid disease E - Electrolyte disturbance S - Sepsis and Sleep apnoea

Answer 78

- Irregular irregular pulse - Hypotension: red flag; suggest haemodynamic instability - Evidence of heart failure: red flag; such as pulmonary oedema

Answer 79

- Palpitations - Dyspnoea - Chest pain: red flag - Syncope: red flag

Answer 80

The presence of adverse features guides the decision to undergo DC cardioversion. - Shock: hypotension (systolic blood pressure <90 mm Hg), pallor, sweating, cold, clammy extremities, confusion or impaired consciousness - Syncope: transient loss of consciousness - Myocardial ischaemia: typical ischaemic chest pain and/or evidence of myocardial ischaemia on 12-lead ECG - Heart failure: pulmonary oedema and/or raised jugular venous pressure

Answer 81

- ECG: irregularly irregular QRS complexes with absent P waves and chaotic baseline - 24-hour ambulatory ECG monitoring is recommended for those with paroxysmal AF in the community - Serum electrolytes: in addition to standard electrolytes, magnesium, calcium and phosphate should also be assessed - TFTs: hyperthyroidism is a secondary cause of AF

Answer 82

- Cardiac biomarkers: request troponin if chest pain is present as this may reflect an MI - Chest x-ray: if there is suspicion of heart failure to assess for pulmonary oedema - Transthoracic ECHO: consider if there is a suspicion of underlying structural or functional heart disease; usually performed prior to cardioversion in chronic cases

Answer 83

- Determine if rate control or rhythm control is more appropriate: - Rate control accepts the fact that the patient is not in sinus rhythm, but the aim is to get the heart rate below 100 to extend the time during diastole when the ventricles can fill with blood. - Rhythm control aims to restore normal sinus rhythm and is known as ‘cardioversion’, can either be electrical or pharmacological

Answer 84

Emergency electrical synchronised DC cardioversion

Answer 85

- Onset of AF < 48 hours: 1) rate control or 2) rhythm-control - Onset of AF > 48 hours / unknown onset: offer rate-control and anticoagulation for at least 3 weeks, then offer rhythm control if appropriate e.g. if rate control is unsuccessful or the patient remains symptomatic

Answer 86

- First line: beta-blocker (e.g. bisoprolol) or a rate-limiting calcium-channel blocker (e.g. verapamil) - Digoxin: may be considered first-line in patients with AF and heart failure - Second line: if refractory then consider combination therapy

Answer 87

- Pharmacological: - Flecainide or amiodarone: if no evidence of structural/ischaemic heart disease - Amiodarone: if structural/ischaemic heart disease is present - Electrical cardioversion: rapidly shock the heart back into sinus rhythm

Answer 88

Left atrial ablation: the pulmonary veins supply the premature depolarisations that trigger AF; radiofrequency energy is delivered in this area

Answer 89

Required if: - Rhythm control is appropriate - Rate-control treatment fails to control the symptoms of AF - The person is found to have valvular disease or left ventricular systolic dysfunction on echocardiography - Wolff–Parkinson–White syndrome or a prolonged QT interval is suggested by electrocardiogram

Answer 90

Anticoagulation: to reduce risk of thromboembolism

Answer 91

- Stroke: blood can pool within the atria, increasing the risk of thromboemboli and subsequent ischaemic stroke - Myocardial infarction: sustained. tachycardia in patients with coronary artery disease can result in acute myocardial infarction - Heart failure: sustained tachycardia in patients with other cardiac co-morbidities can result in myocardial ischaemia and a reduced ejection fraction - Reduced quality of life

Answer 92

The prognosis is ultimately determined by the presence of an underlying condition that may be potentially reversible, such as sepsis or hyperthyroidism. Overall, AF is associated with an increased mortality rate since it acts as an independent risk factor for stroke and myocardial infarction. Review any person with an established AF diagnosis at least annually.

Answer 93

CHA2DS2-VASc used to calculate stroke risk when considering anticoagulation C - Congestive heart failure H - Hypertension A2 - Age 65-74 or 75+ D - Diabetes S2 - Stroke/TIA/thromboembolism history V - Vascular disease S - Female sex If score 2 then give blood thinner

Answer 94

HAS-BLED estimates the risk of major bleeding for patients on anticoagulation to assess risk-benefit in AF care H - Hypertension A - Abnormal renal or liver function S - Stroke history B - Bleeding history L - Labile INR E - Elderly D - Drugs NSAID's e.g. clopidogrel, aspirin, NSAID Above 4 is high risk

Answer 95

Atrial flutter is usually an organised atrial rhythm with an atrial rate typically between 250-350bpm

Answer 96

- Much less common than AF - M>F - Prevalence increases with age

Answer 97

- Idiopathic (30%) - Coronary heart disease - Obesity - Hypertension - Cardiomyopathy - Heart failure - Thyrotoxicosis - COPD - Pericarditis - Acute excess alcohol intoxication

Answer 98

Normally the electrical signal passes through the atria once, simulating a contraction then disappears through the AV node into the ventricles. Atrial flutter is caused by a “re-entrant rhythm” in either atrium. This is where the electrical signal re-circulates in a self-perpetuating loop due to an extra electrical pathway. The signal goes round and round the atrium without interruption. This stimulates atrial contraction at 300 bpm. The signal makes its way into the ventricles every second lap due to the long refractory period to the AV node, causing 150 bpm ventricular contraction. Can be paroxysmal or persistent.

Answer 99

- Palpitations - Breathlessness - Chest pain - Dizziness - Syncope - Fatigue

Answer 100

ECG: regular sawtooth-like atrial flutter waves (F waves) with P-wave after P-wave

Answer 101

- Treat the reversible underlying condition (e.g. hypertension or thyrotoxicosis) - Rate/rhythm control with beta blockers or cardioversion - Radiofrequency ablation of the re-entrant rhythm - Anticoagulation based on CHA2DS2VASc score

Answer 102

Bradycardia is defined as a heart rate below 50 beats per minute, which can either be physiological or due to sinus node or atrioventricular (AV) node conduction dysfunction.

Answer 103

The incidence of AV conduction abnormalities increases with age, corresponding with the age-related incidence of ischaemic heart disease.

Answer 104

- Increasing age (> 70 years old): increases the risk of sinoatrial and AV node dysfunction - Endocrine: hypothyroidism - Infections: e.g. typhoid and diptheria - Electrolytes: hyperkalaemia, hypokalaemia, hypercalcaemia, or hypocalcaemia - Cardiac: recent MI - Drugs: e.g. beta-blockers, non-dihydropyridine calcium channel blockers, digoxin, adenosine and amiodarone - Surgery: intra-operative sinus bradycardia is relatively common - Hypothermia

Answer 105

Sinus node dysfunction may be associated with sinus bradycardia, tachycardia-bradycardia, and sick sinus syndrome. Atrioventricular (AV) conduction disturbances can either be due to poor transmission of atrial depolarisation to the ventricles or a delay in atrial depolarisation. Types of AV blocks: First-degree AV block: occurs where there is delayed atrioventricular conduction through the AV node but every atrial impulse leads to a ventricular contraction. Second-degree AV block (failure of conduction from atria to ventricles): - Mobitz type I / Wenckebach: atrial inputs becomes gradually weaker until it does not pass through the AV node. After failing to stimulate a ventricular contraction the atrial impulse returns to being strong. - Mobitz type II: Usually due to disease of the His-Purkinje system which causes intermitted failure or interruption of AV conduction. This results in missing QRS complexes. There is usually a set ratio of P waves to QRS complexes. Third-degree AV block: complete heart block. This is no observable relationship between P waves and QRS complexes.

Answer 106

- Associated with Cushing’s triad: raised intracranial pressure - Bradycardia - Hypertension - Irregular respirations (apnoea) - JVP: cannon A waves - Occurs in complete heart block due to atrial contraction against a closed tricuspid valve

Answer 107

- Dizziness - Fatigue - Shortness of breath - Syncope

Answer 108

ECG - First-degree AV block: PR interval >0.2s - Mobitz type I / Wenckebach: increasing PR interval culminating in a dropped QRS complex. The PR interval resets and the cycle repeats. - Mobitz type II: the PR interval remains constant, but with intermittent dropped QRS complexes (2:1, 3:1, etc). - Third-degree AV block: P wave and QRS complex are completely dissociated

Answer 109

- TFTs: hypothyroidism may be responsible for the bradycardia - U&Es and metabolic panel: screen for hyperkalaemia, hypokalaemia, hypercalcaemia, or hypocalcaemia - Serum digoxin level: appropriate for patients on digoxin - Holter monitoring: allows the correlation of symptoms with episodes of bradycardia - Tilt-table testing: to assess for neurocardiogenic syncope, whereby head-upright tilting causes a sudden drop in blood pressure followed by bradycardia - ECHO: useful if a permanent pacemaker is being implanted in order to assess left ventricular ejection fraction (LVEF) - LVEF <30% would warrant a pacemaker and an implantable cardioverter-defibrillator

Answer 110

- If stable: observe - If unstable or risk of asystole - First line: atropine 500mcg IV - If no improvement: - Atropine 500mcg IV repeated - Other inotropes (such as noradrenalin) - Transcutaneous cardiac pacing (using a defibrillator) - In patients with high risk of asystole - Temporary transvenous cardiac pacing using an electrode on the end of a wire that is inserted into a vein and fed through the venous system to the right atrium or ventricle to stimulate them directly - Permanent implantable pacemaker when available

Answer 111

- Syncope - Arrhythmias: some patients may go on to develop asystole, ventricular tachycardia or ventricular fibrillation - Congestive heart failure: due to poor cardiac output

Answer 112

Bradycardia treated with a pacemaker will typically have a good prognosis.

Answer 113

- Right bundle branch block (RBBB): - Causes: pulmonary embolism, cor pulmonale, ischaemic heart disease, atrial/ ventricular septal defect - Right bundle no longer conducts, meaning that the two ventricles do not get impulses at the same time. The impulse therefore spreads from left to right, which produces late activation of right ventricle.

Answer 114

- Causes: ischaemic heart disease, hypertension, cardiomyopathy, idiopathic fibrosis - Results in late activation of left ventricle - As the left bundle branch conduction is normally responsible for the initial ventricular activation, left bundle branch block also produce abnormal Q waves - Bifascicular block: blockage of right bundle branch and left anterior fascicle - Complete block: failure of all bundle branches

Answer 115

- Usually asymptomatic - RBBB: wide physiological splitting of second heart sound - LBBB: reverse splitting of the second heart sound

Answer 116

- RBBB: - Wide, slurred S wave in V6 and as a tall late R wave in V1 - MarroW: QRS looks like an M in V1, QRS looks like a W in V5 and V6 - LBBB: - Deep S wave in V1 and a tall late R wave in V6 - WilliaM: QRS looks like a W in V1 and V2, QRS looks like an M in V4-V6

Answer 117

Under normal circumstances at rest, our heart should be in sinus rhythm with an accompanying rate of 60-100 bpm. If the defining features of normal sinus rhythm are met but the heart rate is fast (> 100 bpm), we call it sinus tachycardia.

Answer 118

- Anxiety - Exercise - Pain - Anaemia - Haemorrhage - Thyrotoxicosis - Heart failure - Pulmonary embolism

Answer 119

ECG: one P wave per QRS, constant PR interval

Answer 120

- Treat underlying cause - Beta blockers may be used

Answer 121

Supraventricular tachycardia (SVT) is caused by the electrical signal re-entering the atria from the ventricles

Answer 122

- The incidence of paroxysmal SVT is 1-3 cases per 1000 persons, with a prevalence of 0.2% - Elderly females are the most commonly affected group

Answer 123

- Increasing age: five times more common in elderly patients - Female gender: two times more common in females - Hyperthyroidism - Smoking - Excessive caffeine or alcohol consumption - Stress: physical or emotional stress - Medication: e.g. salbutamol, atropine, decongestants (e.g. pseudoephedrine) - Recreational drug use: cocaine and methamphetamines

Answer 124

The four main types of SVT are atrial fibrillation, paroxysmal SVT, atrial flutter, and Wolff–Parkinson–White (WPW) syndrome. Normally the electrical signal in the heart can only go from the atria to the ventricles. In SVT the electrical signal finds a way from the ventricles back into the atria. Once the signal is back in the atria it travels back through the AV node and causes another ventricular contraction. This causes a self-perpetuating electrical loop without an end point and results in fast narrow complex tachycardia (QRS < 0.12). Paroxysmal SVT describes a situation where SVT reoccurs and remits in the same patient over time. Types: - “Atrioventricular nodal re-entrant tachycardia” is when the re-entry point is back through the AV node. - “Atrioventricular re-entrant tachycardia” is when the re-entry point is an accessory pathway (Wolff-Parkinson-White syndrome). - “Atrial tachycardia” is where the electrical signal originates in the atria somewhere other than the sinoatrial node. This is not caused by a signal re-entering from the ventricles but instead from abnormally generated electrical activity in the atria. This ectopic electrical activity causes an atrial rate of >100bpm. SVTs are often seen in patients with no pre-existing heart disease. It is usually benign and is rarely seen in the peri-arrest setting, making most SVTs ‘unpleasant’, rather than ‘life-threatening’.

Answer 125

- Tachycardia and tachypnoea - Adverse signs: - Hypotension - Pallor - Cold and clammy - Signs of pulmonary oedema - Raised JVP

Answer 126

- Patients may be asymptomatic - Palpitations - Shortness of breath - Chest pain - Dizziness - Adverse symptoms: - Ischaemic chest pain - Syncope - Confusion - Sweating

Answer 127

- Shock: hypotension (systolic blood pressure <90 mm Hg), pallor, sweating, cold, clammy extremities, confusion or impaired consciousness - Syncope: transient loss of consciousness due to a global reduction in blood flow to the brain - Myocardial ischaemia: typical ischaemic chest pain and/or evidence of myocardial ischaemia on 12-lead ECG - Heart failure: pulmonary oedema and/or raised jugular venous pressure

Answer 128

12-lead ECG: regular, narrow-complex tachycardia (QRS <0.12) with a rate of 151 to 250 beats per minute - it looks like a QRS complex followed immediately by a T wave, QRS complex, T wave etc

Answer 129

- TFTs: hyperthyroidism is a secondary cause of SVT - U&Es and metabolic panel: particularly screen for hyperkalaemia and hypokalaemia, as well as hyper- and hypocalcaemia

Answer 130

- Continuous ECG monitoring - Correct reversible causes e.g. electrolyte abnormalities - Stepwise approach: - Valsalva manoeuvre: ask the patient to blow hard against resistance, for example into a plastic syringe. - Carotid sinus massage: massage the carotid on one side gently with two fingers. - Adenosine Works by slowing cardiac conduction primarily though the AV node. It interrupts the AV node / accessory pathway during SVT and “resets” it back to sinus rhythm. It needs to be given as a rapid bolus to ensure it reaches the heart with enough impact to interrupt the pathway. - Verapamil (calcium channel blocker): alternative to adenosine - Direct current cardioversion: may be required if the above treatment fails

Answer 131

If recurrent episodes of SVT: - Medication (beta blockers, calcium channel blockers or amiodarone) - Radiofrequency ablation

Answer 132

- Syncope - Congestive heart failure: chronic, recurrent SVT that is left untreated may weaken the heart and result in heart failure - Life-threatening arrhythmias: rarely, SVT may degenerate into a life-threatening arrhythmia, such as ventricular tachycardia or ventricular fibrillation - Sudden death: occurs very rarely and the risk is generally very low

Answer 133

The prognosis associated with SVT depends on the presence of any underlying structural heart disease. Patients with a structurally normal heart have an excellent prognosis, whilst the risk of sudden death is generally very small in the absence of pre-excitation (WPW syndrome). Recurrent paroxysmal SVT requires lifestyle modifications, including reducing stress and caffeine intake, as well as smoking cessation.

