Structural Heart Disease

Question 1

When does systole start

Answer 1

When mitral and tricuspid valves shut

Question 2

What is the S1, lub sound

Answer 2

When mitral and tricuspid valves shut

Question 3

What happens when the aortic pressure is higher than the ventricular pressure

Answer 3

Systole stops
Tricuspid and aortic valves closing *

Question 4

What valves open at the start of diastole

Answer 4

Tricuspid and mitral

Question 5

What happens in diastole

Answer 5

Volume increases but pressure stays the same

Question 6

What are structural heart diseases

Answer 6

Cover a number of defects which affect the valves and chambers of the heart and aorta
Some congenital and some later

Question 7

Congenital heart diseases?

Answer 7

Atrial septal defect
Ventricular septal defect
Coarctation of aorta
Patent foramen ovale
Patent ductus arteriosus
Tetralogy of fallot

Question 8

Heart diseases that develop later in life?

Answer 8

Valvular dysfunctions (atrial stenosis / regurgitation or muscular (cardiomyopathies)

Question 9

Ventricular septal defect?

Answer 9

Hole in septum
Mix of oxygenated and deoxygenated blood
More blood rushing from high pressure oxygenated left side to right
Leads to right side failure cause it’s overloaded

Question 10

Tetralogy of fallot?

Answer 10

1, Ventricular septal defect
2. Overriding aorta - blood from both sides go to aorta
3. Leads to ventricular septal hypertrophy
4. Pulmonary atresia

Sinotic spell and can Therefore get breathless etc
Emergency surgery needed
Can be shown in scan before baby is born

Question 11

Atrial septal defect?

Answer 11

Milder than VSD

Question 12

Coarctation of the aorta?

Answer 12

Wall of the aorta is constricting
Less cardiac output–> reduced stroke volume
Emergency repair needed

Question 13

What is the most common cause of regurgitation and stenosis?

Answer 13

Rheumatic heart disease (25-49)

Calcific aortic valve disease
Degenerative mitral valve disease (70+ most common)

Question 14

Is aortic or mitral valve disease more prevalent?

Answer 14

Mitral ; esp 55 onwards

Mild mitral regurgitation most common

Question 14

What is the most common valvular disease?

Does it affect older or younger people?

What is is preceded by?

Answer 14

Aortic stenosis

Older people

Aortic sclerosis- aortic valve thickening without flow limitation

Question 14

How is aortic stenosis suspected?

How is it confirmed?

Answer 14

Presence of an early-peaking, systolic ejection murmur

Echocardiography

Question 14

What are risk factors for aortic stenosis?

Answer 14

Hypertension
LDL
Smoking
Elevated C reactive protein
Congenital bicuspid valves
Chronic kidney disease
Radiotherapy
Old age

Question 14

What are the causes of aortic stenosis?

Answer 14

Rheumatic heart disease
Congenial heart disease
Calcium build up

Question 15

What happens during aortic stenosis?

Answer 15

The valvular endocardium is damaged as the result of abnormal blood flow across the valve (in the case of a bicuspid valve) or by an unknown trigger.
Endocardial injury initiates an inflammatory process similar to atherosclerosis and ultimately leads to leaflet fibrosis and deposition of calcium on the valve.
Progressive fibrosis and calcium deposition limit aortic leaflet mobility and eventually produce stenosis.
In rheumatic disease, an autoimmune inflammatory reaction is triggered by prior Streptococcus infection that targets the valvular endothelium, leading to inflammation and eventually calcification.

Long-standing pressure overloadleft ventricular hypertrophy (LVH).

Ventricle to maintain a normal wall stress (afterload) despite the pressure overload produced by stenosis As the stenosis worsens, the adaptive mechanism fails and left ventricular wall stress increases.

Systolic function declines as wall stress increases, with resultant systolic heart failure.

Question 16

What is the history and presentation of aortic stenosis?

Answer 16

Exertional dyspnoea and fatigue
Chest pain
Ejection systolic murmur (≥3/6 is present with a crescendo-decrescendo pattern that peaks in mid-systole and radiates to the carotid)
H/O Rheumatic fever, high lipoprotein, high LDL, CKD, age >65

Question 17

What are the investigations for stenosis and regurgitation?

Answer 17

Transthoracic echocardiography
ECG Chest X ray (LVH)
Cardiac catheterisation
Cardiac MRI

Question 18

What is the management for aortic stenosis?

