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Flashcards in Cardiomyopathy Deck (24)
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1

Pathophys features of Hypertrophic Cardiomyopathy (6)

1. Massive septal hypertrophy and small left ventricle
2. Left ventricular outflow tract obstruction with
3. Systolic anterior mitral motion (mitral regurge)
4. Diastolic dysfunction (heart can't relax)
5. Myocardial Fibrosis
6. Microvascular ischemia >> Arrhythmias

2

Autosomal dominant transmission, gene mutations coding for contractile proteins of cardiac sarcomere. Most common cause of sudden cardiac death in patients < 30 years. Apical variety more common in Chinese and Japanese.

Hypertrophic CM

3

Clinical presentation of Hypertrophic CM

1. Most common, DYSPNEA and ANGINA
2. Syncope and arrhythmias also common
3. May be asymptomatic or present as sudden death

4

Physical Exam findings of Hypertrophic CM

1. Systolic murmur from LVOT obstruction or Mitral Regurge

(Hypertrophic Heart findings):
2. Sustained PMI or triple apical impulse
3. Loud S4 gallop
4. Bisferiens carotid pulse

5

A pulse marked by two systolic peaks on the pulse waveform. It is characteristic of aortic regurgitation (with or without aortic stenosis) and hypertrophic cardiomyopathy.

Bisferiens carotid pulse

6

EKC abnormalities in Hypertrophic CM

1. Nonspeciic ST - T wave changes
2. Exagerrated septal Q waves
3. Left ventricular hypertrophy (axis)

7

Key diagnostic study for Hypertrophic CM

Echocardiography

1. LVH
2. Asymmetric septal hypertrophy
3. Small Left ventricle
4. Diastolic dysfunction

8

Hypertrophic CM treatment (4)

GOAL: TREAT SYMPTOMS, PREVENT SUDDEN DEATH

1. Initial treatment: BETA or Ca+ CHANNEL BLOCKERS

2. Persistant exertional dyspnea / chest pain:
DISOPYRAMIDE or AMIODARONE (anti arrhythmic, neg inotropic)

3. Severe medically refractory symptoms develop in ~5% of patients and require:
Alcohol Ablation or Surgical Myectomy of Septum

4. Dual chamber pacing, defibrillator implants,

MV replacement (Mitral valve repair or replacement is usually unnecessary as associated eccentric mitral regurgitation resolves with myectomy alone)

9

Causes of stroke and sudden death in Hypertrophic Cardiomyopathy

Sudden death: Ventricular Tachyarrhythmias

Stroke: Atrial Fibrillation

10

When an implantable defibrillator is indicated for patients with Hypertrophic Cardiomyopathy (5 risks)

Implantable cardioverter-defibrillator is advised for patients with two or more risk factors and is advised on a selected basis for patient with one risk factor.

1. History of cardiac arrest or arrhythmia
2. Family history of sudden death
3. Spontaneous ventricular tachy
4. LV thickness >30mm
5. Abnormal BP response to exercise / Syncope

11

Most common type of cardiomyopathy, associated with reduced strength of ventricular contraction. Most are genetic or idiopathic. Can also be from excessive alcohol consumption, postpartum state, chemotherapy, myocarditis. More common in what population?

(resulting in dilation of left ventricle )

Dilated Cardiomyopathy

More common in black men

12

Clinical features of Dilated Cardiomyopathy

Symptoms of CHF (left or biventricular)

1. DYSPNEA
2. S3 gallop
3. Pulmonary crackles (rales)
4. Increased JVP

13

Pathophys Features of Dilated Cardiomyopathy

1. Dilation and impaired contraction of ventricles (EF < 40%)

14

Diagnosis of Primary Dilated CM

1. Excluding coronary artery disease, valvular disease, and all other causes
2. Echo, stress test, cardiac cath, **endomyocardial biopsy**
3. Routine bloodworm for excluding nutritional deficiencies, endocrine disorders, inflammatory conditions, etc

15

Treatment of Dilated CM

1. Abstinence of alcohol is essential
2. Treatment of underlying disease
3. CHF support

16

Peripartum cardiomyopathy

Obese, multiparous black females. Present with heart failure in last trimester or first 6 months after delivery. Subsequent pregnancies are high risk.

17

A young to middle-aged adult who develops progressive dyspnea and weakness within a few days to weeks after a viral syndrome that was accompanied by fever and myalgias.

Acute Viral Myocarditis

18

Diagnosis of myocarditis, aside from typical cardiac workup

MRI, shows evidence of increased tissue edema

An increase in circulating viral titers between acute and convalescent blood samples supports a diagnosis of acute viral myocarditis with potential spontaneous improvement.

19

Most common cause of infective cardiomyopathy

CHAGAS (T. cruzi)

20

Results from fibrosis or infiltration of ventricular or atrial walls due to collagen defects, AMYLOIDOSIS, radiation, diabetes, endomyocardial fibrosis

Restrictive Cardiomyopathy

21

Pathophys features of Restrictive CM

1. Left ventricle is small or normal, with mildly reduced function
2. Both atria enlarge
3. Restrictive ventricular filling ( = high ventricular filling pressures)

22

Clinical features of Restrictive CM

1. Patients present first with decreased exercise tolerance, later with right-side CHF
2. Pulmonary HTN
3. Low pulse volume due to reduced stroke volume and tachycardia

**must be differentiated from constrictive pericarditis**

23

Diagnosis of Restrictive CM

1. CXR shows mild-moderate enlarged cardiac silhouette

2. Echo is key - showing thickened walls of both ventricles without major chamber dilation. The atria are markedly dilated, consistent with chronically elevated ventricular filling pressures

4. **Endomyocardial biopsy may be needed to differentiate from constrictive pericarditis**

24

Treatment of Restrictive CM

1. Symptoms of right CHF - Diuretics
2. Underlying amyloidosis (WBC disorder)

*Transplant contraindicated w amyloidosis / blood disorders**