Answer 134

Rapid ventricular beating that may result in inadequate ventricular filling

Answer 135

Ventricular tachycardia (VT) occurs due to rapid, recurrent ventricular depolarisation from a focus within the ventricles. This is commonly due to scarring of the ventricles following myocardial infarction. VT is a life-threatening arrhythmia that may lead to loss of consciousness, loss of cardiac output and ultimately cardiac arrest. Polymorphic VT: Polymorphic VT is a particular type of VT that is due to depolarisation of multiple foci within the ventricles leading to variable QRS complexes. It is usually secondary to myocardial ischaemia. Torsades de pointes is a subtype of polymorphic VT that is characterised by ventricular tachycardia that ‘twists’ around the isoelectric line. This subtype occurs secondary to a prolonged QT interval.

Answer 136

- Breathlessness - Chest pain - Palpitations - Dizziness/ syncope - Hypotension - Cardiac arrest

Answer 137

ECG: rapid, broad-complex tachycardia (QRS >120 ms)

Answer 138

- Consider up to 3 synchronised shocks - IV amiodarone infusion - Beta blockers: for management of symptoms

Answer 139

This involves very rapid irregular ventricular activation with no mechanical effect i.e. no cardiac output. Ventricular fibrillation (VF) occurs when the ventricular muscle fibres contract independently.

Answer 140

Cardiac arrest: - Pulselessness - Unconsciousness - Respiration ceases

Answer 141

Patient is likely to be unconscious! - ECG: no QRS can be identified, ECG is completely disorganised

Answer 142

- Immediate DC cardioversion

Answer 143

Ventricular ectopics are premature ventricular beats

Answer 144

- They are relatively common at all ages and in healthy patients however they are more common in patients with pre-existing heart conditions, especially post-MI

Answer 145

Premature ventricular beat caused by electrical discharges from outside the atria. Following a premature beat, there is usually a complete compensatory pause because the AV node or ventricle is refractory to the next sinus impulse - resulting in a missed beat. Bigeminy: ventricular ectopics are occurring so frequently that they happen after every sinus beat. The ECG looks like a normal sinus beat followed immediately by an ectopic, then a normal beat, then ectopic and so on.

Answer 146

- Palpitations: complaints of extra beats, missed beats or heavy beats - Patients may feel faint or dizzy

Answer 147

- ECG: individual random, abnormal, broad QRS complexes on a background of a normal ECG. - Check bloods for anaemia, electrolyte disturbance and thyroid abnormalities

Answer 148

- Reassurance and no treatment in otherwise healthy people - Seek expert advice in patients with background heart conditions or other concerning features or findings (e.g. chest pain, syncope, murmur, family history of sudden death)

Answer 149

- If the ectopics are frequent then left ventricular dysfunction may develop - Can provoke VF = fatal

Answer 150

A prolonged QT interval is the ECG finding of prolonged repolarisation of the muscle cells in the heart after a contraction.

Answer 151

Approximately 1 in 2500-7000 people are affected by long QT syndrome

Answer 152

- Romano-Ward syndrome: the commonest cause of congenital LQT syndrome due to inheritance of a single copy of the variant gene (most commonly KCNQ1), resulting in LQT syndrome without deafness (autosomal dominant) - Jervell-Lange-Nielsen syndrome: due to inheritance of two copies of the variant gene, resulting in marked QT prolongation and sensorineural deafness (autosomal recessive) - Electrolyte imbalances - QT-prolonging drugs

Answer 153

A normal corrected QT interval is <430 ms in males and <450 ms in females. However, in long QT syndrome, the ion channels involved in myocardial ventricular repolarisation are affected, resulting in a prolonged QT interval on an ECG. Long QT syndrome can either be congenital or acquired. Drugs that cause long QT syndrome most commonly do so by blocking potassium channels.

Answer 154

- Micrognathia - Low-set ears - Widely spaced eyes

Answer 155

- Syncope - LQT1: exertional syncope, often swimming - LQT2: syncope following emotional stress, arousal or exercise - LQT3: syncope at night or at rest - Dizziness - Palpitations - Dyspnoea - Collapse or sudden cardiac death

Answer 156

- ECG: to identify the presence of long QT syndrome (QTc >430 ms in males and >450 in females) - Serum electrolytes: especially potassium, magnesium, and calcium

Answer 157

- Holter monitor: allows evaluation of the QT interval behaviour at night (when the patient is bradycardic), and during day-time activity (when the patient is tachycardic) - Exercise tolerance test: especially useful in the diagnosis of LQT1 - Echocardiogram: helpful in ruling out structural or valvular pathology - Genetic testing: useful if a positive family history is uncovered as it allows pinpointing of the channelopathy and subtype responsible

Answer 158

- Conservative: avoid precipitating factors (e.g. stress and exercise), correct electrolyte imbalance, cease QT-prolonging medication - Beta-blocker: - Prevents ventricular tachyarrhythmias in long QT syndrome but does not shorten the QT interval - Propanolol is most commonly used and sotalol must be avoided - Implantable cardioverter-defibrillator (ICD) is indicated for the following patients: - Previous cardiac arrest - Recurrent syncope despite beta-blocker treatment - Unable to tolerate beta-blockers - High-risk patients with a QTc >500ms

Answer 159

- Torsade de pointes: polymorphic ventricular tachycardia (VT) secondary to genetic mutations that result in slow repolarisation and a predisposition to VT. Waiting a longer time for repolarisation (long QT) can result in random spontaneous depolarisation in some areas of heart myocytes. Depolarisation without proper repolarisation is called Torsade de pointes. Management: correct the cause & magnesium infusion - Cardiac arrest and sudden death: secondary to VT degenerating into ventricular fibrillation (VF)

Answer 160

The prognosis of patients with long QT syndrome will be determined by the length of QT prolongation (the longer the interval, the greater the risk of a cardiac event), symptom presence and syncopal episode.

Answer 161

- Caused by congenital accessory conduction pathway between atria and ventricles. The extra pathway that is present in Wolff-Parkinson White Syndrome is often called the Bundle of Kent. - There are 2 types - Type A: +ve delta wave in V1 - Type B: -ve delta wave in V1

Answer 162

Supraventricular tachycardia: may be due to AVRT or pre-excited AF/ flutter

Answer 163

ECG: short PR interval, slurred upstroke (delta wave) and wide QRS complex

Answer 164

- Flecainide, propafenone, sotalol, or amiodarone - Ablation of the accessory pathway

Answer 165

- Prone to AF - May degenerate to VF and cause sudden death

Answer 166

True aneurysms involve all three layers of the arterial wall - the tunica intima, which has endothelial cells; the tunica media, which has smooth muscle; and the tunica adventitia, which has connective tissue as well as vasa vasorum which are the vessels nourishing the aortic wall itself. False/ pseudo-aneurysms only involve one layer of the arterial wall e.g. aortic dissection involves the tunica intima. There are two major types of aneurysms: fusiform aneurysms, which are uniform in shape with symmetrical dilatation and involves the entire circumference of the aortic wall; and saccular aneurysms, which are localised outpouchings of only a portion of the aortic wall.

Answer 167

An abdominal aortic aneurysm (AAA) describes a dilatation in vessel wall diameter of >50%, which typically means a diameter of >3 cm.

Answer 168

- AAAs have a reported prevalence of 1.3-12.7% in the UK - M>F - Most common in the elderly: >60

Answer 169

- Increasing age - Male gender - Atherosclerosis - Smoking - Hypertension - Hyperlipidaemia - Diabetes - Connective tissue disorders: such as Ehlers Danlos and Marfan syndrome, due to changes in the balance of collagen and elastic fibres - Family history

Answer 170

- Degradation of the tunica media and adventitia → vessel dilatation The most important risk factor for abdominal aortic aneurysms is atherosclerosis. In atherosclerosis, chronic inflammation results in the release of enzymes called matrix metalloproteinases which degrade the extracellular matrix in the tunica media, weakening the aortic wall. - An AAA most commonly forms below the level of the renal arteries, known as an infra-renal aneurysm. This is because below this level, the abdominal aorta lacks vasa vasorum (small blood vessels in the adventitial layer that provide nutrients to the aorta). The absence of vasa vasorum in this part of the aorta makes the tunica media particularly susceptible to ischaemia. Thickening of the intima makes it harder for oxygen to diffuse to the tunica media. - An AAA is almost always a true aneurysm, but in 1% of cases may be a false aneurysm (pseudoaneurysm; usually due to trauma). - Inflammatory AAA is a type of AAA that usually affects younger patients and is associated with smoking, atherosclerosis and vasculitis, accounting for 5-10% of aortic aneurysms cases. An inflammatory AAA presents similarly to a normal AAA but may also be associated with fever.

Answer 171

Most aneurysms are asymptomatic and discovered as an incidental finding, whilst symptoms generally only occur in the case of rupture or impending rupture. - Pulsatile abdominal mass - Tachycardia and hypotension: red flags signifying ruptured AAA - Grey-Turner’s sign: flank bruising secondary to retroperitoneal haemorrhage - Cullen’s sign: pre-umbilical bruising

Answer 172

- Flank, back or abdominal pain - Pulsating abdominal sensation

Answer 173

Abdominal ultrasound: definitive diagnosis with high sensitivity (92-99%) and specificity (~100%)

Answer 174

- FBC: leaking AAA may cause anaemia, inflammatory AAA may cause leukocytosis - U&Es: baseline renal function is important prior to a CT angiogram as it requires contrast, whilst hypovolaemia may cause pre-renal acute kidney injury - CRP/ESR: raised in inflammatory AAA - Group and save & crossmatch: vital if a ruptured AAA is suspected in order to ensure blood is available for transfusion - CT angiogram: for more detailed anatomical information, particularly if near the renal arteries; it is also the test of choice for pre-operative planning - MRI: MRI is preferred over CT for females of child-bearing age and those allergic to iodinated contrast

Answer 175

The screening programme in England is offered to all males aged 65 and over as a one-off abdominal ultrasound, and further surveillance is organised if the aneurysm exceeds 3 cm.

Answer 176

- Surveillance: monitor size and offer advice regarding lifestyle measures and controlling cardiovascular co-morbidities e.g. BP control, lowering lipids - Surgical repair: can be elective if asymptomatic and dependent on size or urgent if symptomatic or ruptured AAA - Either open or endovascular aortic repair (EVAR) can be performed - Other: inflammatory AAA may be treated with steroids or immunosuppressants

Answer 177

- AAA rupture: surgical emergency and associated with high mortality and morbidity - Thromboembolism: a thrombus can form in the section of the dilated aneurysm and embolise to distal vessels, causing occlusion -Fistula: e.g. an aortovenous fistula (fistulation with the inferior vena cava) or aortoenteric fistula (fistulation with the gastrointestinal tract) - Urological: - Ureteric obstruction - Surgical: - Open repair is associated with low late graft related complications but higher perioperative complications compared to EVAR - Endovascular repair has higher rates of delayed complications requiring reintervention, such as endoleak

Answer 178

Aneurysmal size directly correlates with the risk of rupture. 20% rupture anteriorly into the peritoneal cavity (poor prognosis), whilst 80% rupture posteriorly into the retroperitoneal space. A symptomatic aneurysm has an 80% mortality if left untreated, whilst a ruptured AAA is a surgical emergency and has a 100% mortality without surgery.

Answer 179

A thoracic aortic aneurysm (AAA) describes a dilatation in vessel wall diameter. The aneurysm may be in the ascending or descending thoracic aorta. The normal size of the mid-descending thoracic aorta is 26-28mm.

Answer 180

- Less common than abdominal aortic aneurysms - Ascending thoraco-abdominal aneurysms occur most commonly in patients with Marfan syndrome or hypertension. - Descending or arch TAAs occur secondary to atherosclerosis and less commonly due to syphilis.

Answer 181

- Increasing age - Family history - Atherosclerosis - Hypertension - Smoking - Bicuspid aortic valve: increased risk of aneurysm - Connective tissue diseases e.g. Marfan syndrome or Ehlers-Danlos syndrome - Previous aortic aneurysm repair - Mycotic aneurysm: due to infection e.g. syphilis - Traumatic injury can lead to aneurysm

Answer 182

Aneurysms are caused by a weakness in the blood vessel wall, so anything that causes the wall to weaken can cause an aneurysm. When a blood vessel wall weakens, it struggles to contain the pressure of the blood pushing against the walls so the diameter of the blood vessel lumen increases. Also, pressure on the blood vessel walls increases as the diameter of the lumen also increases. This makes the aneurysm diameter even bigger, creating a cycle of continuous bulging. General pathology: Involves inflammation, proteolysis and reduced survival of the smooth muscle cells in the aortic wall. TAA's are usually associated with cystic medial degeneration (caused by collagen linking defects leading to deposition of basophilic ground substance in the media). On microscopy, the tunica media appears fragmented and small cleft-shaped spaces appear. Mycotic e.g. due to syphillis: Tertiary syphilis affects the vasa vasorum as syphilis causes inflammation in the tunica intima of the vasa vasorum. This ultimately leads to a narrower lumen and restricts blood supply to the vessel walls of the thoracic aorta, causing atrophy. This is known as endarteritis obliterans.

