Valvular Disease, HF, Cardiomyopathies Flashcards

Question

Aortic Insufficiency Causes * Infective \_\_\_\_\_\_ * Traumatic/non-traumatic _______ of ascending aorta (aortic cusp prolapse) * (1) processes: * SLE, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, Takayasu vasculitis, Marfan/Ehlers-Danlos syndromes, Behcet disease * _______ AI causes: * infective endocarditis, myxomatous valve degermation, bicuspid AV, AV calcification, aortic dissection, Whipple disease, Chrohn disease, systemic HTN * T\_\_\_\_\_ Syndrome

Answer 1

* Infective endocarditis * Traumatic/non-traumatic rupture of ascending aorta (aortic cusp prolapse) * Rheumatologic processes: * SLE, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, Takayasu vasculitis, Marfan/Ehlers-Danlos syndromes, Behcet disease * Chronic AI causes: * infective endocarditis, myxomatous valve degermation, bicuspid AV, AV calcification, aortic dissection, Whipple disease, Chrohn disease, systemic HTN * Turner Syndrome

Answer 2

Common Leads to LVOT Obstruction * LV obstruction caused by AS increases LV systolic pressure and LV ejection time , and LV end-diastolic pressure. * AS decreases aortic pressure * Increased afterload/volume overload \>LV dysfunction/failure * Myocardial oxygen consumption increases with increased LV systolic pressure/LVET

Answer 3

* Congenital (bicuspid/unicuspid) leaflet deformities * Bicuspid disease most common cause for pts \< 70 * Calcification * Rheumatic disease (most common cause in developing countries) * SLE * Radiation of chest * Homozygous Type II Lipoproteinemia * Fabry Disease * Hypertrophic cardiomyopathy (subvalvular stenosis)

Answer 4

Ventral Septal Defect

Answer 5

Aortic Stenosis (AS)

Answer 6

Mitral valve prolapse (MVP)

Answer 7

Pulmonic Stenosis (PS)

Answer 8

Tricuspid regurgitation (TR)

Answer 9

Hypertrophic Cardiomyopathy (HCM)

Answer 10

Atrial septal defect (ASD)

Answer 11

Right sided heart failure 1. **Echo** 1. right atrium = dilated 2. right ventricle = dilated 3. right ventricle function = normal, hyperdynamic, or reduced 2. **CXR** 1. Cardiomegaly heart size with severe TR 2. Ascites/Pleural effusion 3. **Labs** 1. MAY see abnormal liver function, hyperbilirubinemia 2/2 to hepatic congestion

Answer 12

* Ascites * Jugular vein distension * S3 gallop (extremely dilated RV) * Pansystolic murmur * Jaundice * Atrial fibrillation * Peripheral edema * Cachexia

Answer 13

➢Diuretics ➢Antiarrhythmics ➢ARBs ➢Anticoagulants\*

Answer 14

Severe TR ➢ Isolated tricuspid valve replacement rare ➢Usually, will do along with left sided valve repair/replacement

Answer 15

**Murmur:** low frequency pre-systolic, mid-diastolic murmur: best heard at lower left sternal border 4th intercostal space * Lungs are clear in isolated TS * Reduced exertional capacity (exertional syncope as TS worsens), fatigue, leg edema, ascites, hepatic congestion, anasarca, and other signs of RV failure

Answer 16

* **ECG =** Tall, peaked p waves in II, III, and aVF (if pt is in sinus rhythm) * **Echo =** will show stenosis * **Cardiac MRI =** preferred over echo to evaluate RV

Answer 17

➢**Loop diuretics** (prevent/limit systemic and hepatic congestion in severe symptomatic TS). \*\*\*Caution\*\*\* ➢Not every case needs invasive intervention ➢ Appropriate treatment of underlying causes most helpful (i.e. SLE)

Answer 18

**Valvotomy** 1, 2, or 3 balloons used **Repair** preferred over Replacement * If repair cannot be done, open is preferred over transcatheter * No difference between bioprosthetic vs mechanical except with carcinoid syndrome * Best done in conjunction with left sided valve surgery