Answer 18

The primary treatment of symptomatic AS
Asymptomatic patients with severe AS who have an LVEF <50% or who are undergoing other cardiac surgery.
AVR may be considered in asymptomatic patients with very severe AS or severe AS with rapid progression, an abnormal exercise test, or elevated serum B-type natriuretic peptide (BNP) levels
Balloon aortic valvuloplasty
Antihypertensive
ACE inhibitors
Statins

Question 19

What is aortic regurgitation

Answer 19

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

It occurs due to incompetence of valve leaflets resulting from either intrinsic valve disease or dilation of the aortic root

AR is not as common as aortic stenosis and mitral regurgitation

It can be chronic culminate into congestive cardiac failure

It can be acute  medical emergency, presenting with sudden onset of pulmonary oedema and hypotension or cardiogenic shock

Question 20

What are the congenital and acquired causes for aortic regurgitation?

Answer 20

Rheumatic heart disease
Infective endocarditis
Aortic valve stenosis
Congenital heart defects
Congenital bicuspid valves

Question 21

What are the causes for aortic regurgitation related to aortic root dilation?

Answer 21

Marfan’s Syndrome Connective tissue disease/collagen vascular diseases Idiopathic Ankylosing spondilytis Traumatic

Question 22

What is the pathophysiology of acute AR?

Answer 22

Infective endocarditis can lead to rupture of leaflets or even paravalvular leaks. Vegetations on the valvular cusps can also cause inadequate closure of leaflets, resulting in leakage of blood. Chest trauma can cause tear in ascending aorta leading to AR Acute AR Increase blood volume in LV during systoleLV end diastolic pressure increases increase in pulmonary venous pressure dyspnea and pulmonary oedemaheart failure cardiogenic shock

Question 23

What is the pathophysiology of chronic AR?

Answer 23

Bicuspid aortic valve Rheumatic feverfibrotic changes causing thickening and retraction of leaflets Gradually increase in LV volumeLV enlargement and eccentric hypertrophy Early stages Ejection fraction normal or slightly increase after some time Ejection fraction falls and LV end systolic volume rises Eventually LV dyspnoea lower coronary perfusion  ischemia, necrosis and apoptosis

Question 24

What is the history and presentation of acute AR?

Answer 24

Cardiogenic shock Tachycardia Cyanosis Pulmonary edema Austin flint murmur

Question 25

What is the history and presentation of chronic AR?

Answer 25

Wide pulse pressure Corrigan (wate hammer pulse) Pistol shot pulse (Traube sign)

Question 26

What is the management of acute AR?

Answer 26

Ionotropes/vasodilators & valve replacement & repair

Question 27

What is the management of chronic AR?

Answer 27

Chronic asymptomaticIf LV function is normal can be managed by drugs or reassurance Chronic symptomatic--) First line is valve replacement with adjunct vasodilator therapy

Question 28

What is mitral stenosis?

Answer 28

Obstruction to left ventricular inflow at the level of mitral valve due to structural abnormality of the mitral valve Rheumatic fever is the main cause ( in developing countries) As disease progresses it leads to pulmonary hypertension and right heart failure occurs

Question 29

What are the causes for mitral stenosis?

Answer 29

Rheumatic fever Carcinoid syndrome Use of ergot/serotonergic drugs SLE Mitral annular calcification due to aging Amyloidosis Rheumatoid arthritis Congenital deformity of the valve Whipple disease

Question 30

What is the pathophysiology for mitral stenosis?

Answer 30

Initially moderate exercise or tachycardia result in exertional dyspnoea due to increased left atrial pressure Severe mitral stenosis leads to increase in left atrial pressure , transudation of fluid into the lung interstitium leading to dyspnoea at rest or exertion Pulmonary hypertension may develop as the result of it The restricted orifice limits filling of left ventricle limiting cardiac output Hemoptysis if bronchial vein rupture

Question 31

What is the history and presentation of mitral stenosis?

Answer 31

H/0 of Rheumatic fever Dyspnoea orthopnoea Diastolic murmur Loud P2 Neck vein distention Hemoptysis 40-50 years age

Question 32

What is the management of mitral stenosis?