Answer 183

Thoracic aortic aneurysms usually don’t have any symptoms and may be diagnosed incidentally. - Signs - Aortic regurgitation murmur: due to dilation of area of aortic valve

Answer 184

- Pain in chest, neck, upper back, mid-back or epigastrium - Fever if there is infective cause - May be haemoptysis if bleeding - Symptoms due to compression of local structures e.g. back pain, dysphagia, hoarseness and cough

Answer 185

- Ultrasound - CT or MRI - Aortography may be helpful for assessing the position of the key branches in relation to the aneurysm - Transoesophageal echocardiography can be useful for identifying aortic dissection

Answer 186

- Surveillance: monitor size and control risk factors e.g. smoking cessation, BP control, lower lipids etc - Treat underlying cause - Elective surgery for asymptomatic TAA, depending on size - Urgent surgery for symptomatic or ruptured TAA

Answer 187

- Rupture: once the aorta reaches a crucial diameter it loses all distensibility and a rise in BP to around 200mmHg can exceed the arterial wall strength and may trigger dissection or rupture - Can lead to collapse, shock and sudden death - Cardiac tamponade if there is bleeding into pericardial space - Aortic regurgitation/ insufficiency: if the aneurysm is right above the aortic valve, the aneurysm dilates and can prevent the aortic valve closing properly, leading to regurgitation - Thrombosis: thrombus can form in the aneurysm and break off and embolise to another area, causing occlusion

Answer 188

Aortic dissection describes the condition when a separation has occurred in aortic wall intima, causing blood flow into a new false channel between the tunica intima and tunica media. It is a potentially life-threatening surgical emergency.

Answer 189

- Aortic dissection has a worldwide incidence of 0.5 to 2.95 per 100,000 people annually - M>F - Most common between the ages of 50-70 and is rare below 40

Answer 190

- Hypertension: the most important risk factor - Smoking - Family history of aortic aneurysm or dissection - Coarctation of the aorta: narrowing of vessel - Bicuspid aortic valve - Connective tissue disorders: Marfan and Ehlers-Danlos syndrome - Turner and Noonan syndrome - Trauma - Pregnancy: increased blood plasma volume - Syphilis

Answer 191

An aortic dissection is where there is a tear in part of the tunica intima of the aorta. High-pressured blood flowing through the aorta begins to tunnel between the tunica intima and the tunica media, separating the two layers. As the high-pressured blood continues to shear more and more of the tunica intima off the tunica media, blood starts to pool between the two layers, increasing the outside diameter of the blood vessel. The area where blood collects between the tunica intima and the media is called a false lumen. This most commonly occurs around the ascending aorta and aortic arch, but can affect any part of the aorta. Hypertension is a key risk factor. In order for an aortic dissection to occur, an underlying condition also usually has to exist that weakens the aorta’s wall. Connective tissue disorders e.g. Marfan’s and Ehlers-Danlos syndrome can cause a dissection. Alternatively, aneurysms can be a cause of aortic dissection

Answer 192

- Weak downstream pulses: radio-radial and/or radio-femoral delay - A difference in blood pressure between two arms: >10 mmHg - Hypertension - Tachycardia and hypotension: as condition progresses - Diastolic murmur: due to aortic regurgitation - Involvement of specific arteries - Spinal arteries → paraplegia - Coronary arteries → angina - Distal aorta → limb ischaemia - Marfan syndrome signs - Tall - Arachnodactyly - Hypermobile joints - Narrow face - Ehlers-Danlos syndrome signs - Translucent skin - Easy bruising - Hypermobility of small joints

Answer 193

- Sudden onset, severe ‘tearing’ or ‘ripping’ chest pain that may radiate to the back - Syncope: red flag symptom

Answer 194

There are two main classification systems for aortic dissection: Stanford and DeBakey. Stanford: - Type A: dissection involves the ascending aorta with or without the involvement of the arch and descending aorta, occurring proximal to the left subclavian artery (⅔ of cases) - Type B: dissection does not involve the ascending aorta. Involves only the descending thoracic or the abdominal aorta, occurring distal to the left subclavian artery (⅓ of cases) DeBakey: - Type I: originates in the ascending aorta and involves at least the aortic arch, but can extend distally - Type II: originates and confined to the ascending aorta - Type III: originates in the descending aorta and extends distally, but can extend proximally

Answer 195

- **ECG:** non-specific changes such as T-wave inversion or ST-segment depression. ST-segment elevation may be seen in the inferior leads in some patients - **FBC:** anaemia may be present - **Group & save and crossmatch**: all patients with suspected dissection should have blood crossmatched in case of a transfusion - **Chest X-ray:** may demonstrate a widened mediastinum - **U&Es**: required prior to a CT with contrast and to detect pre-renal acute kidney injury secondary to hypovolaemia - **Contrast-enhanced CT angiogram:** gold-standard imaging. The thorax, abdomen and pelvis should ideally be visualised to determine the extent of aortic disease

Answer 196

Echocardiogram (echo): a transthoracic or transoesophageal echo should be considered in a very unstable patient.

Answer 197

- Blood transfusion - Beta blocker e.g. IV labetalol: aim for a systolic blood pressure of 100-120 mmHg; high pressures are associated with extension of the dissection - Urgent surgical repair/ stenting: open surgery with replacement of the ascending aorta should be performed immediately upon diagnosis

Answer 198

- Conservative management: bed rest and analgesia - Beta blocker e.g. IV labetalol: aim for a systolic blood pressure of 100-120 mmHg; patients are usually monitored closely on the high dependency or intensive care unit - Thoracic endovascular aortic repair (TEVAR): may be performed to reduce the risk of future dissection

Answer 199

- Aortic regurgitation: dissection extends proximally, leading to loss of support for valve leaflets - Myocardial infarction: occurs when dissection propagates proximally; inferior ST-elevation may be seen due to right coronary involvement - Stroke - Renal failure: blood could continue to tunnel between the tunica intima and the tunica media until it reaches another artery that branches off the aorta e.g. renal arteries. The blood in the false lumen can put pressure on this branching artery decreasing blood flow to the kidneys - Pericardial tamponade: blood from the false lumen leaks into pericardial space, and may cause tamponade - Haemorrhage and shock: blood flowing through the false lumen could puncture a hole through the tunica media and tunica externa and bleed into the mediastinum. This can result in rapid death

Answer 200

An untreated proximal aortic dissection will result in a false channel rupture with fatal haemorrhage in 50-60% of patients within 24 hours. It is estimated that 20% of patients die before reaching hospital and a further 30% die before reaching theatre. However, the 5-year survival after surgery for Type A and B dissections is up to 80%.

Answer 201

Peripheral arterial disease (PAD) is a major circulatory disorder characterised by arterial obstruction, leading to reduced blood supply and ischaemia in the lower limbs.

Answer 202

- PAD affects around 13% of the Western population aged over 50 years old - 60% of patients have co-existing ischaemic heart disease - M>F - Prevalence increases with advancing age - Commonly caused by atherosclerosis and usually affects the aorta-iliac and infra-inguinal arteries

Answer 203

- Advancing age: PAD affects 1% of people in their 40s, compared to 15% of those over 70 years old - Male gender: men are affected at a younger age than women - Smoking: the single greatest risk factor and is thought to confer more than a 4-fold increased risk of PAD - Diabetes mellitus - Hypercholesterolaemia - Hypertension - Chronic kidney disease - High serum homocysteine

Answer 204

Peripheral arterial disease is a major circulatory disorder characterised by arterial obstruction, mainly due to atherosclerosis and thrombosis, leading to reduced blood supply and ischaemia in the lower limbs. Other causes may include emboli or functional issues such as vasospasms of the vessels causing constriction. When the muscle receives less oxygen and becomes ischaemic, the cells release adenosine which affects nearby nerves, causing pain. Lactic acid production may also contribute to pain. The three main patterns of presentation are intermittent claudication, critical limb ischaemia and acute limb-threatening ischaemia: Intermittent claudication reflects an inadequate increase in skeletal muscle perfusion during exercise, whilst critical limb ischaemia is considered an advanced form of chronic limb ischaemia. Acute limb-threatening ischaemia is most commonly caused by emboli, usually of cardiac origin, resulting in a sudden decrease in limb perfusion. Emboli tend to lodge at artery bifurcations or in areas where vessels abruptly narrow.

Answer 205

Stage 1 - asymptomatic - low ABPI or lack of palpable pulse Stage 2 - Intermittent claudication - aching or burning in the legs which is relieved with rest - examine for temp differences, skin changes, thin shiny skin, tissue loss on heel, hair loss, bruits. Do Buergers test Stage 3 - Critical limb ischaemia - rest pain, dangling leg over edge of bed for pain relief, risk of limb loss Type 4 - Tissue loss: ulceration or gangrene

Answer 206

- Common iliac: unilateral buttock - Common femoral: unilateral thigh - Superficial femoral: unilateral calf - Aortoiliac (Leriche syndrome) may cause the triad of: - Bilateral buttock and thigh claudication - Absent or decreased femoral pulses - Erectile dysfunction

Answer 207

- **History and examination, assessment of risk factors, family history of thrombosis and impact of symptoms on work, daily living, and socialising** - Primary investigations - **Ankle-brachial pressure index (ABPI):** record systolic blood pressure with an appropriately sized cuff in both arms and in the posterior tibial, dorsalis pedis, and, where possible, peroneal arteries - Duplex ultrasound: first-line imaging, offering some information on the location and severity of stenosis. - Assessment of cardiovascular risk factors: ECG, FBC, U&E, random glucose or HbA1c, serum cholesterol and lipid profiles - Other investigations to consider - Exercise ABPI: for people in whom the diagnosis is uncertain on the basis of resting ABPI (e.g. normal resting ABPI despite exertional claudication) - Computed tomography angiography: vital if surgical management is being planned as it is more accurate than ultrasound in determining the precise anatomy and degree of stenosis - Magnetic resonance angiography: performed as an alternative to CT angiogram if it is contraindicated, e.g. in pregnant women or those in mild to moderate chronic kidney disease

Answer 208

Management of intermittent claudication: - Exercise: - Can be supervised or unsupervised exercise programme - Management of cardiovascular risk factors: smoking cessation, HbA1c control, BP control, diet and weight management, lipid modification (statins), antiplatelet agents (e.g. clopidogrel) - Surgical intervention: - Referral to a vascular surgeon is required if quality of life does not improve after a 3-month course of supervised exercise therapy - Endovascular procedures e.g. balloon dilatation (angioplasty), stents, and atherectomy - Performed on lesions that are deemed to be haemodynamically significant and stenosis has a reasonable likelihood of limiting perfusion to the distal limb - Bypass surgery: diverts blood around blocked artery - Consideration of naftidrofuryl oxalate: vasodilator - If supervised exercise has not led to satisfactory improvement, and the patient prefers not to be referred for consideration of angioplasty or bypass surgery.

Answer 209

- Critical limb ischaemia and acute limb ischaemia - Ulceration and gangrene - Infection and poor tissue healing - Amputation - Multiorgan dysfunction may occur, especially in people with acute limb ischaemia

Answer 210

The course of peripheral arterial disease is not always predictable. It can progress gradually or suddenly. Over a 5-year-period, patients with intermittent claudication often continue to have stable symptoms, whilst 10-20% develop worsening symptoms and 5-10% develop critical limb ischaemia. Amputation is eventually required in 1-2% of these patients. Critical limb ischaemia carries a high risk of amputation and premature death. Most patients with peripheral arterial disease also have atherosclerotic disease affecting the brain or heart and are three times more likely to die of cardiovascular causes

Answer 211

Critical limb ischaemia is defined as rest or night pain for greater than 2 weeks, with or without tissue loss such as ulceration

Answer 212

- There are approximately 500-1000 new cases of critical limb ischaemia per 1,000,000 people - Prevalence increases with age

Answer 213

- Increasing age - Peripheral vascular disease - Cardiovascular co-morbidities: diabetes, hypercholesterolaemia, hypertension, obesity - Family history of vascular disease - Smoking - Sedentary lifestyle

Answer 214

The term chronic limb-threatening ischaemia encompasses critical limb ischaemia and reflects severe peripheral vascular disease, commonly resulting from extensive atherosclerosis. Stenosis of peripheral arteries causes reduced blood flow to the hands, feet and legs, resulting in ischaemia and the formation of ulcers, gangrene and severe pain. Critical limb ischaemia is a chronic condition, as opposed to acute limb ischaemia, and is associated with the formation of collateral vessels, which may result in late presentations.

Answer 215

- Non-healing ulcer or wound with shiny, hairless skin - Gangrene - Absent or diminished pulses in the legs or feet - Reactive hyperaemia: the leg is pale when elevated and red when lowered

Answer 216

- Pain: a history of ‘aching’ intermittent leg claudication for 2 or more weeks - Rest or night pain must be present - Patients often hang their legs out of bed at night to relieve the pain - Evidence of aortoiliac disease (Leriche syndrome) - Claudication of the buttocks and thighs - Absent or decreased femoral pulses - Erectile dysfunction - Cool peripheries

Answer 217

- Ankle-brachial pressure index (ABPI): significant collateral vessels may form in chronic limb ischaemia, resulting in ABPI results that are more difficult to interpret - >1.4 = abnormally calcified vessels; false-negative often due to diabetes - 0.9-1.2 = normal; does not exclude the diagnosis if clinically suspected - 0.5-0.9 = claudication; mild to moderate arterial disease - <0.5 = rest pain, ulceration, gangrene (critical limb ischaemia) - Duplex ultrasound: first-line imaging, offering information on the location and severity of stenosis

Answer 218

- Contrast-enhanced CT angiogram: vital pre-operatively as it is more accurate than ultrasound in determining the precise anatomy and degree of stenosis - Magnetic resonance angiography: performed as an alternative to CT angiogram if it is contraindicated, such as in pregnant women or patients allergic to iodinated contrast

Answer 219

- Conservative management: appropriate for only 20% of patients - Multidisciplinary team (MDT) referral: all patients should be referred to a vascular MDT unless inappropriate, such as palliative patients - Analgesia: paracetamol with either a weak or strong opioid (depending on pain level) - Supervised exercise programme: first-line for patients with chronic limb ischaemia and minimal symptoms such as intermittent claudication, but is less appropriate for critical disease - Revascularisation: intervention is required in 65% of patients with critical limb ischaemia - Endovascular intervention: angioplasty or stenting is offered to 40% of patients and should be offered for short occlusions - Bypass surgery: offered to 25% of patients; preferred for long occlusions - Amputation: considered if revascularisation has been unsuccessful or is inappropriate - Long-term management: - Reduce cardiovascular risk: smoking cessation, exercise (supervised exercise programme), dietary modifications, managing cardiovascular comorbidities, weight loss - Antiplatelet therapy: clopidogrel - Statin: atorvastatin

Answer 220

- Permanent limb weakness or pain: revascularise expediently to avoid this complication - Acute-on-chronic limb ischaemia: patients with longstanding peripheral arterial disease may develop acute limb ischaemia - Gangrene: occurs in the non-viable leg and usually requires amputation

Answer 221

Prognosis is poor. Patients with critical limb ischaemia have a 5-year mortality of 60%, probably due to the fact that these patients are usually vasculopaths with numerous cardiovascular co-morbidities.

Answer 222

Acute limb ischaemia (also known as acute limb-threatening ischaemia) describes a sudden decrease in perfusion due to arterial occlusion, and can result in rapid ischaemia.

