Cardiomyopathies Flashcards
Reduction in the contractile function of the ventricles in the absence of pressure overload, volume overload, & CAD is known as?
dilated cardiomyopathy -> HF
What are the primary causes of dilated cardiomyopathy?
- idiopathic (50%)) -> could be familial
- genetic predisposition -> mutations of TTN gene (titin) & MYH7 (b-myosin heavy chain)
What are the secondary causes of dilated cardiomyopathy?
- peripartum cardiomyopathy
- infection (coxackie, chagas)
- SLE
- valvular heart disease
- radiation
- alcohol, cocaine, cardio toxic drugs (doxorubicin, daunorubicin, & trastuzumab)
- malnutrition
- chronic tachycardia
- endocrinopathies
What is the pathophysiology of dilated cardiomyopathy?
1- causative factors decrease contractility of the myocardium
2- compensation to maintain cardiac output
3- eccentric hypertrophy -> dilation of the ventricle
4- systolic dysfunction -> decreased ejection fraction -> HF
What are the clinical features of dilated cardiomyopathy?
GENERAL
- exertional dyspnea
- ankle edema & ascites
- angina pectoris
- palpitations
What will be seen on physical examination of patient suffering dilated cardiomyopathy?
- mitral & tricuspid valve regurgitation (systolic murmur)
- S3 gallop
- left ventricular impulse displacement
- jugular venous distention
- rales over both lung fields
- diffuse abdominal & peripheral edema
What will be your approach towards a patient with dilated cardiomyopathy?
1- investigate underlying cause -> BNP (increased), troponin & CK-MB
2- assess cardiac function -> ECHO, ECG
3- assess structural remodeling -> ECHO, X-ray (signs of HF)
What will the ECHO of a patient with dilated cardiomyopathy show?
- LV cavity size -> increased
- EF -> significantly decreased
- Wall thickness -> normal or decreased
How should dilated cardiomyopathy be managed?
- treat underlying cause
- treat HF
- surgical treatment -> LVEF <35% -> automatic implantable cardioverter-defibrillator (AICD)
- > if medical treatment fails -> heart transplant
Genetic hypertrophy of the left ventricle (not caused by other cardiac or systemic diseases) will lead to?
hypertrophic cardiomyopathy
- > HOCM (70%)
- > HCM (30%)
What is the most common cause of cardiac sudden death in young athletes?
hypertrophic cardiomyopathy (HCM) -> autosomal dominant trait
- myosin binding protein C
- B-myosin heavy chain
What is the pathophysiology of hypertrophic cardiomyopathy?
1- concentric hypertrophy of left ventricle (including interventricular septum)
2- decreased ventricular relaxation
3- decreased diastolic filling & systolic output
4- decrease myocardial & peripheral perfusion
What is the pathophysiology of HOCM?
left ventricular outflow tract obstruction & mitral regurgitation
What are the clinical features of hypertrophic cardiomyopathy?
- usually asymptomatic (esp non-obstructive)
- symptoms worsen with exercise, dehydration, diuretics, ACEIs/ARBs, digoxin
- exertional dyspnea
- angina pectoris
- dizziness, lightheadedness, syncope
- palpitations, cardiac arrhythmias
- sudden cardiac death after intense physical activity
- systolic ejection murmur (crescendo-decrescendo)
- > increases with valsalva maneuver, standing
- > decreases with hand grip, squatting, leg elevation
- sustained apex beat
- S4 gallop
- pulsus bisferiens
What is the best initial & confirmatory test for hypertrophic cardiomyopathy?
ECHO
- LV cavity size -> decreased
- EF -> normal
- wall thickness -> increased