Answer 19

* Significant dyspnea at rest, exacerbated in supine position * Cough, with clear or pink, frothy sputum * May have chest pain radiating to neck, jaw, shoulders, etc. (sxs associated with myocardial ischemia) * Tachycardia OR bradycardia (if ischemic involvement of conduction system) * Tachypnea, hypoxemia, cyanosis, hypotension * JVD, diffuse crackles in lungs, * Apical holosystolic murmur with radiation to axilla

Answer 20

Typically related to papillary muscle rupture from ACS or fulminant destruction of valvular apparatus 2/2 to acute bacterial endocarditis

Answer 21

* May remain asymptomatic until late in course * Fatigue, dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea, weight gain, pulse pressure begins to widen, apical holosystolic murmur with radiation to axilla, dependent edema, JVD * Advanced cases: cyanosis, syncope/near syncope, digit clubbing, hepatomegaly, ascites with fluid wave, pleural and/or pericardial effusions

Answer 22

* **Echo:** primary and essential diagnostic test * **CXR**: With chronic MR, cardiomegaly 2/2 to left atrial and/or right sided heart enlargement * **ECG**: Atrial fibrillation is most common ECG finding in pts with MR * **Cardiac MRI:** Helps assess severity of MR, also helps with RV and LV size/function

Answer 23

➢ACE/ARB ➢ Beta blockers: not useful in primary MR but have been shown to help in secondary MR ➢Loop diuretics

Answer 24

Repair preferred over replacement when possible except when there is extensive tissue destruction or infective endocarditis ➢Mechanical preferred over bioprosthetic

Answer 25

* 1st heart sound usually loud and may be palpable 2/2 to force with MV closing * P2 (pulmonic) part of 2nd heart sound will be loud if severe pulmonary HTN is cause of MS * Mid-diastolic rumbling murmur with pre-systolic accentuation heard (low pitch). Best heard with stethoscope bell at apex

Answer 26

* Orthopnea and paroxysmal nocturnal dyspnea * May have palpitations, chest pain, and hemoptysis (left atrial volume increased) * Ascites, edema, and hepatomegaly (right-sided heart failure) 41 Mitral Stenosis

Answer 27

Presents 20-40 years after episode of rheumatic fever **Echo** most useful If echo does not reveal enough detail, **cardiac catheterization** is favored diagnostic

Answer 28

➢No medical therapy can relieve fixed obstruction ➢ Medical therapy focuses on preventing endocarditis, decreasing new cases of rheumatic fever, thromboembolic risk, etc.

Answer 29

➢Mitral balloon valvuloplasty ➢Mitral valve replacement if valvuloplasty not possible

Answer 30

S3, diastolic decrescendo murmur (2nd/3rd intercostal spaces) increases during inspiration and decreases with Valsalva maneuver

Answer 31

* Most patients are asymptomatic * Exertional dyspnea (because of decreased cardiac output 2/2 volume overload) * Decreased exercise tolerance * Severe PR: Right heart failure: peripheral (esp pedal) edema, congestive hepatomegaly, and rarely jugular vein distension

Answer 32

* **Echo**: Best way to detect * CXR will not really yield anything * Exercise stress ECG: Used to assess risks associated with progressive RV dilation

Answer 33

No treatment recommended unless at least 2**/**4 criteria are met then surgery is recommended 1. Mid or moderate RV or LV systolic dysfunction 2. Severe RV dilation (RV end diastolic volume \< 160 ml/m2) 3. RV ventricular systolic pressure due to RV outflow tract \< 2/3 of systemic pressure 4. Progressively reduced exercise tolerance

Answer 34

* Requires significant provider diagnostic skill * Parasternal heave * Left upper sternal border-systolic ejection murmur that radiates to the back * May hear 4th heart sound and tricuspid regurgitation

Answer 35

Most patients are asymptomatic * Those who are symptomatic have: * Dyspnea on exertion and fatigue * Rarely-angina/sudden cardiac arrest * Associated septal defect: cyanosis