Answer 223

Modifiable risk factors: - Diabetes - Smoking - Hyperlipidaemia - Hypertension - Sedentary lifestyle Non-modifiable risk factors: - Family history of coronary artery or peripheral vascular disease - >40 years of age

Answer 224

The most common cause of ALI is embolisation, followed by thrombosis. - Causes of embolisation include atrial fibrillation, a recent myocardial infarction (mural thrombus) and valvular vegetations. The most common site of embolisation is to the femoral artery. - Thrombosis occurs when an atheromatous plaque in an artery ruptures and a thrombus forms on the plaque’s cap. Peripheral vascular disease is the underlying cause of thrombosis. Nerves are the first to be affected, with irreversible damage after 6 hours. Muscles are more tolerant, with irreversible damage after 6-10 hours, whilst the skin is the last to show necrosis.

Answer 225

6 P's Pain, Pallor, Pulselessness, Paresthesia, Perishingly cold, Paralysis

Answer 226

Embolic - sudden onset, Cardiac history, limited history of peripheral artery disease, arrythmia, normal contralateral limb, cold and mottled skin, clear demarcation Thrombotic - vague, progressive, less severe, often no significant cardiac history, significant history of peripheral arterial disease, no arrythmia, abnormal contralateral limb, cool and cyanotic, no clear demarcation

Answer 227

Do not delay surgical intervention by performing investigations if there is a strong clinical suspicion of limb-threatening ischaemia. - Ankle-brachial pressure index (ABPI): - >1.4 = abnormally calcified vessels; false-negative often due to diabetes - 0.9-1.2 = normal; does not exclude the diagnosis if clinically suspected - 0.5-0.9 = claudication; mild to moderate arterial disease - <0.5 = rest pain, ulceration, gangrene (critical limb ischaemia) - ECG: to identify arrhythmias, AF or an acute cardiac event that may have resulted in an embolus - Duplex ultrasound: first-line imaging, offering some information on the location and severity of stenosis - Contrast-enhanced CT angiogram: the most important pre-operative investigation to determine the precise vasculature

Answer 228

- U&Es: electrolyte disturbance may be associated with an underlying arrhythmia, whilst hyperkalemia will be evident if rhabdomyolysis has taken place. Renal function is also required prior to CT angiogram. - Creatinine kinase (CK): elevated if rhabdomyolysis has taken place - Magnetic resonance angiography: as an alternative to CT angiogram if it is contraindicated, such as in pregnancy or in chronic kidney disease

Answer 229

I: Viable IIA: Threatened (salvagaeable if promptly treated) IIB: Threatened (immediate reconstruction needed) III: Irreversible

Answer 230

IV unfractionated heparin (UFH): UFH has a shorter half-life than low-molecular-weight heparin (LMWH), making it an effective, more reversible pre-operative anticoagulant I: revascularise with catheter directed thrombolysis or thrombectomy IIA: revascularise quickly IIB: quick revascularise can be percutaneous or open III: amputatation or palliation - revascularisation would kill the patient Long term: - Reduce cardiovascular risk: smoking cessation, a supervised exercise programme, dietary modifications and managing cardiovascular comorbidities - Antiplatelet therapy: clopidogrel - Statin: atorvastatin 80mg

Answer 231

- Gangrene: occurs in the non-viable leg and usually requires amputation (Rutherford III) - Rhabdomyolysis: due to the death of muscle fibres and release of their contents into the bloodstream, potentially resulting in acute renal failure and arrhythmias - Ischaemia-reperfusion injury: reperfusion of the ischaemic limb can release reactive oxygen radicals and inflammatory mediators, resulting in swelling and muscle ischaemia. Damaged muscle cells release toxic metabolites, resulting in arrhythmias and acute kidney injury - Permanent limb pain/weakness

Answer 232

The prognosis will be determined by the speed of diagnosis and subsequent revascularisation. An amputation is carried out in up to 40% of cases, and the 30-day mortality can be up to 30%.

Answer 233

Infective endocarditis (IE) is an infection of the endocardium (including the valvular structures, the chordae tendineae, sites of septal defects etc). Endocarditis typically affects valves - native valves or prosthetic valves.

Answer 234

- IE is a rare disease. Between 2009-2010, there were 3,969 episodes of acute and subacute endocarditis in the UK - M>F - More common in developing countries - The mitral valve is most commonly affected overall - The tricuspid valve is most associated with IV drug use

Answer 235

Staph Aureus - most common cause overall - associated with IV drug use and prosthetic heart valves, has a high mortality Staph epidermidis - associated with indwelling lines and prosthetic valves Strep bovis - associated with colon cancer Strep viridans (e.g strep mitis and sanguinis) - second most common cause as a group HACEK (rare culture negative causes): H - Haemophilius A - Aggregatibacter C - Cardiobacterium E - Eikenella K - Kingella Other causes: Coxiella burnetti, Bartonella, Brucella, Previous antibiotic therapy, Group B strep, Candida albicans, fungus or may be non abcterial thrombotic endocarditis (Malignancy or SLE)

Answer 236

- Male gender: men are 2.5 times more prone to endocarditis than women - Previous infective endocarditis - Prosthetic heart valves or implantable cardiac device - Congenital heart disease esp affecting the valves - Rheumatic heart disease causing damage to the valves - Poor dental hygiene/ dental treatment: a way of microbes entering - Intravenous drug use (IVDU) typically affects tricuspid valve - Intravenous catheter - Immunosuppression

Answer 237

Firstly, there is an abnormal endocardium. This can be due to congenital heart disease, rheumatic heart disease, prosthetic valves etc. This can cause turbulent blood flow and damaged endothelium which exposes the underlying collagen and tissue factor, causing platelets and fibrin to adhere, which forms a thrombus. This is called Nonbacterial Thrombotic Endocarditis. If bacteria is added to this, it results in infective endocarditis. Microbes can be introduced into the bloodstream in many ways e.g. open wound, abscess, surgical procedures, IV drug use etc. Mostly, these organisms are killed but sometimes they survive and find this thrombus and attach to them, setting up an infection (vegetation). The microbes use proteins (adhesins) to adhere to one another as well as creating a biofilm which allows them to further aggregate. These vegetations usually occur in areas of lower pressure e.g. on the atrial side of the valve, rather than the ventricular side. Sometimes, the vegetations can detach from the valve, and little clumps of pathogens, called septic emboli, can float through the bloodstream. This causes a range of symptoms depending on its destination. There may also be an immune reaction with antigen-antibody complexes that form and deposit in different parts of the body. Endocarditis can be categorised as follows: Non-bacterial thrombotic 'marantic': - A non-infective cause of endocarditis secondary to thrombus formation on the valvular surface - Associated with malignancy or SLE (Libman-Sacks endocarditis: antigen-antibody complexes attack the endocardium) Acute: - Develops over days to weeks - Most commonly associated with S. aureus - Rapid valvular destruction Subacute: - Develops over weeks to months - Most commonly associated with S. viridans

Answer 238

- Heart murmur: due to turbulent blood flow - Splinter haemorrhages: red-plum lines under the nails due to microemboli deposition - Janeway lesions: painless plaques on palms and soles due to septic microemboli deposition - Osler’s nodes: painful nodules on fingers or toes due to immune complex deposition - Roth’s spots: white centred retinal haemorrhages due to immune complex deposition - Signs of glomerulonephritis: due to immune complex deposition in kidneys - Mild splenomegaly

Answer 239

- Fever or chills - Headache - Shortness of breath - Night sweats, malaise, fatigue, weight loss - Joint pain: may be due to septic emboli

Answer 240

- Inflammatory markers: raised WCC often with neutrophilia, as well as raised CRP and ESR - Blood cultures: 3 sets of blood cultures, 1 hour apart, ideally before initiating antibiotic therapy - Some organisms cause culture negative endocarditis as they are hard to culture. Serology or PCR may be used for these. - Other patients may have culture negative endocarditis as they had taken antibiotics prior to blood culture. - Echocardiogram (echo): confirms diagnosis. Used to visualise the heart and look for vegetations or the way the valve’s move etc - A transthoracic echo is first-line but if this is negative and clinical suspicion remains, a transoesophageal echo should be performed, which has a higher sensitivity for endocarditis - Chest X-ray: to screen for other causes of dyspnoea, and to detect evidence of heart failure e.g. pulmonary congestion - 12-lead ECG: disease progression is associated with conduction defects. Usually shows prolonged PR interval - Urinalysis: glomerulonephritis secondary to septic emboli may cause haematuria

Answer 241

Need 2 major or 1 major and 3 minor Major criteria: - Two positive blood cultures - Endocardial involvement on echo (e.g. vegetation, Perivalvular abscess, new partial dehiscence of prosthetic valve, new valvular regurgitation Minor criteria: - Predisposing heart condition or IVDU - Fever >38 - Immunological phenomena (glomerulonephritis, Osler nodes, Roths spots, Rheumatoid factor) - Microbiological evidence - Positive blood cultures not meeting major criteria - Vascular abnormalities - Arterial emboli, Septic emboli, Pulmonary infarct, Intracranial haemorrhage

Answer 242

IV antibiotics: generally for 4-6 weeks. Patients usually require a central line or peripherally inserted central catheter (PICC)

Answer 243

Empirical: Native valve - Amoxicillin Empirical: Prosthetic valve - Vancomycin and gentamicin and rifampicin Staph: native valve - Flucloxacillin Staph (MRSA): native - Vancomycin and rifampicin Staph - prosthetic: Flucloxacillin, Rifampicin and Gentamycin Staph (MRSA) - prosthetic: Vancomycin, Rafampicin and gentamicin Strep - native: Benzylpenicillin w/ or wo/ gentamicin Enterococci: Amoxacillin HACEK: Ceftriaxone w/ or wo/ gentamicin

Answer 244

- Surgery: aim to remove infected tissue and repair or replace affected valves - Indications include: decompensated heart failure, resistance to antibiotic therapy, severe sepsis, perivalvular abscess, intracardiac fistulae, prosthetic valve endocarditis

Answer 245

- Regurgitation: as heart valves are damaged due to microbes - Congestive heart failure: heart failure has the greatest impact on prognosis - Septic embolisation: occurs in up to 50% of cases and may involve the lungs, spleen, joints, brain and coronary arteries. - Embolisation to the brain may result in a stroke - Valvular rupture or fistula: mostly involves patients with prosthetic valves or staphylococcus aureus infection - Aortic root abscess: should be suspected in patients with aortic valve endocarditis that fails to improve within 72 hours of appropriate antibiotics; prolonged PR interval on ECG

Answer 246

Death is mainly due to congestive heart failure. However, after one year, the main prognostic factor is age.

Answer 247

Inflammation of the pericardium

Answer 248

- Pericarditis accounts for up to 5% of presentations to the emergency department - M>F - Most common 20-50 years of age

Answer 249

- Idiopathic - Viral: - Coxsackievirus - Mumps - Epstein-Barr virus (EBV) - Cytomegalovirus (CMV) - Varicella-Zoster virus (VZV) - HIV - Bacterial - Tuberculosis - Dressler syndrome: post MI inflammation - Uraemia secondary to kidney disease: high levels of urea irritate the serous pericardium, making it secrete a thick pericardial fluid full of fibrin strands and white blood cells - Systemic autoimmune disorders: immune system attacks own tissue including pericardium - Connective tissue disorder - Hypothyroidism - Trauma - Malignancy - Certain medication e.g. penicillin, anticonvulsants

Answer 250

- Male gender - 20-50 years of age - Previous MI - Viral or bacterial infection - Systemic autoimmune disorders

Answer 251

**The pericardium:** Is composed of two-layers (possessing a space that contains 15-35 mL of fluid), the pericardium is responsible for protecting and restraining the heart. It is innervated by the phrenic nerve, and hence when inflamed, can result in severe pain. Acute pericarditis and effusion: Inflammation is usually idiopathic or caused by a virus, with coxsackievirus being the most common causative pathogen. Fluid as well as immune cells start moving from tiny blood vessels in the fibrous and serous pericardium into the interstitium of those layers, making the layer itself thicker. Due to inflammation of the pericardium, an effusion may develop within the pericardial space, known as a pericardial effusion. The serous pericardium can’t remove the fluid as quickly as it comes in. A pericardial effusion that is large enough to adversely affect heart function is called cardiac tamponade, which is potentially life-threatening. The heart is unable to pump properly, leading to a reduction in cardiac output.

Answer 252

Acute pericarditis generally lasts just a few weeks, whereas chronic pericarditis lasts longer, usually more than 6 months. - Signs - Pericardial rub - Heard at the left sternal edge as the patient leans forward - Extra heart sound of a to-and-fro character - High-pitched or squeaky - Diminished heart sounds: if there is large effusion - Tachycardia - Tachypnoea

Answer 253

- Chest pain - Sudden onset, sharp, central and pleuritic - Relief upon sitting up or leaning forward - Exacerbated by lying flat - May last from hours to days - Fever and myalgia - Shortness of breath - Hiccups: if phrenic involvement - Low BP and light headedness: if effusion is large - Peripheral oedema: suggests right-sided heart failure secondary to constrictive pericarditis - Prodromal viral illness: e.g. upper respiratory tract infection

Answer 254

- ECG: - Pericarditis: widespread saddle-shaped ST-elevation (highly sensitive) and PR depression (highly specific) followed by T-wave flattening and eventual T-wave inversion. - Pericardial effusions: low QRS complex voltage or electrical alternans (QRS complexes have different heights) - Chest X-ray: may demonstrate an associated pericardial effusion showing “water-bottle heart” as there is pooling of fluid at the bottom of the heart - Transthoracic echocardiogram: pericardial effusion shows a 'dancing' heart as it moves around in the fluid - ESR and CRP: elevated secondary to inflammation

Answer 255

- Troponin: elevated in 35-50% of patients - Urea: elevated levels indicate a uraemic cause

Answer 256

- 1st line: NSAIDs and colchicine are often both used together - 2nd line: NSAIDs, colchicine and low-dose prednisolone Colchicine: down regulation of multiple inflammatory pathways and modulation of innate immunity

Answer 257

IV antibiotics and pericardiocentesis (removal of fluid) with washout, culture and sensitivities

Answer 258

Urgent therapeutic pericardiocentesis

Answer 259

- Pericardectomy may be considered for refractory cases of pericarditis unresponsive to medical therapy

Answer 260

- Pericardial effusion: accumulation of fluid in the pericardial sac secondary to pericardial inflammation. Perform urgent pericardiocentesis if there is evidence of cardiac tamponade, such as a raised JVP and hypotension, as this is potentially life-threatening. - Myocarditis: inflammation of the myocardium. Patients may require steroids initially, whilst chronic cases of myocarditis can result in heart failure. - Constrictive pericarditis: a thickened, fibrotic pericardium limits the heart's ability to function normally, potentially resulting in congestive heart failure, and is most commonly associated with tuberculosis. Complete resection of pericardium may be required.