Answer 32

Progressive asymptomatic No therapy required Severe asymptomatic no therapy generally required adjuvant balloon valvotomy Severe symptomatic diuretic, balloon valvotomy, valve replacement & repair adjunct b blockers

Question 33

What is mitral regurgitation?

Answer 33

Abnormal reversal of blood flow from the left ventricle to the left atrium. It is the most frequent valvular heart disease It is caused by the disruption in any part of the mitral valve apparatus

Question 34

What are the causes for acute mitral regurgitation

Answer 34

Mitral valve prolapse] Rheumatic heart disease Infective endocarditis Following valvular surgery Prosthetic mitral valve dysfunction

Question 35

What are the causes for chronic mitral regurgitation

Answer 35

Rheumatic heart disease Drug related SLE Scleroderma Hypertrophic cardiomyopathy

Question 36

What does infectious endocarditis lead to?

Answer 36

Abscess formation, vegetations, rupture of chordae tendineae and leaflet perforation

Question 37

What is the pathophysiology of chronic MR?

Answer 37

Progression leads to eccentric hypertrophy leading to elongation of myocardial fibres and increased left end diastolic volume Increase in preload & a decrease in afterload increase in end- diastolic volume and a decrease in end-systolic volume Eventually prolonged volume overload leads to left ventricular dysfunction and increased left ventricular end-systolic diameter

Question 38

What is the history and presentation of MR?

Answer 38

Dyspnea diminished S1, murmur high Fatigue pitched, blowing xx Orthopnea Chest pain Atrial fibrillation

Question 39

What is the management of acute MR?

Answer 39

Emergency Surgery adjunct preoperative diuretics adjunct intra-aortic balloon counterpulsation

Question 39

What is the management of chronic MR?

Answer 39

Chronic asymptomatic 1st ACE inhibitors Beta blockers if left ventricular ejection fraction is less than 60% 1st line is surgery Chronic symptomatic1st surgery plus medical treatment If left ventricular ejection fraction is less than 30% 1st line is Intra-aortic balloon counterpulsation

Question 40

What is cardiomyopathy?

Answer 40

A disease of the heart muscle that makes it harder for heart to pump blood to rest of your body. Can lead to heart failure The estimated prevalence of dilated cardiomyopathy is 1:2500. This condition is among the most common causes of heart failure. Dilated cardiomyopathy may manifest clinically at a wide range of ages, but this condition most commonly occurs in the third or fourth decade of life. Dilated cardiomyopathy is a progressive, usually irreversible, disease causing global systolic (contractile) dysfunction with heart failure.

Question 41

What are the 3 main types of cardiomyopathy?

Answer 41

Dilated Hypertrophic Restrictive

Question 42

How much of the cardiomyopathies are caused without family history and are idiopathic?

Answer 42

25%

Question 43

What are secondary causes of cardiomyopathy?

Answer 43

Heart valve disease After child birth Thyroid disease Myocarditis Alcoholism Autoimmune disorders Ingestion of drugs Mitochondrial disorders

Question 44

What is the pathophysiology of dilated cardiomyopathy?

Answer 44

Dilated cardiomyopathy is characterized by left ventricular chamber enlargement and systolic dysfunction with normal left ventricular wall thickness. the hallmark gross finding at autopsy is left ventricular dilatation, usually more than 4 cm.  Familial DCM is a genetic condition. In rare autosomal dominant inheritance patterns (at least two family member have idiopathic DCM), first degree relatives (parents, siblings, children) have a 50 percent chance of inheriting the condition lower ejection fraction and increase in the ventricular wall stress and end systolic volumes. Early compensatory mechanisms include an increase in heart rate and tone of the peripheral vascular system. neurohumoral activation of the renin-angiotensin aldosterone system and an increase in circulating levels of catecholamines. levels of natriuretic peptides are also increased. Eventually these compensatory mechanisms become overwhelmed and the heart fails.

Question 45

What is the history and presentation of dilated cardiomyopathy?

Answer 45

Dyspnoea, displaced apex beat, S3 or systolic murmur fatigue, angina, pulmonary congestion low cardiac output

Question 46

What are the investigations for dilated cardiomyopathy?

Answer 46

Genetic Testing Viral serology ECG Chest X ray Cardiac catheterisation Cardiac MRI/CT Scan Exercise stress test Echocardiography

Question 47

What is the management for dilated cardiomyopathy?***

Answer 47

Refer to diagram

Question 48

What is hypertrophic cardiomyopathy?