Answer 36

**Echo**: Best way to detect CXR will not really yield anything

Answer 37

➢Asymptomatic: Follow up with echos ➢Symptomatic: Balloon valvotomy for doppler gradient \> 500 mm hg and domed PV ➢Surgical intervention for severe pulmonary stenosis

Answer 38

* History is extremely important! * Those with acute AI can present as if they are experiencing a rapid onset of cardiogenic shock * Cough, dyspnea on exertion, chest pain, and palpitations common

Answer 39

* **Diastolic murmurs**: Best heard at 3rd or 4th intercostal space at left sternal border * **Austin Flint Murmur:** Mimics mitral stenosis-low-pitched and rumbling (mid/late diastolic) ….best heard at apex. Caused by regurgitant jet of AI * **Pulsus bisferiens**: biphasic pulse with 2 systolic peaks seen on pressure tracings

Answer 40

* **Corrigan sign**: Bounding pulse in large arteries (e.g. carotids) that expands and suddenly collapses and empties between beats * **De Musset Sign**: Head nodding with every heartbeat * **Muller Sign**: Systolic pulsation of uvula * **Quincke Sign**: Repeatedly alternating blushing/blanching of capillaries in nail folds when palpated

Answer 41

* **Traube’s Sign**: Pistol shot sounds heard on femoral artery when artery compressed * **Waterhammer Pulse**: Peripheral pulses are either rapidly faint or bounding. * **Chronic AI**: Widened pulse pressure * May have laterally/inferiorly displaced apical impulse

Answer 42

* Echocardiogram gold standard * Cardiac MRI: Class I indication if echo does not provide enough information * ECG: Nonspecific ST-T wave changes may be noted * Signs of LVH

Answer 43

HTN Management * ACE, ARBs, Calcium Channel Blockers * β-blockers for aortic dissection BB: Avoid for severe AI dt Increase diastolic filling time * Allow for regurgitant blood flow * Block compensatory tachycardia that is needed

Answer 44

Aortic Valve Replacement * Stage D AI or Stage C AI with LVEF \< 50% or needing cardiac surgery for other reason * Not recommended for pts with LVEF \<35% in most cases because of poor postoperative outcomes Transcatheter Aortic Valve Replacement * appropriate in high-risk patients depending on causal factors

Answer 45

* **Tri-leaflet calcific stenosis** = Present after age 70 * **Bicuspid aortic valve** = Begin presenting between ages 50-70

Answer 46

* Cardiac * Exertional dyspnea, angina, fatigue, paroxysmal nocturnal dyspnea, orthopnea, and progressively decreasing exercise tolerance * Non-cardiac: * Cerebral perfusion issues: start with mild memory/cognitive deficits then progresses to pre-syncope –syncope * GI bleeds, cerebral emboli

Answer 47

* Pulsus parvus et tardus * Slow-rising, late-peaking and low amplitude carotid impulse in severe AS * 2nd heart sound may lack a split * Systolic ejection murmur * Late peaking * Heard best at heart base and radiates to carotids

Answer 48

Gold Standard = Echo Cardiac MRI = good to assess LV mass function and volume (if not available from echo)

Answer 49

➢Aortic valve replacement (AVR) superior to medical therapy in severe symptomatic AS ➢Cautious management of HTN in severe AS because of fixed afterload ➢Cautious use of diuretics can help decrease congestion

Answer 50

➢**AVR** indicated in pts with heart failure and volume overload and all patients who are symptomatic, regardless of severity ➢AVR also indicated in asymptomatic pts with severe AS with LVEF \> 50% who undergo CABG or other cardiac surgery procedure ➢**TAVR** **(Transcatheter aortic valve replacement) has become gold standard for patients at high mortality risk** for standard sternal approach to aortic valve replacement ➢ \*\*Used to be common to give huge amounts of fluid after AVR because of dependence on preload. We now know that this brings on RV much quicker