Answer 261

The majority of cases of idiopathic and viral pericarditis are self-limiting, whereas bacterial (purulent) pericarditis can be fatal if untreated. Factors associated with a poor prognosis include a pericardial effusion, high fever, a sub-acute course and resistance to NSAIDs.

Answer 262

If the inflammation persists for weeks to months, the process is called chronic pericarditis.

Answer 263

- Certain causes of pericarditis such as tuberculosis, bacterial infection and rheumatic heart disease result in the pericardium becoming thick, fibrous and calcified. - Can occur after any form of pericarditis.

Answer 264

In chronic pericarditis, immune cells initiate fibrosis of the serous pericardium which produces an inelastic shell around the heart making it hard for the ventricles to expand. Over time, it becomes harder for the heart to relax or expand, and the stroke volume decreases. To compensate, the heart rate increases. As these changes are chronic, allowing the body time to compensate, this condition is not as immediately life-threatening as cardiac tamponade. In the later stages of constrictive pericarditis, the sub-endocardial layers of the myocardium may undergo fibrosis, atrophy and calcification

Answer 265

- Kussmaul’s sign: rise in jugular venous pressure and increased neck vein distension during inspiration - Pulsus paradoxus: an exaggeration in the normal variation in pulse pressure seen with inspiration, such that there is a drop in systolic blood pressure - Diffuse heart sounds - Right heart failure signs - Ascites - Oedema - Atrial dilatation

Answer 266

- CXR: small heart with/without pericardial calcification - ECG: low voltage QRS - ECHO: thickened calcified pericardium restricting the heart’s movement; small ventricular cavities

Answer 267

- May require complete resection of the pericardium

Answer 268

Congestive heart failure

Answer 269

Cardiac tamponade describes a reduction in cardiac output due to a raised intrapericardial pressure secondary to a pericardial effusion

Answer 270

Cardiac tamponade is a rare condition

Answer 271

- Idiopathic - Pericarditis e.g. due to - Infectious: viral, TB - Uraemia: renal failure - Autoimmune - Dressler’s syndrome: post MI inflammation - Iatrogenic - Interventional cardiac procedures - Cardiothoracic surgery - Malignancy - Lung - Breast - Haematological - Trauma - e.g. thoracic knife wound - Aortic dissection - Type A dissection: blood pools in the wall of the aorta, this can rupture and spill into the pericardium - Rheumatological - SLE - Rheumatoid arthritis - Scleroderma

Answer 272

Pericardial effusion causes raised intrapericardial pressure. As the intrapericardial pressures exceeds intrachamber pressures, the chambers of the heart are compressed, reducing stroke volume and cardiac output. This then leads to hypotension. The heart tries to compensate by beating faster. Pericardial effusion can develop into cardiac tamponade depending on how much fluid accumulates. The presentation of cardiac tamponade varies depending on the rate of accumulation of fluid. In cases where fluid accumulates quickly, a small volume may cause tamponade acutely. Conversely, when fluid accumulates gradually, the pericardium may increase in compliance allowing for larger volumes before symptoms develop.

Answer 273

- Beck’s triad: - Hypotension: due to reduced cardiac output - Kussmaul’s sign: raised JVP (heart failure) & distended jugular veins The atria can’t distend enough to accommodate the venous blood returning to the heart. That blood will have nowhere to go but back into the veins, causing distended jugular veins. - Muffled heart sounds (pericardial fluid muffles sound transmission) - Tachycardia: compensatory mechanism - Pulsus paradoxus: systolic blood pressure reduction of >10mmHg on inspiration Normally, inspiration creates a negative pressure which pulls blood into the heart, momentarily increasing systemic venous return. When that happens, the right heart volumes increases, and the right ventricle expands into the pericardial space, so it doesn’t affect the left heart volume at all. During inspiration in someone with cardiac tamponade, the right ventricle can’t move into the pericardial space, so the extra volume pushes the interventricular septum leftwards. This leads to a reduction in left ventricular diastolic volume, a lower stroke volume, and a drop in systolic blood pressure during inspiration. - Prolonged capillary refill time - Cool peripheries

Answer 274

- Dyspnoea - Coughing - Chest discomfort - Lightheadedness: if large effusion - Peripheral oedema: heart failure - Confusion: decreased CNS perfusion

Answer 275

- Primary investigations - ECG: tachycardia, low QRS complex voltage and electrical alternans (QRS complex varies in amplitude as the heart moves in the fluid) - 3Bloods - Inflammatory markers: if raised may suggest underlying pericarditis - Troponin: myocardial infarction can cause ventricular rupture and subsequent tamponade - Imaging - Transthoracic echocardiogram: diagnostic and allows for visualisation of the effusion and the effect on cardiac function. Shows a 'dancing' heart as it moves in the fluid. - Chest X-Ray: enlarged cardiac silhouette - Other investigations to consider - Pericardial fluid analysis: this is obtained through pericardiocentesis, which is a therapeutic procedure, and may reveal the underlying aetiology

Answer 276

- Pericardial effusion with no evidence of tamponade - Conservative management: repeat echocardiograms and monitor blood pressure - NSAIDs or colchicine if the underlying cause is suspected pericarditis - Pericardiocentesis: may be required for larger effusions - Perciardial effusion with evidence of tamponade - Urgent pericardiocentesis: a needle is inserted between the xiphisternum and left costal margin and directed towards the left shoulder. This can be done under ultrasound guidance. Pericardial fluid can be aspirated to relieve intrapericardial pressure - Urgent surgical drainage is usually indicated in cases related to neoplasia, a purulent effusion, or haemopericardium (e.g. trauma)

Answer 277

- Cardiac arrest: increased intrapericardial pressures may eventually lead to a significantly reduced cardiac output and cardiac arrest - Constrictive pericarditis: a small proportion of patients with a pericardial effusion will go on to develop constrictive pericarditis once the effusion is treated

Answer 278

There is a risk of haemodynamic instability and cardiac arrest. The prognosis of idiopathic pericardial effusions is usually good. However, if the pericardial effusion is caused by malignancy, the prognosis is poor

Answer 279

Aortic stenosis (AS) represents obstruction of blood flow across the aortic valve due to pathological narrowing. It can be supravalvular (e.g. fibrous ridge above valve), subvalvular (e.g. fibrous ridge is below valve) or valvular.

Answer 280

- Aortic stenosis is the most common valvular disease in Europe and North America. - Typically a disease of the elderly, presenting in the seventh or eighth decades of life. - M>F

Answer 281

- Hypercholesterolaemia - Hypertension - Smoking - Diabetes - Rheumatic heart disease - Bicuspid aortic valve

Answer 282

Physiology: The aortic valve is normally made up of three leaflets: the left, the right, and the posterior leaflet. It opens during systole to allow blood to be ejected to the body. During diastole, it closes to allow the heart to fill with blood and get ready for another systole. Pathophysiology: Aortic stenosis refers to when the aortic valve doesn't fully open, making it harder for blood to be pumped out. Usually, the aortic valve opens to about 3-4 cm^2, but with stenosis it can become less than 1 cm^2. Causes include: - Mechanical stress over time: damages endothelial cells around the valves, causing fibrosis and calcification, which hardens the valve and makes it more difficult to open completely. - Bicuspid valve: 2 leaflets instead of 3. This makes them more at risk of fibrosis and calcification as the mechanical stress that’s usually distributed between three leaflets is now split between only two leaflets - Other congenital variations of the valve: patients may have one, two, three or four leaflets. - Chronic rheumatic fever: causes repeated inflammation and repair, leading to fibrosis. In this case, the leaflets can fuse together — commissural fusion The valve doesn't open as easily but as the left ventricle contracts, it creates a high pressure that eventually pushes on the valve until it finally snaps open, causing a characteristic “ejection click.” As the blood has to flow through a narrow opening, there’s turbulence which creates a murmur - initially gets louder as more blood flows past the opening, and then becomes quieter as the amount of blood flowing subsides (crescendo-decrescendo murmur). As the left ventricle has to generate higher pressures, it undergoes concentric hypertrophy. Despite this, enough blood may still not leave the heart. This leads to symptoms of heart failure which will vary depending on which organ is affected e.g. brain = syncope

Answer 283

- Ejection systolic murmur: heard loudest at the aortic area; crescendo-decrescendo character. Loudness of murmur is not related to severity. - Ejection click: as valve snaps open due to high pressure from LV - Soft/ absent S2: aortic component of the second heart sound may become quieter in more severe disease as the valve leaflets fail to oppose each other forcefully. - Fourth heart sound (S4): caused by the atria contracting against stiff ventricles. - Palpable thrill during systole - Slow rising pulse (pulsus tardus) and narrow pulse pressure (pulsus parvus) - Left ventricular hypertrophy on imaging

Answer 284

(Symptoms may be worse on exertion) - Fatigue - Shortness of breath - Angina - Dizziness - Fainting - Epistaxis and bruising: turbulent flow across the stenotic aortic valve can lead to an acquired von Willebrand deficiency

Answer 285

ECHO: gold standard investigation. 2 measurements are obtained: left ventricular size and function, and doppler derived gradient and valve area

Answer 286

- ECG: left ventricular hypertrophy (deep S-waves in V1 and V2, tall R-waves in V5 and V6); may also show left ventricular strain in severe disease - ECG exercise stress test - CXR: shows dilated ascending aorta and left ventricular hypertrophy; cardiomegaly if heart failure develops - BP - FBC - Clotting

Answer 287

Echocardiograms, ECGs and exercise testing to monitor the progression of the condition.

Answer 288

- Patients with more significant stenosis may need to restrict physical activities. - Treatment options include: - Percutaneous balloon aortic valvoplasty: dilate narrow valve - Surgical aortic valvotomy: stenotic valve leaflets are forced apart - Valve replacement: can be mechanical or bioprosthetic - Transcutaneous Aortic Valve Implantation (TAVI): inflate balloon across narrow valve and then leave a stent in place - Prophylaxis for increased risk of infective endocarditis is no longer used

Answer 289

- Left ventricular outflow tract obstruction - Heart failure: as blood flow to the body (and back to the heart) is reduced - Ventricular arrhythmia - Bacterial endocarditis - Microangiopathic haemolytic anaemia: damage to red blood cells as they are forced through a smaller valve, splitting them into fragments called schistocytes. - Sudden death, often on exertion

Answer 290

Aortic regurgitation (AR) is the diastolic leakage of blood from the aorta into the left ventricle.

Answer 291

- Tends to present between the fourth and sixth decades of life. The prevalence of aortic regurgitation increases with advancing age. - M>F - Severe disease is seen in < 1% of the population.

Answer 292

IE, Rheumatic fever, congenital abnormalities, connective tissue disorder, aortic dissection, aortic aneurysms

Answer 293

Aortic regurgitation/ insufficiency refers to when the aortic valve doesn't close properly, allowing blood to leak back into the left ventricle. Causes include: - Aortic root dilation: most are idiopathic but can also be caused by aortic dissection, aneurysms, syphilis, connective tissue disorders and aortitis. - Valvular damage e.g. due to infective endocarditis or rheumatic fever: chronic inflammation leads to fibrosis, but instead of fusing the valve leaflets together, it makes it so that they don’t form a strong seal, and instead let blood leak through. - Congenital causes: e.g. bicuspid or quadcuspid valve Patients with aortic regurgitation will have an early decrescendo diastolic murmur, caused by the blood flowing back through the valve. As blood leaks back from the aorta into the left ventricle, the left ventricular blood volume increases, which increases the stroke volume. More blood pumped out of the heart per squeeze requires more pressure, so systolic blood pressure increases. During diastole, though, there’s less blood volume in the aorta so diastolic blood pressure decreases. A high systolic pressure and low diastolic pressure is known as hyperdynamic circulation. Patients with a hyperdynamic circulation have bounding pulses, or water-hammer pulses, as the blood slams against the walls of the arteries with each heartbeat. Over time, the increase in blood volume in the left ventricle causes it to undergo eccentric ventricular hypertrophy. Aortic regurgitation can be acute or chronic. Chronically, there can be compensation (LV hypertrophy). However, acutely valvular incompetence develops and blood starts to back up in the atria → pulmonary system etc

Answer 294

- Early decrescendo murmur: due to blood flowing back into LV - Soft S1 and S2 - Apex beat is displaced laterally - Wide pulse pressure: due to high systolic pressure and low diastolic pressure - Bounding pulses/ water-hammer pulse - de Musset’s: head bobbing with each beat due to severe bounding pulses - Quincke’s sign: pulsation of capillary beds in fingernails due to bounding pulses - Traube’s: pistol shot femoral pulses - Duroziez’s: to and fro murmur heard when stethoscope compresses femoral vessels. - Müller’s: pulsation of uvula. - Raised JVP: if acute regurgitation as blood backs up - LV hypertrophy seen on imaging

Answer 295

Dyspnoea, chest pain, palpitations and syncope

Answer 296

ECHO: gold standard investigation; allows visualisation of the origin of regurgitant jet and its width, detection of aortic valve pathology and compensatory changes e.g. LV hypertrophy and function Other: BP ECG: left ven hypertrophy in chronic AR (deep S wave in V1 and V2, tall R-waves in V5 and V6) CXR MRI CT Angiography

Answer 297

serial echocardiograms

Answer 298

- Vasodilators e.g. ACE-inhibitors (ramipril) will improve stroke volume and reduce regurgitation (only if patient is symptomatic or has hypertension) - Root replacement, if needed - Replacement of the valve: can be mechanical or bioprosthetic - Infective endocarditis prophylaxis no longer recommended

Answer 299

- Heart failure - Pulmonary oedema and cardiogenic shock: as blood backs up during acute aortic regurgitation as there is no compensatory mechanism. Cardiac output is also not maintained in acute regurgitation as there is no LV hypertrophy to pump out the extra blood that has leaked back.

Answer 300

Mitral stenosis is a narrowing of the mitral valve orifice, making it difficult for blood to flow from the left atria to the left ventricle.

Answer 301

- M>F - Prevalence and incidence is decreasing due to a reduction of rheumatic heart disease

Answer 302

Mitral valve stenosis refers to when the mitral valve doesn't open enough which makes it harder to fill the left ventricle. Causes include: - Rheumatic fever: most common cause; inflammation causing the leaflets to fuse together (commissural fusion). The normal mitral valve opening (~4-6 cm^2), can narrow down to 2cm^2. Over time, the condition causes valve thickening, cusp fusion, calcium deposition, a severely narrowed (stenotic) valve orifice and progressive immobility of the valve cusps. - Congenital MS - Mitral annular calcification - Radiation associated MS - Carcinoid associated valve disease - Fabry's disease As the volume of blood in the left atrium increases it causes higher pressures in the left atrium. Higher pressures flowing through the fibrotic valve make a “snap” sound when the valve opens, which is followed by a diastolic rumble as the blood is forced through the smaller opening. A constant elevation in both blood volume and pressure in the left atrium causes it to dilate, and can allow blood to back up into the pulmonary circulation (pulmonary congestion and pulmonary oedema). This can ultimately result in pulmonary hypertension and right sided heart failure. As the left atrium dilates, the muscle walls stretch and the pacemaker cells become more irritable, increasing the risk of atrial fibrillation. The dilated atria may also press on nearby structures, causing certain symptoms.