Answer 48

Hypertrophic cardiomyopathy (HCM) is a genetic cardiovascular disease. It is defined by an increase in left ventricular wall thickness that is not solely explained by abnormal loading conditions. HCM is the leading cause of sudden cardiac death in preadolescent and adolescent children. Familial HCM occurs as an autosomal dominant Mendelian-inherited disease in approximately 50% of cases. Most patients with HCM are asymptomatic. Unfortunately, the first clinical manifestation of the disease in such individuals may be sudden death, likely from ventricular tachycardia or fibrillation.

Question 49

What is the history and presentation of hypertrophic cardiomyopathy?

Answer 49

Sudden cardiac death Double carotid artery impulse S3 gallop, Syncope ejection systolic murmur Presyncope Congestive heart failure Dizziness Palpitations Angina

Question 50

What are the investigations for hypertrophic cardiomyopathy?

Answer 50

Hemoglobin level: Anemia exacerbates chest pain and dyspnea Brain natriuretic peptide (BNP), troponin T levels: Elevated BNP, NT-proBNP, and troponin T levels are associated with a higher risk of cardiovascular events, heart failure, and death Echocardiography Chest Xray Cardiac MRI

Question 51

What is the management for hypertrophic cardiomyopathy?***

Answer 51

Refer to diagram

Question 52

What is restrictive cardiomyopathy?

Answer 52

It is a  less well-defined cardiomyopathy as its diagnosis is based on establishing the presence of a restrictive ventricular filling pattern. It is characterized by diastolic dysfunction with restrictive ventricular physiology, whereas systolic function often remains normal. Atrial enlargement occurs due to impaired ventricular filling during diastole, but the volume and wall thickness of the ventricles are usually normal. RCM accounts for approximately 5% of all cases of diagnosed cardiomyopathies It may be idiopathic, familial (has been related to troponin I or desmin mutations, the latter often in association with a skeletal myopathy), or associated with various systemic disorders, such as haemochromatosis, amyloidosis, sarcoidosis, Fabry's disease, carcinoid syndrome, scleroderma, anthracycline toxicity, or previous radiation. 

Question 53

What is the pathophysiology for restrictive cardiomyopathy?

Answer 53

Increased stiffness of the myocardium due to familial or other secondary causes e.g amyloidosis Infiltrative cardiomyopathies are characterized by deposition of abnormal substances (ie, amyloid proteins, noncaseating granulomas, iron) within the heart tissue. Infiltration causes the ventricular walls to stiffen, leading to diastolic dysfunction. Restrictive physiology predominates in the early stages, causing conduction abnormalities and diastolic heart failure. Adverse remodelling may lead to systolic dysfunction and ventricular arrhythmias in advanced cases.  Increased stiffness of the myocardium causes ventricular pressures to rise precipitously with small increases in volume. Thus, accentuated filling occurs in early diastole and terminates abruptly at the end of the rapid filling phase. Patients typically have reduced compliance (increased diastolic stiffness), and the left ventricle cannot fill adequately at normal filling pressures Reduced left ventricular filling volume leads to a reduced cardiac output. 7

Question 54

What is the history and presentation of restrictive cardiomyopathy?

Answer 54

comfortable in the sitting position because of fluid in the abdomen or lungs, and they frequently have ascites and pitting edema of the lower extremities. The liver is usually enlarged and full of fluid, which may be painful. Weight loss and cardiac cachexia are not uncommon. Easy bruising, periorbital purpura, macroglossia, and other systemic findings, such as carpal tunnel syndrome, should be an indication for the clinician to consider amyloidosis. Increased jugular venous pressure is present. The pulse volume is decreased, consistent with decreased stroke volume and cardiac output.

Question 55

What are the investigations for restrictive cardiomyopathy?

Answer 55

CBC, Serology, Amylodosis check, Chest Xray ,ECG ,Echocardiography, Catheterisation, MRI/Biopsy

Question 56

What is the management for restrictive cardiomyopathy?

Answer 56

Heart failure medication Guideline-directed medical therapy for heart failure, including angiotensin-converting enzyme inhibitors or angiotensin receptor II blockers, diuretics and aldosterone inhibitors should be initiated in patients with reduced LV Antiarrhythmic Therapy Immunosuppression- Steroids Pacemaker Cardiac transplantation