Answer 51

* Inability of heart to keep up with the demands put on it * Inefficiency in pumping blood * Inability to provide adequate blood flow to other organs within the body * May be due to failure of right or left or both ventricles * Symptoms will depend on which side(s) is involved

Answer 52

Also known as systolic dysfunction/failure = **PUMP PROBLEM** * Represents ~ 50% of HF cases * Most common cause: CAD Other causes: Valvular disease, HTN, hyperthyroidism, myocardial infarction, age, diabetes mellitus,

Answer 53

Well established management algorithms established Characteristics * LV remodeling with dilation * Impaired contractility (pumping) * Thin LV

Answer 54

Also known as diastolic dysfunction/failure = **RELAXATION PROBLEM** * Expected to increase in prevalence because of aging population in U.S. * Common for pts with HFpEF to have higher rate of chronic comorbidities with about ½ having 5 or more of them * 3 most common characteristics: Female, older, obese

Answer 55

* HTN (60-89% prevalence) * obesity * CAD * diabetes mellitus * atrial fibrillation * hyperlipidemia * pulmonary HTN * anemia * CKD * COPD * frailty * obstructive sleep apnea

Answer 56

* **Borderline HFpEF is an EF of 41%-49%. These patients fall into a borderline or intermediate group.** * **Their characteristics, treatment patterns, and outcomes appear similar to those of patients with HFpEF**

Answer 57

* Age and gender may also play * Different roles in the prevalence of HFpEF vs. HFrEF * Prevalence increases overall for HFpEF and HFrEF with increasing age, with higher rates of HFpEF typically seen in older women

Answer 58

**F**atigue **A**ctivities, limited **C**ough **E**dema **S**hortness of breath

Answer 59

* Crackles/rales * JVD * Dyspnea/paroxysmal nocturnal dyspnea * Pulmonary edema * Weight gain * S3 gallop * Hepatojugular reflux + * Hepatomegaly * Cough at night * Ankle edema * Pleural effusions * Tachycardia

Answer 60

* CXR: cardiomegaly, pleural effusions, interstitial edema, alveolar edema, dilated prominent upper lobe vessels * ECG: Atrial abnormality, conduction abnormalities/arrhythmias**,** ventricular hypertrophy (early) * Echo: Used to differentiate systolic/diastolic dysfunction, reduced LVEF (\<45%), may have valvular malfunction, dilated LV with thin walls (later) * Labs: CBC (anemia), BMP (renal function, Na+ levels, electrolytes), magnesium, thyroid function tests (hyperthyroidism can bring on HF/hypothyroidism exacerbates), BNP

Answer 61

* **Functions of Natriuretic peptides (ANP and BNP)** 1. Downregulates sympathetic nervous system and RAAS 2. Facilitates natriuresis and diuresis 3. Increase smooth muscle relaxation and decrease peripheral resistance * **Uses in Heart Failure** 1. A negative result rules out disease more effectively than a positive result rules in disease 2. Predictor of prognosis 3. Monitoring

Answer 62

1. \<100 pg/ml: Heart failure unlikely 2. 100-250 pg/ml: Compensated heart failure 3. 250-500 pg/ml: Both diastolic and systolic dysfunction 4. 500-1000 pg/ml: Decompensated heart failure 5. \>1000 pg/ml: Increased risk of substantial heart failure

Answer 63

* CV Causes (80-85%) * Worsening HF * Cardiogenic shock * Low output state * ~40% of cases * Sudden Death * Ventricular tachyarrhythmia * Bradyarrhythmia * Non CV * Infection/sepsis * Renal * Respiratory * Malignancy

Answer 64

**B**eta blockers **A**CE Inhibitors/ARBs **N**itrates (dinitrate)/hydralazine **D**iuretics **A**ldosterone antagonist **I**vabradine **D**evices (AICD, CRT, or both) **D**igoxin

Answer 65

* Shortness of breath, especially with exertion * Edema, peripheral and abdominal * Severe fatigue