Answer 303

- Loud S1 snap: due to valve opening under high pressures; as the valve cusps become more immobile the sound softens - Mid-diastolic murmur: as blood flows through small opening; heard best with patient lying on the left side in held expiration - The more severe the stenosis, the longer the diastolic murmur and the closer the opening snap is to S2 - Atrial fibrillation - Mitral facies (malar flush): due to vasoconstriction in response to diminished cardiac output - Signs of right sided heart failure: raised JVP, peripheral oedema, hepatomegaly - Signs of pulmonary hypertension: right ventricular heave, prominant a-wave

Answer 304

- Dyspnoea: due to pulmonary congestion - Reduced exercise tolerance - Haemoptysis: due to rupture of bronchial vessels due to the elevated pulmonary pressure - Chest pain (angina) - Dysphagia: if dilated atria presses on nearby oesophagus - Ortner syndrome: haorse voice as dilated atria causes left recurrent laryngeal nerve palsy

Answer 305

- ECHO: gold standard investigation; doming pattern of valve - Transthoracic ECHO: both the valve area and transmitral gradient can be assessed - Transoesophageal ECHO: performed to look for left atrial thrombosis either after an embolic episode (e.g. stroke) or prior to percutaneous mitral commissurotomy. - CXR: left atrial enlargement - double right heart border, splayed trachea, prominent atrial appendage; pulmonary oedema and congestion; calcified mitral valve - ECG: atrial fibrillation; p-mitrale signifying enlarged atria; right axis deviation and tall R-waves in V1 if right ventricular hypertrophy - Stress testing e.g. exercise stress echo or dobutamine stress test.

Answer 306

- Beta-blockers e.g. atenolol and digoxin: control heart rate and prolong diastole for improved diastolic filling - Valve repair or surgical replacement of the valve. Options include: - Percutaneous mitral commissurotomy (PMC): balloon enters via right femoral vein. Balloon is inflated to alleviate the stenosis. - Open surgery - Diuretics: for fluid overload - Anticoagulation/ Vitamin K antagonist: due to risk of thrombus formation

Answer 307

- Pulmonary hypertension and right sided heart failure: as blood backs up in the pulmonary system - Atrial fibrillation - Thrombus formation: due to AF causing blood to stagnate. These thrombi can make it into the systemic circulation - Stroke: due to thrombus embolising to brain

Answer 308

Mitral regurgitation refers to an incompetence of the valve that may occur due to abnormalities to the valve leaflets, subvalvular apparatus or left ventricle. This ultimately causes blood to flow back into the left atria from the left ventricle.

Answer 309

Mitral regurgitation is the second most common indication for valvular surgery

Answer 310

- Associated with females - Advanced age - Connective tissue disease e.g. Marfans syndrome or Ehlers-Danlos syndrome - Prior MI - Infective endocarditis - Rheumatic fever - Cardiomyopathies - Certain medications e.g. ergotamine, bromocriptine, pergolide

Answer 311

Mitral regurgitation refers to when the mitral valve doesn't completely shut allowing blood to leak back into the left atria from the left ventricle. Causes include: - Mitral valve prolapse: during left ventricular contraction there is a lot of pressure generated. This pushes on the mitral valve. Normally, the papillary muscles and chordae tendineae keep the valve from prolapsing. But in MV prolapse, there is myxomatous degeneration (weakening of connective tissue). This is sometimes related to connective tissue diseases. This causes a larger valve leaflet area and elongation of the chordae tendineae, which can sometimes rupture which results in the posterior valve leaflet folding up into the left atrium. Patients with a mitral valve prolapse are usually asymptomatic but have a mid-systolic click followed by a systolic murmur. - Damage to papillary muscles post MI: papillary muscles are no longer able to anchor the chordae tendinae which results in the valve leaflets flopping back into the atria. - Dilated and hypertrophic cardiomyopathies - Left sided heart failure: causes ventricular dilation which causes stretching of the mitral valve annulus so it doesn't shut properly. - Infective endocarditis/ rheumatic fever: chronic inflammation leads to leaflet fibrosis, which prevents the leaflets from forming a seal. - Medications (e.g. ergotamine, bromocriptine, pergolide)

Answer 312

- Chronic disease - Compensated state: development of left sided dilatation and eccentric hypertrophy to cope with left atrial and left ventricular volume overload. This is able to maintain a larger stroke volume and ejection fraction. - Decompensated state: eventually the compensation cannot maintain normal cardiac function. This leads to left sided heart failure and blood backs up into the pulmonary system. - Acute disease There is no time for compensation. The regurgitation causes increased pressure within a non-compliant left atrium. This causes a rise in pressure in the pulmonary circulation. This pulmonary hypertension may cause pulmonary oedema to develop and ultimately right sided heart failure.

Answer 313

- Mid-systolic click: in mitral prolapse due to leaflet folding into the atrium and being stopped by the chordae tendineae. - Pansystolic murmur: due to leaking of blood into the left atrium. Intensity of murmur does not correlate to severity. - Soft S1: due to incomplete closure of valve - Additional S3 sound: caused by rapid filling of a dilated ventricle - Apex beat may be displaced laterally - Systolic thrill may be felt in severe disease - Tachycardia: to maintain cardiac output in decompensated states - Signs of heart failure

Answer 314

- Dyspnoea and orthopnoea: due to pulmonary hypertension - Fatigue and malaise: due to reduced cardiac output - Palpitations: due to initial compensation and increased stroke volume - Peripheral oedema: if right sided heart failure

Answer 315

- ECHO: confirmation of an incompetent valve, assessment of the severity and identification the underlying cause. - CXR: left sided enlargement; pulmonary oedema in acute MR - ECG: may reflect recent MI; p-mitrale suggestive of left atrial enlargement; may be AF; signs of LV hypertrophy Other: - Exercise testing - Cardiac MRI - Cardiac catheterisation: used for evaluation of the coronary vessels prior to valvular surgery. Right sided catheterisation can be used to confirm pulmonary hypertension.

Answer 316

- Vasodilators e.g. ACE-inhibitors (ramipril or hydralazine) - Diuretics e.g. furosemide or spiranolactone: for fluid overload - Beta blockers e.g. atenolol/ calcium channel blockers/ digoxin: for heart rate control - Cardiac resynchronisation therapy (CRT) used when appropriate - Anticoagulation in atrial fibrillation and flutter: to prevent thrombus formation - Valve repair or surgical replacement of the valve. - Other surgical measures include: ventricular assist devices, cardiac restraint devices and heart transplantation.

Answer 317

Serial echocardiography: - Mild: 2-3 years - Moderate: 1-2 years - Severe: 6-12 months

Answer 318

Left sided heart failure, Pulmonary congestion, Cardiogenic shock, A Fib, Thrombus formations

Answer 319

- Once patients ejection fraction becomes less than 60% and/or becomes symptomatic then mortality rises sharply - Severe mitral regurgitation has a 5%/year mortality rate

Answer 320

Pulmonary stenosis refers to the pulmonary valve not opening fully, making it harder for blood to reach the lungs. The general narrowing of the right ventricular outflow can be valvular, subvalvular or supravalvular.

Answer 321

- Congenital e.g. associated with Tetralogy of Fallot, William syndrome, Noonan syndrome, congenital rubella syndrome - Mechanical stress

Answer 322

The pulmonary valve is unable to fully open to let blood pass into the lungs. This can be congenital (e.g. irregularly shaped, thickened, fused or not fully developed leaflets) Or due to mechanical stress. This damages endothelial cells around the valves. The damage causes fibrosis and calcification, which harden the valve and make it more difficult for the valve to open all the way. As it is harder for blood to leave the right ventricle, the valve resists for a second before finally snapping open, and this causes a characteristic “ejection click.” As the blood has to flow through a narrow opening, there is increased turbulence, which creates a murmur. This murmur gets louder as more blood flows past the opening, and then it gets quieter as the amount of blood flowing through the valve decreases (crescendo-decrescendo murmur). Overtime, there is concentric right ventricular hypertrophy to help it deal with the higher pressures. Although this occurs, blood may still get backed up in the system causing a variety of symptoms.

Answer 323

- Ejection click - Ejection systolic murmur: crescendo-decrescendo murmur heard loudest at the pulmonary area - Palpable thrill in the pulmonary area - Right ventricular heave due to right ventricular hypertrophy - Raised JVP: as blood backs up - Distended neck veins: due to raised JVP - Hepatosplenomegaly: due to backing up of blood - Cyanosis: as less blood makes it to the lungs

Answer 324

- Swelling of ankles and feet: as blood backs up in the body - Shortness of breath - Fatigue: on exertion - Dizziness - Fainting

Answer 325

- May be found as a murmur on routine baby checks - ECHO: may show right ventricular hypertrophy

Answer 326

- Watch and wait: for mild pulmonary stenosis - Balloon valvuloplasty: balloon is inserted and then inflated to open the narrowed valve and allow more blood flow through it. - Open heart surgery: if valvuloplasty is not appropriate

Answer 327

- Collapse - Right sided heart failure: as blood backs up on the right side. - Microangiopathic haemolytic anaemia: damage to red blood cells as they’re forced through the smaller valve. This splits them into smaller fragments called schistocytes.

Answer 328

Pulmonary regurgitation refers to an ineffective pulmonary valve that allows blood to leak back into the right ventricle.

Answer 329

- Rare and usually asymptomatic - Commonly associated with younger ages

Answer 330

Pulmonary hypertension, Infective endocarditis, rheumatic fever, Previous heart surgery, Congenital defects

Answer 331

Pulmonary regurgitation/ insufficiency refers to when the pulmonary valve doesn’t close all the way, causing blood to leak back into the right ventricle. Causes include: - **Valvular damage e.g. due to infective endocarditis or rheumatic fever**: chronic inflammation leads to fibrosis. Instead of fusing the valve leaflets together, the fibrosis prevents the leaflets from forming a seal so they let blood leak through. - **Previous heart surgery:** e.g. balloon pulmonary valvuloplasty for pulmonary stenosis could have opened the valve too far, or the person might have a malfunctioning prosthetic valve In pulmonary regurgitation, the blood leaking back from the pulmonary artery into the right ventricle causes a diastolic decrescendo murmur (starts out loudest and then becomes more quiet over time). The backflow of blood increases the right ventricular blood volume and the amount of work that the right ventricle has to do during systole. Over time, the right ventricle undergoes eccentric ventricular hypertrophy. Despite this, the right ventricle might not be able to keep up, which can lead to right-sided heart failure.

Answer 332

Initially, pulmonary valve regurgitation might cause no symptoms at all. Over time, it can lead to right-sided heart failure - Decrescendo murmur - RV hypertrophy on imaging - Raised JVP: as blood backs up - Distended neck veins: due to raised JVP - Swelling of ankles and feet: due to backing up of blood - Hepatosplenomegaly: due to backing up of blood in body - Dyspnoea - Decreased exercise tolerance

Answer 333

Valve replacement

Answer 334

Right sided heart failure

Answer 335

Shock is a life-threatening, generalised form of acute circulatory failure with inadequate oxygen delivery to, and consequently oxygen utilisation by, the cells. Often defined by low BP (mean arterial pressure <65mmHg), evidence of tissue hypoperfusion, raised serum lactate. The term ‘shock’ describes a pathophysiological state with many different causes and is not a specific diagnosis.

Answer 336

Blood pressure is one of the key determinants of tissue perfusion: *Blood pressure = cardiac output x resistance to blood flow* *Cardiac output = stroke volume x heart rate* Shock is essentially ischaemia on a global scale - it is circulatory failure of the whole body; blood flow to tissues is dangerously low, which leads to cellular injury, damage of multiple organs, and even lead to multiple organ failure if not treated immediately.

Answer 337

Hypovolemic shock is shock induced by a low fluid volume of blood. A loss of around 20% of total blood volume can be enough to induce hypovolaemic shock. This can be haemorrhagic or non-haemorrhagic: - **Non-haemorrhagic:** the loss of fluid volume isn’t from bleeding e.g. dehydration, burns - **Haemorrhagic:** loss of blood volume through ruptured blood vessels (i.e. loss of blood volume from bleeding) - e.g. GI bleeding, trauma, peri/post-operative This results in total volume filling of the heart going down which ultimately causes stroke volume to go down. This results in decreased cardiac output and then decreased blood pressure. When cardiac output goes down, catecholamines e.g. epinephrine and norepinephrine, ADH, and angiotensin II are released. These all cause vasoconstriction of blood vessels, which increases vascular resistance and heart rate, and in turn, this increases cardiac output. These combined effects increase blood pressure. An indicator that tissues are not getting enough oxygen due to hypovolaemia is a decreased mixed venous oxygen saturation (MVO2). This is the amount of oxygen bound to haemoglobin in the blood coming to the right side of the heart from the tissues. If blood volume is down, then oxygen is down, and so MVO2 will be down. As blood flow also provides heat to the tissues, when it’s down, the skin starts to feel cool and clammy; thus, hypovolaemic shock is considered a cold shock.

Answer 338

This is shock related to pathology of the heart, which prevents it from pumping enough blood to the tissues. - The most common cause is acute myocardial infarction. When the cardiac myocytes die, it can’t contract as hard, which means the stroke volume and cardiac output is reduced. - Another cause is related to obstruction which doesn’t allow the heart to fill properly with blood e.g. pericardial effusion/ cardiac tamponade physically constricts the ventricles and prevents the heart from expanding and contracting normally, reducing the stroke volume and cardiac output. - Other causes include: arrhythmias, pulmonary embolus, tension pneumothorax, myocarditis, valvular disease, aortic dissection In the same way as with hypovolaemic shock, the body releases vasoconstrictors to increase vascular resistance and help maintain blood pressure. MVO2 will also be down since there’s less oxygen being pumped out. A reduction in cardiac output leads to lowered blood flow, so the skin gets cool and clammy; so cardiogenic shock is also considered a cold shock.