Answer 66

* Comparable to what is seen in HFrEF in many cases * Heart sounds: S4 heard just before S1 –representative of noncompliant LV (Sometimes, not necessarily a reliable sign * Physical Examination in most cases of heart failure will not really help you differentiate HFrEF vs HFpEF. An echocardiogram will help

Answer 67

* **Labs**: Same as with HFrEF * BNP is often just as (if not more) elevated in HFpEF as is seen in HFrEF * **Cardiac catheterization** –helps reveal increased ventricular diastolic pressure with preserved systolic function and normal ventricular volumes. Will also see increased left atrial and pulmonary capillary wedge pressures * **CXR**: May/may not have edema. Many have normal cardiac silhouette. * **Echo**: Used most often * Measures LVEF * Easily identifies LVH * Presence/absence of valvular heart disease * Left atrial size

Answer 68

**2 Major Objectives with HFpEF Management** 1. Reverse consequences of diastolic dysfunction (venous congestion) 2. Eliminate or reduce the factors responsible for diastolic dysfunction, fibrosis, and/or myocardial hypertrophy

Answer 69

**Patients with HFpEF Utilize More Health Care Resources than Patients with HFrEF**

Answer 70

More than 50% of HFpEF Patients May Die Within 5 Years

Answer 71

* Data indicate that patients with HFpEF have high rates of mortality, which increase after HF-related hospitalizations. * In-hospital mortality ranges from 2.4% to 4.9% in observational studies * Death rate is 5% within 30 days * Death rate is 9.5% within 60-90 days * At 1-year post-hospitalization, 20%-29% of patients may die • Prior hospitalization can increase mortality rates * Within 5 years, at least 50% of patients may die, with published mortality estimates ranging from 53% to 74%

Answer 72

100% likelihood of diastolic dysfunction with **concentric LVH** Eccentric LVH = “volume overload” mitral regurg, athlete Concentric LVH = “pressure overload”, chronic HTN or aortic stenosis

Answer 73

**Cardiomyopathies**

Answer 74

**Dilated** **Hypertrophic** **Restrictive** * Peripartum * Arrhythmogenic RV Dysplasia * LV Non-compaction * Transthyretin Amyloid

Answer 75

**Dilated Cardiomyopathy**

Answer 76

Most are idiopathic * Viruses * Familial (genetic) * Valvular * ETOH * Illicit/toxic drug use * DM * Thyroid disease

Answer 77

LV is enlarged, dilated, and weak → pump fails Often asymptomatic until advanced then comparable to HFrEF * *Primary CM** = when diseased myocardium develops without obvious cause * *Secondary CM** = develops because of another cause

Answer 78

➢**CXR**: Cardiomegaly, pleural effusions ➢**Echo**: LV dilation with normal/thinned walls and reduced EF ➢**Cardiac Cath**: done to exclude ischemic disease ➢**Cardiac biopsy** can help differentiate cause ➢**EKG**: atrial fibrillation, LAE, ventricular arrythmias common ➢**Genetic Testing**: TTN mutations

Answer 79

**Similar to HFrEF** * ACE/ARB * Beta blockers * Diuretics * Low Na+ diet * Anticoagulants **Devices** * Cardiac resynchronization therapy * AICD **Surgery** * Transplant

Answer 80

**Hypertrophic Cardiomyopathy**

Answer 81

* Other causes: Fabry disease, Friedreich’s ataxia, meds like tacrolimus * Leading cause of sudden death in young athletes in the US * Italy: huge issues → began screening in teen athletes (*high prevalence in greek/italian)* * Mortality for HCM now \<1% therefore large scale screening not done in US, UK, or Canada, does continue in Italy

Answer 82

Many live entire life asymptomatic, however HCM also major cause of sudden cardiac death Myocardial walls become thickened without an obvious cause * Interventricular septum and ventricles most often affected * Causes heart to be able to pump blood effectively * Can also cause conduction/arrhythmia issues

Answer 83

* Leg edema * Shortness of breath * Chest pain * Syncope * Atrial and ventricular arrhythmias * Heart failure * Sudden cardiac death