Answer 339

- Tachycardia - Tachypnoea - Pulse is weak - Pulse pressure reduced - mean arterial pressure (MAP) may be maintained until a very large amount of blood has been lost! - Skin is warm and flushed: in warm shock - Skin is cold and clammy: in cold shock - Reduced urine output - Weakness - Confusion - Collapse - Coma

Answer 340

- Assess using ABCDE - Capillary refill time: takes more than 3 seconds to turn pink after 5 second of compression; earliest and most accurate sign of shock - Additional investigations based on suspected underlying cause e.g. ECG, troponin, CXR

Answer 341

The treatment of shock depends on the cause. - ABC - Airways: intubation if necessary - Breathing: give O2 - Circulation: establish IV access, raise legs if hypovolaemic, fluid resuscitation and blood transfusion if necessary, ensure haemostasis - Medications that increase heart contractility, cause vasoconstriction, and retain fluid can be administered. - Manage underlying causes e.g. - Septic shock: may require antibiotics - Anaphylactic shock: remove causative agent + adrenaline, chlorphenamine and hydrocortisone - Cardiogenic shock: may require revascularisation

Answer 342

- Organ failure: due to prolonged hypotension - Kidneys - acute tubular necrosis - Lung - Acute Respiratory Distress Syndrome (ARDs) - Heart - myocardial ischaemia and infarction - Brain - confusion, irritability and coma

Answer 343

An atrial septal defect describes when there’s an opening in the heart between the right and left atria.

Answer 344

- ASD due to the ostium secundum accounts for about 10-15% of all congenital heart defects and is the most common congenital heart defect in adults. - ASD due to ostium primum defects is found in around 25% of patients with Down syndrome. - F>M

Answer 345

Down syndrome, foetal alcohol syndrome

Answer 346

An atrial septal defect describes when the septum between the right and left atrium doesn’t close up all the way and remains open even after birth. The types of atrial septal defect from most to least common are: - Ostium secondum, where the septum secondum fails to fully close, leaving a hole in the wall. - Patent foramen ovale, where the foramen ovale fails to close (although this not strictly classified as an ASD). - Ostium primum, where the septum primum fails to fully close, leaving a hole in the wall. This tends to lead to atrioventricular valve defects making it an atrioventricular septal defect.

Answer 347

- Mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border - Splitting S2 sound: due to pulmonary valve closing after the aortic valve - Cyanosis: if reversal of shunt

Answer 348

- Dyspnoea - Difficulty feeding - Poor weight gain - Lower respiratory tract infections

Answer 349

- Atrial septal defects are often picked up through antenatal scans or newborn examinations. - CXR: large pulmonary arteries; may show large heart - ECG: may show right bundle branch block due to right ventricle dilatation - ECHO: hypertrophy and dilation of right side of heart and pulmonary arteries

Answer 350

- Watch and wait: for small ASD as it may spontaneously resolve - Surgical closure of ASD - Trans-catheter closure - Open heart surgery - Anticoagulants: used to reduce the risk of clots and stroke in adults

Answer 351

- Stroke: embolus may cause stroke. Usually an embolus e.g. from DVT travels to right atria → right ventricle → lungs. But, if there is an ASD this may get into the left atria → left ventricle. From here it can end up anywhere in the body. If it reaches the brain, it may cause a stroke. This is known as a paradoxical embolism. - Pulmonary hypertension and right sided heart failure due to right sided overload - Atrial fibrillation or atrial flutter due to right atrial dilatation - Eisenmenger syndrome as right sided pressure increases and blood is shunted from right to left = deoxygenated blood in the systemic circulation

Answer 352

Ventricular septal defect refers to when the ventricular septum has a gap in it. This can vary in size from tiny to the entire septum, forming one large ventricle.

Answer 353

The most common congenital defect

Answer 354

Fetal alcohol syndrome, Downs, Turners

Answer 355

The septum is formed during development as a muscular ridge of tissue grows upward from the apex, and then fuses with a thinner membranous region coming down from the endocardial cushions. If these tissues don’t fuse, then a gap is left between the two chambers; this is a ventricular septal defect, or VSD. The majority of cases are caused by a defect in the membranous portion of the septum. It is a common congenital defect but usually closes spontaneously and so aren't common in adults. **Blood flow in the presence of a VSD:** Deoxygenated blood flows exits the right atrium and can choose to travel into the right ventricles and pulmonary artery or shunt via the septal defect, into the left ventricle. As blood prefers to travel in the direction of less pressure, it continues down into the pulmonary artery. Oxygenated blood then enters the left atrium and then the left ventricle. Here, blood travels from the high pressure LV to the low pressure RV (left to right shunt). The blood flowing through the VSD causes a holosystolic murmur. At this point, patient's are acyanotic as there is no deoxygenated blood entering the systemic circulation. Overtime, the volume of blood on the right side increases, causing an increase in pressure. This reverses the shunt and means that deoxygenated blood can now enter the systemic circulation via the aorta and cause cyanosis - this is known as Eisenmenger syndrome.

Answer 356

- **Pansystolic murmur** at the lower left sternal border - May be a systolic thrill on palpation - **Cyanosis:** due to deoxygenated blood in systemic circulation - **Tachypnoea**

Answer 357

Dyspnoea, Poor feeding, failure to thrive

Answer 358

ECHO, CXR, murmur in new born check

Answer 359

- Watch and wait: for small VSD as they can close spontaneously - Surgical closure of VSD - Trans-catheter closure - Open heart surgery - Antibiotic prophylaxis: should be considered due to increased risk of infective endocarditis

Answer 360

Right sided heart failure, pulmonary hypertension, IE

Answer 361

AV septal defect essentially refers to a hole in the centre of the heart.

Answer 362

An AV septal defect is a hole in the heart. It usually involves the ventricular septum, the atrial septum, the mitral and tricuspid valves. It may be complete or partial. Instead of 2 separate AV valves, there is just one large malformed valve which is usually leaky.

Answer 363

Complete: Breathlessness as neonate, pounding heart, poor weight gain, poor feeding, Cyanosis due to Eisenmengers syndrome, swelling of legs or belly Partial: May present later in adulthood, may present similar to VSD or ASD

Answer 364

ECHO, CXR, ECG, antenatal tests

Answer 365

- Pulmonary artery banding: if large defect in infancy. The band reduces blood flow to lungs thereby reducing pulmonary hypertension and chance of Eisenmenger’s syndrome - Surgical repair (can be challenging) - Partial defect may be left alone if there is no right heart dilatation

Answer 366

Congestive heart failure

Answer 367

Patent ductus arteriosus (PDA) describes the persistent opening of a fetal structure, known as the ductus arteriosus, after birth.

Answer 368

- This condition accounts for about 10% of all congenital heart defects, of which the vast majority, about 90%, are isolated heart defects. - In a minority of cases, PDA can be associated with other congenital problems, such as congenital rubella syndrome - F>M

Answer 369

Maternal infection, Prematurity

Answer 370

Patent ductus arteriosus refers to the structure staying open after birth. - As blood leaves the right ventricle, it has the option of continuing down the pulmonary artery to the lungs or going down the ductus arteriosus to the aorta. As blood moves from high pressure (e.g. aorta) to low pressure (e.g. pulmonary artery) the blood goes to the pulmonary artery. However, when the oxygenated blood goes to the left atrium then the left ventricle, it travels down the pressure gradient from the aorta to the pulmonary artery. This deprives the body of oxygenated blood. At this point, the shunt is left to right, so there is not yet deoxygenated blood travelling to the body. Patient's are not cyanotic. - As the volume in the pulmonary circulation increases, this causes pulmonary hypertension which increases the right pressure. Overtime, this reverses the shunt into a right to left shunt. At this point, deoxygenated blood can reach the systemic circulation and patient's may be cyanotic. This is known as Eisenmenger's syndrome.

Answer 371

Continuous crescendo-decrescendo murmur, bounding pulse, cyanosis, tachycardia Shortness of breath, difficulty feeding, poor weight gain, Lower rep tract infections

Answer 372

ECHO and doppler flow studies, CXR, ECG

Answer 373

- Monitor with ECHO until 1 years of age: may close spontaneously - Indomethacin: NSAID that inhibits prostaglandin E2 so PDA can close - Trans-catheter closure of PDA - Surgical ligation of PDA

Answer 374

Coarctation of the aorta is defined as a narrowing in the aorta. There is an infant (70%) and an adult form (30%).

Answer 375

- Weak femoral pulses - Performing a four limb blood pressure will reveal high blood pressure in the limbs supplied from arteries that come before the narrowing, and lower blood pressure in limbs that come after the narrowing - Tachypnoea - Left ventricular heave due to left ventricular hypertrophy - Cyanosis of lower extremities: seen in infantile coarctation - Bruits over the scapulae and back from collateral vessels - May be a systolic murmur heard below the left clavicle and below the left scapula - Hypertension: as reduced kidney perfusion activated RAAS - Underdeveloped left arm where there is reduced flow to the left subclavian artery: in infantile form - Underdevelopment of the legs: in infantile form - Signs of aneurysm and dissection in adult form - Rib notching seen on CXR

Answer 376

Poor feeding, Grey and floppy baby, claudication in lower extremities, headaches and nose bleeds

Answer 377

CXR, ECG, CT

Answer 378

Management depends on severity - Infantile coarctation: prostaglandin E is used keep the ductus arteriosus open while waiting for surgery Surgical options: - Balloon dilation: used to widen the aorta - Surgical removal of the narrowed area of the aorta - Infantile coartation: surgical ligation of ductus arteriosus

Answer 379

Tetralogy of Fallot (TOF) is a congenital cardiac malformation. It is the most common form of congenital cyanotic heart disease.

Answer 380

- TOF accounts for up to 10% of congenital cardiac abnormalities, making it the most common cyanotic heart defect. - Typically manifests at 1-2 months of life

Answer 381

- Neonates and babies: typically manifests at around 1-2 months of life - Family history of congenital heart disease - Rubella infection - Increased age of the mother (over 40 years) - Alcohol consumption in pregnancy - Diabetic mother - Down syndrome: trisomy 21 - DiGeorge syndrome: chromosome 22q11 deletion - Edwards’ syndrome: trisomy 18 - Patau syndrome: trisomy 13

Answer 382

Tetralogy of fallot is characterised by the following four congenital abnormalities: - Pulmonary stenosis: right ventricular outflow obstruction. This could either be narrowing of the valve itself or narrowing of the infundibulum. This makes it harder for deoxygenated blood to get to the pulmonary circulation. - Right ventricular hypertrophy in order to contract harder and push blood past the stenosis - Ventricular septal defect (VSD) allows blood to shunt between the ventricles. Normally in a septal defect, the blood would shunt from left to right but the right ventricular hypertrophy means pressure on the right exceeds pressure on the left, so blood is shunted from right to left. This means deoxygenated blood enters the left ventricle and then the systemic circulation. - Overriding aorta: entrance to the aorta (the aortic valve) is placed further to the right than normal, above the VSD. This means that when the right ventricle contracts and sends blood upwards, the aorta is in the direction of travel of that blood, therefore a greater proportion of deoxygenated blood enters the aorta. The degree of pulmonary stenosis determines the degree of cyanosis and clinical severity. If the obstruction isn't severe then deoxygenated blood may not enter the systemic circulation. If it does, this results in cyanosis as well as other symptoms such as, clubbing, difficulty feeding, failure to gain weight etc. In the early neonatal period, a patent ductus arteriosus (PDA) may compensate for this, allowing deoxygenated blood to enter the pulmonary circulation. The ductus arteriosus closes around day 2 and babies start to become symptomatic. **Tet spells/ hypercyanotic spells:** This refers to when cyanosis is exacerbated as the infants demand for oxygen increase (e.g. when crying or feeding). Their heart will try to pump more (deoxygenated) blood, leading to a sudden decrease in oxygen saturation. Infants often respond by bringing their knees to their chest as if they are squatting. In doing so, the femoral arteries are partially occluded, thereby increasing systemic vascular resistance and left ventricular pressure. This, in turn, reduces the right to left shunt.

Answer 383

- Ejection systolic murmur: due to pulmonary stenosis - Reduced SpO2, particularly when distressed - Respiratory distress - Cyanosis - Clubbing

Answer 384

- Poor weight gain or ‘failure to thrive’ - Difficulty feeding - Hypercyanotic or ‘tet’ spells: cyanosis, breathlessness and syncope, particularly when crying or feeding - Squatting posture

Answer 385

- May be picked up during antenatal scans - Primary investigations - ECG: evidence of right ventricular hypertrophy, such as right axis deviation - Echocardiogram + doppler flow studies: definitive investigation and will reveal right ventricular outflow obstruction, right ventricular hypertrophy, a ventricular septal defect and an overriding aorta - Chest X-ray: demonstrates a characteristic ‘boot-shaped’ heart due to RV hypertrophy, right-sided aortic arch is seen in 25% of patients

Answer 386

- Conservative: ensure the infant is kept calm and their knees are placed to their chest to reduce the right to left shunt - Supplementary oxygen is essential in hypoxic children as hypoxia can be fatal - Beta blockers can relax the right ventricle and improve flow to the pulmonary vessels - IV fluids can increase pre-load, increasing the volume of blood flowing to the pulmonary vessels - Morphine can decrease respiratory drive, resulting in more effective breathing - Sodium bicarbonate can buffer any metabolic acidosis that occurs - Phenylephrine infusion can increase systemic vascular resistance

Answer 387

Prostaglandin e.g. alprostadil: used in symptomatic babies at birth to maintain a ductus arteriosus, thus allowing shunting of deoxygenated blood into the pulmonary circulation

Answer 388

- Blalock-Taussig (BT) shunt: a temporary palliative measure performed for patients that remain persistently cyanotic whilst awaiting definitive surgery. It increases pulmonary arterial blood flow. - Definitive surgery: all patients eventually need surgical correction of the abnormality, which involves closure of the VSD with a patch and reconstruction of the right ventricular outflow tract.

Answer 389

- Cardiovascular: - Hypercyanotic (‘tet’) spells - severe spells can lead to reduced consciousness, seizures and potentially death - Congestive cardiac failure - Paradoxical emboli - Post-surgical: - Atrial and ventricular arrhythmias: increased long-term risk - Pulmonary regurgitation

Answer 390

In untreated disease, survival is poor with only a quarter of patients reaching age 10. Prognosis following surgical correction is dependent on the patient's anatomy but, in general, is very good. One study reports 30-year survival at almost 89%.

Answer 391

Transposition of the great arteries refers to the switching of places of the pulmonary artery and the aorta.

Answer 392

More males than females

Answer 393

Cause is idiopathic but risk factors include the expecting mother having: - Diabetes - Rubella - Poor nutrition - Alcohol consumption - Being over 40 years old

Answer 394

Normally, there is one big circuit and mixing of blood. When transposition of the great arteries occurs, there are 2 smaller circuits instead of 1 big circuit and there is no mixing of blood. This means blood going to the body is never oxygenated. - **After birth**, all the shunts close. This leads to death, unless there is some way for the deoxygenated and oxygenated blood to mix between pulmonary and systemic circulations. This is possible if the foramen ovale or ductus arteriosus stay open, or if the baby has a ventricular septal defect. However, deoxygenated blood can still get into systemic circulation and cause cyanosis.

Answer 395

Cyanosis, Tachycardia, Respiratory distress, poor feeding, poor weight gain, sweating

Answer 396

ECHO, antenatal ultrasound scans often find it

Answer 397

- Close monitoring during the pregnancy and arrangements should be made so that the woman gives birth in a hospital capable of managing the condition after birth. - Babies are given prostaglandin E to keep ductus arteriosus open (short term solution) - Balloon septostomy: insert a catheter into the foramen ovale via the umbilicus, and inflating a balloon to create a large atrial septal defect. - Open heart surgery: definitive management. A cardiopulmonary bypass machine is used to perform an “arterial switch” procedure within a few days of birth. If present, a VSD or ASD can be corrected at the same time.

Answer 398

Congestive heart failure: the right ventricle pumps out to the higher-pressure systemic circuit, even though it’s built for low-pressure systems, and the left ventricle pumps out to the lower pressure pulmonary circuit, even though it’s built for high pressure systems. So, in response, the right ventricle can hypertrophy and the left ventricle might atrophy. This can lead to heart failure.

Answer 399

Cardiomyopathy is a broad term used to describe a variety of issues that result from disease of the myocardium. Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterised by left ventricular hypertrophy (LVH).

Answer 400

- Second most common cardiomyopathy (after dilated) - 0.2% prevalence - May present at any age - Most common cause of sudden cardiac death in the young

Answer 401

- Autosomal dominant trait: genetic missense mutation in one of the genes that encode proteins in the sarcomere of heart muscle. - Beta-myosin heavy chain mutation is the most common, but it could also be mutations in the myosin binding protein C and Troponin T. - 50% of mutations are sporadic - It is a primary form of cardiomyopathy i.e. not in response to other underlying disease e.g. hypertension or valvular disease

Answer 402

- Family history - Friedreich's Ataxia (autosomal recessive neurodegenerative disease): patients with Friedreich's ataxia often develop hypertrophic cardiomyopathy

Answer 403

Hypertrophic cardiomyopathy refers to when the walls get thick, heavy, and hypercontractile. The muscles grow larger as new sarcomeres are added in parallel to existing ones. The left ventricle is most affected, and this muscle growth is asymmetrical (interventricular septum grows larger relative to the other ventricular wall). Consequences: - Walls take up more room so the cavity is smaller and less able to fill with blood. - Walls are more stiff and less compliant so can't stretch to fill with more blood. - This means less blood is pumped out of the heart - stroke volume is reduced. This can lead to heart failure. In some patients, the muscle growth of the interventricular septum gets in the way of the left ventricular outflow tract during systole. This increases blood velocity through this smaller opening, and pulls the anterior leaflet of the mitral valve toward the septum. This further obstructs the left ventricular outflow tract. As blood is forced through a tiny opening it causes a crescendo-decrescendo murmur (louder as blood first rushes out, and then softer as less blood moves out). Also, patient's might have a bifid pulse: two pulses are felt because the mitral valve is moving toward the outflow tract and causing increased obstruction mid-systole. Another issue with thick powerful heart is that there is a disarray of cardiac myocytes. This affects conduction and can lead to arrhythmias. Furthermore, the bigger heart muscle requires more oxygen (which it doesn't receive). This causes ischaemia which further adds to the risk of developing fast arrhythmias.

Answer 404

- Ejection systolic murmur: crescendo-decrescendo character due to blood flowing through the obstructed left ventricular outflow tract. - The intensity of the murmur can change depending on the level of obstruction - When squatting vascular resistance increases, which makes it harder to eject blood out and increases afterload. This means that the ventricle has more blood stretching it out, so it becomes less obstructed, and the murmur becomes less intense. - When standing or doing a valsalva maneuver, venous return decreases. This decreases preload so less blood is stretching out the ventricle before ejection. The obstruction gets larger and the murmur’s intensity increases. - Bifid pulse: two pulses due to mitral valve moving towards outflow tract mid-systole and causing further obstruction - S4 sound: due to atria contracting and pushing blood into a non-compliant ventricular wall during diastole. - Systolic thrill may be felt - Arrhythmias - Hypertrophy seen on imaging

Answer 405

- Dyspnoea - Palpitations - Chest pain - Dizziness - Syncope - Sudden death, may be first manifestation

Answer 406

ECG (progressive T wave inversion, deep Q waves), ECHO

Answer 407

- Beta blockers or calcium channel blockers: control heart rate. - Digoxin is contraindicated because it tends to increase force of contraction, which can increase the obstruction. - Anti-arrhythmic medication e.g. amiodarone - Consider defibrillator if at high risk of arrhythmias - Anticoagulation: if AF is present as there is higher risk of thrombus formation - Septal myectomy: surgery to remove part of septum causing obstruction

Answer 408

- Left ventricular outflow obstruction - Heart failure - Arrhythmias: as there is disarray of cardiac myocytes as well as due to the heart becoming ischaemic - Sudden death: due to fast arrhythmias

Answer 409

Mortality: 5.9% per year if <14 years, 2.5%/ yr if >14 years Poor prognostic factors: age >14 years or syncope at presentation, family history of HCM or sudden death

Answer 410

Cardiomyopathy is a broad term used to describe a variety of issues that result from disease of the myocardium. Dilated cardiomyopathy refers to when all 4 chambers of the heart dilate (but don't get thicker).

Answer 411

- Most common type of cardiomyopathy - Prevalence: 0.2%

Answer 412

Most often idiopathic - Autosomal dominant - familial - Certain genetic conditions e.g. Duchenne Muscular Dystrophy and haemochromatosis - Infection e.g. coxsackievirus B or Chagas disease, a protozoal infection - Alcohol abuse: alcohol and its metabolites have a direct toxic effect on the myocardium - Chemotherapy drugs e.g. doxorubicin and daunorubicin - Drugs e.g. cocaine - Thyroid disorder - Wet beriberi: Vitamin B1 deficiency - Peripartum cardiomyopathy: dilated cardiomyopathy can develop in the third trimester of pregnancy or in the weeks following delivery. About half of patients recover following pregnancy.

Answer 413

- **Family history** - **Certain conditions** e.g. haemochromatosis - **Alcohol abuse** - **Drug abuse** - **Chemotherapy** - **Certain infections** - **Thyroid disorders** - **Increased BP**

Answer 414

Dilated cardiomyopathy can cause all four chambers of the heart to dilate. It is caused by cytoskeletal gene mutations which result in new sarcomeres being added in series to enlarge the chambers. However, the walls are relatively thin compared to the large chamber size. This leads to weak contractions - less blood is pumped out each contraction. Eventually, patient's develop biventricular congestive heart failure as there is a lower stroke volume (right ventricle unable to push out blood to pulmonary circulation and left ventricle unable to push out blood to systemic circulation) Additionally, when the chambers get larger, they stretch out the atrio-ventricular valves. This can mean the valves don't shut properly, causing regurgitation. Regurgitation may be heard as a systolic murmur. There may also be an S3 heart sound on auscultation, which is the result of blood rushing and slamming into the dilated ventricular wall during diastole. Furthermore, stretching out the muscle walls can irritate the cells in the conduction system, which can lead to arrhythmias.

Answer 415

- Larger heart seen on imaging - Systolic murmur: due to regurgitation - S3 gallop: due to blood rushing hitting the dilated ventricular wall during diastole - Increased pulse - Decreased BP - Displaced and diffuse apex - Arrhythmias - Signs of heart failure e.g. pulmonary oedema, increased JVP

Answer 416

Fatigue and Dyspnoea

Answer 417

- Bloods: BNP elevated; low Na+ implies poor prognosis - CXR: cardiac enlargement; pulmonary oedema - ECG: tachycardia, non-specific T wave changes and poor R-wave progression; may be AF or VT - ECHO: shows dilated heart and low ejection fraction

Answer 418

- Bed rest - Diuretics: to deal with oedema - Beta blockers: to control heart rate - ACE inhibitors: dilate vessels to improve blood flow - Anticoagulation: due to increased risk of thrombus - Biventricular pacing - ICD - Left ventricular assist device (LVAD): mechanical pump that assists the heart in distributing blood - Heart transplant, in extreme cases

Answer 419

Heart failure, Mitral and tricuspid valve regurgitation, Arrhythmias, Thromboembolism, sudden death

Answer 420

Cardiomyopathy is a broad term used to describe a variety of issues that result from disease of the myocardium. Restrictive cardiomyopathy describes when the heart muscle becomes stiffer and less compliant.

Answer 421

Idiopathic, Sarcoidosis, Amyloidosis, Endocardial fibroelastosis, loffler syndrome (eosinophils collect in heart tissue), Haemachromatosis (iron overload), Scleroderma (chronic connective tissue disease causing hardening), Radiation

Answer 422

Restrictive cardiomyopathy describes when the heart muscle is restricted, meaning that it becomes stiffer and less compliant. However, the muscles and size of the ventricles stay roughly the same size, or only get slightly enlarged. Normally, when blood fills the ventricles they stretch out and allow more blood to fill in. When blood fills restricted ventricles, however, they can’t expand as much. This means the ventricles fill with less blood and therefore pump out less blood. This eventually leads to heart failure.

Answer 423

- 3rd and 4th heart sounds - Signs of heart failure - Increased JVP - Elevation of venous pressure with inspiration - Oedema - Hepatomegaly

Answer 424

Fatigue, Dyspnoea, Embolic symptoms

Answer 425

ECG, ECHO, CXR, MRI, Cardiac catheterisation

Answer 426

Treat underlying causes, heart transplant

Answer 427

Heart failure

Answer 428

Right ventricular myocardium is replaced with fibro-fatty material. There is a desmosome (normally hold cardiac cells together) gene mutation, followed by muscle cell replacement with fat and fibrous tissue. Cause is unknown. Familial form is usually autosomal dominant with incomplete penetrance but can be recessive.

Answer 429

- Cardiac cells are held less together thus conduction issues (arrhythmias) - Palpitations - Syncope: during exercise - Signs of heart failure in late disease

Answer 430

ECG change, ECHO, Genetic testing

Answer 431

Beta blockers, Anti-arrhythmic drugs (amiodarone), Cardiac transplant occasionally

Answer 432

Acute rheumatic fever is an autoimmune condition triggered by streptococcus bacteria.

Answer 433

- 325,000 children develop rheumatic fever every year, whilst 2.4 million children have rheumatic heart disease worldwide - Peak age: 5-17 years old - More common in the developing world

Answer 434

Group A β-haemolytic Streptococcus pyogenes infection (rheumatic fever usually follows strep throat)

Answer 435

Child, Developing world, Malnutrition, Overcrowding, Family history, HLA class II

Answer 436

Rheumatic fever develops 2-4 weeks following pharyngeal infection with Group A β-haemolytic Streptococcus pyogenes, in a susceptible population. Some strep bacteria have an M protein on their cell wall. The immune system recognises it as a foreign molecule, and mounts an immune response which produces antibodies against these proteins. These antibodies also cross-react with proteins on some of the body’s own cells, e.g. cells in the myocardium and heart valves, the joints, the skin and the brain. This is known as molecular mimicry and is a type 2 hypersensitivity reaction. Once bound to cardiac tissue, the antibodies activate nearby immune cells, which causes a cytokine-mediated inflammatory response and tissue destruction. Most patients experience joint effects as well as pan-carditis (inflammation affecting all layers of the heart - endocarditis, myocarditis, pericarditis) Histologically, Aschoff bodies (nodules) are found in the hearts of people with rheumatic heart disease due to inflammation. If repeatedly exposed to group A beta-hemolytic streptococcus, the body continues to launch immune attacks against the various tissues leading to chronic rheumatic heart disease. Here, the valves (typically the mitral valve) develop scar tissue from repeated inflammation. The leaflets of the valves become thicker and can fuse together. The chordae tendinae which are attached to the valves can become thickened as well. These changes can cause complications with the valves, most commonly regurgitation. This can progress to stenosis and might even increase the risk of infective endocarditis due to microbial attachment.

Answer 437

- Heart murmur: strongly associated with mitral stenosis (chronic) and regurgitation (acute), and aortic regurgitation - Tachycardia or bradycardia - Pericardial rub on auscultation - Palpitations - Tender joints: usually not swollen - Erythema marginatum: pink or red macules (flat) and papules (raised) with a clear centre on the trunk or limbs - Subcutaneous nodules: firm lumps under the skin made up of collagen, on extensor surfaces

Answer 438

- Recent sore throat or scarlet fever (occurs 2-4 weeks before chest pain) - Chest pain: pleuritic - Shortness of breath - Fever and rigors - Non-pruritic rash - Joint pain: oligo- or poly-arthritis - Severe pain - Often involves the lower limbs - Migratory: different joints become inflamed and improve at different times - Sydenham’s chorea - Rapid, uncoordinated jerking movements of the face, hands and feet - Due to autoimmune reaction against the basal ganglia of the brain - Occurs up to 6 months after initial infection (late symptom)

Answer 439

- Throat swab - Anti-streptococcal antibodies (ASO) titres - Chest X-ray: cardiomegaly and/or evidence of congestive heart failure may be seen - Echocardiogram: may demonstrate mitral and aortic valvular pathology, as well as the presence of pericarditis or a pericardial effusion - Blood cultures: if the patient is pyrexial - Raised WCC - Raised ESR and CRP

Answer 440

Jones Criteria is used to diagnose rheumatic fever and is dependent on evidence of recent streptococcal infection in addition to 2 major criteria, or 1 major and 2 minor criteria: Recent evidence of strep infection: - Rapid group A streptococcal antigen test: positive - Throat culture: positive - Streptococcal antibodies (DNase B, or anti-streptolysin O): elevated or rising - Recent scarlet fever Major criteria: - J – Joint arthritis - O – Organ inflammation, such as carditis - N – Nodules - E – Erythema marginatum rash - S – Sydenham chorea Minor criteria - Fever - ECG Changes (prolonged PR interval) without carditis - Arthralgia without arthritis - Raised inflammatory markers (CRP and ESR)

Answer 441

- Conservative: bed rest, analgesia, immobilise joints in severe arthritis - Antibiotic therapy: benzylpenicillin IV STAT, followed by oral phenoxymethylpenicillin for 10 days - Aspirin and steroids: used to treat carditis - Haloperidol or diazepam: if severe Sydenham’s chorea is present

Answer 442

- All patients should be put on long-term, regular antibiotics to lower chance of developing chronic rheumatic heart disease - Antibiotics: IM benzylpenicillin. The duration will vary depending on patient factors (e.g. could be for 10 years or life-long)

Answer 443

- Rheumatic heart disease: occurs in 30-50% of patients, most commonly affecting the mitral valve, followed by the aortic valve. - Heart failure: may occur in chronic or recurrent cases - Infective endocarditis: previous rheumatic heart disease increases the risk of infective endocarditis - Atrial fibrillation

Answer 444

If left untreated, rheumatic fever usually resolves within 12 weeks. With treatment, rheumatic fever can resolve within a few weeks. 30% to 50% of patients with rheumatic fever develop chronic rheumatic heart disease.

Cardiology Flashcards

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