Answer 84

* Septum thickens * LV walls become stiff * Septum or LV wall may reduce/block blood from LV to aorta * Systolic anterior motion (SAM) of mitral valve can occur with HOCM * Displacement of the distal portion of the anterior leaflet of MV toward the LVOT obstruction * 31-61% prevalence in HCOM * those with SAM, 25-50% have resting LVOT obstruction *(SAM increases risk of sudden cardiac death)*

Answer 85

* LV stiff * Blood flow not blocked

Answer 86

**ECG**: May see signs of LVH **Echo**: 80% accuracy **Cardiac MRI**: Gold standard * Really useful to see segmental lateral ventricular hypertrophy * LVH may not be seen in children under age 13

Answer 87

**Must stay hydrated!** **Β-blockers****:** 1st line agents, Increases diastolic filling, Decreases contractility **Calcium channel blockers:** Extreme caution should be used, May cause increased mortality in pts with severe outflow obstruction **Disopyramide**: Reduces LV contractility and subaortic gradient, Reduces afterload, Slows LV ejection acceleration **Diuretics** (\*Rarely used)

Answer 88

**Surgical Myectomy** **Alcohol Ablation** ASA similar mortality but 1/10 need pacemaker vs 1 /25 myectomy, 1/13 needs reintervention

Answer 89

**Implantable pacemaker/defibrillator** * Used to cause asynchronous contraction of LV * For pts who have one of the major risk factors for sudden cardiac death, an AICD or PPM/AICD may be recommended **MitraClip** * Used to correct motion of the mitral valve in those with severe obstructive HCM * Not established as long-term reliability of myectomy or alcohol septal ablation but being used more often to give option **Transplantation** * For end-stage HF * Must occur before pulmonary HTN, significant kidney disease, and/or thromboembolism occur for transplant to be successful

Answer 90

**Most common cause-Amyloidosis** **Genetic** * DES, CRYAB, FLNC mutations **Non Infiltrative** * Idiopathic, Diabetic CM, Scleroderma, myofibrillar myopathies, pseudoxanthoma elasticum, Werner’s syndrome **Infiltrative** * Amyloidosis, sarcoidosis, primary hyperoxaluria **Storage Diseases** * Carcinoid heart disease, endomyocardial fibrosis, idiopathic, hypereosinophilic syndrome, chronic eosinophilic leukemia, endocardial fibroelastosis, radiation, and meds (serotonin, methysergide, ergotamine, mercurial agents, anthracyclines, busulfan

Answer 91

* Decreased exercise tolerance * Fatigue * JVD * Peripheral edema * Ascites * Arrhythmias and conduction blocks

Answer 92

* Bi-atrial enlargement * Thickened LV walls with normal chamber size * Thickened RV free wall with normal chamber size * Elevated right atrial pressure (\> 12 mm Hg) * Moderate pulmonary HTN * Normal systolic function * Poor diastolic function (Grade II-IV diastolic HF)

Answer 93

Restrictive Cardiomyopathy Cardiac walls are rigid, not thickened * Heart restricted from stretching and filling with blood * Rhythmicity and contractility may be normal, but the stiff walls of atria/ventricles cause reduced preload and end-diastolic volume * Over time, they develop diastolic dysfunction and eventually diastolic heart failure Not the same as constrictive pericarditis = Very similar presentation but markedly different treatment and prognosis

Answer 94

1. Treatment of conditions causing issues (example: sarcoidosis → give corticosteroids) and slowing the progression of CM 2. Na+ restricted diet, diuretics, ACE inhibitors, and anticoagulants may be helpful 3. Calcium channel blockers-: Contraindicated 2/2 to negative inotropic effect, especially if amyloidosis is underlying cause 4. Digoxin and beta-adrenergic meds: generally, not helpful except with RCM with atrial fibrillation 5. Vasodilators usually ineffective because systolic function is normal 6. **Most common treatment: Transplantation or LVAD until transplantation can occur**

Valvular Disease, HF, Cardiomyopathies Flashcards

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