Cardiomyopathies Flashcards

1
Q

Reduction in the contractile function of the ventricles in the absence of pressure overload, volume overload, & CAD is known as?

A

dilated cardiomyopathy -> HF

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2
Q

What are the primary causes of dilated cardiomyopathy?

A
  • idiopathic (50%)) -> could be familial

- genetic predisposition -> mutations of TTN gene (titin) & MYH7 (b-myosin heavy chain)

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3
Q

What are the secondary causes of dilated cardiomyopathy?

A
  • peripartum cardiomyopathy
  • infection (coxackie, chagas)
  • SLE
  • valvular heart disease
  • radiation
  • alcohol, cocaine, cardio toxic drugs (doxorubicin, daunorubicin, & trastuzumab)
  • malnutrition
  • chronic tachycardia
  • endocrinopathies
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4
Q

What is the pathophysiology of dilated cardiomyopathy?

A

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

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5
Q

What are the clinical features of dilated cardiomyopathy?

A

GENERAL

  • exertional dyspnea
  • ankle edema & ascites
  • angina pectoris
  • palpitations
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6
Q

What will be seen on physical examination of patient suffering dilated cardiomyopathy?

A
  • mitral & tricuspid valve regurgitation (systolic murmur)
  • S3 gallop
  • left ventricular impulse displacement
  • jugular venous distention
  • rales over both lung fields
  • diffuse abdominal & peripheral edema
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7
Q

What will be your approach towards a patient with dilated cardiomyopathy?

A

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)

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8
Q

What will the ECHO of a patient with dilated cardiomyopathy show?

A
  • LV cavity size -> increased
  • EF -> significantly decreased
  • Wall thickness -> normal or decreased
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9
Q

How should dilated cardiomyopathy be managed?

A
  • treat underlying cause
  • treat HF
  • surgical treatment -> LVEF <35% -> automatic implantable cardioverter-defibrillator (AICD)
    - > if medical treatment fails -> heart transplant
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10
Q

Genetic hypertrophy of the left ventricle (not caused by other cardiac or systemic diseases) will lead to?

A

hypertrophic cardiomyopathy

  • > HOCM (70%)
  • > HCM (30%)
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11
Q

What is the most common cause of cardiac sudden death in young athletes?

A

hypertrophic cardiomyopathy (HCM) -> autosomal dominant trait

  • myosin binding protein C
  • B-myosin heavy chain
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12
Q

What is the pathophysiology of hypertrophic cardiomyopathy?

A

1- concentric hypertrophy of left ventricle (including interventricular septum)
2- decreased ventricular relaxation
3- decreased diastolic filling & systolic output
4- decrease myocardial & peripheral perfusion

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13
Q

What is the pathophysiology of HOCM?

A

left ventricular outflow tract obstruction & mitral regurgitation

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14
Q

What are the clinical features of hypertrophic cardiomyopathy?

A
  • 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
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15
Q

What is the best initial & confirmatory test for hypertrophic cardiomyopathy?

A

ECHO

  • LV cavity size -> decreased
  • EF -> normal
  • wall thickness -> increased
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16
Q

How should we manage patients diagnosed with hypertrophic cardiomyopathy?

A

ALL PATIENTS

  • avoid dehydration & alcohol & extreme exercise or anything causing vasodilation
  • healthy body weight
  • recommend in high risk patients -> AICD for primary & secondary prevention of sudden cardiac death

SYMPTOMATIC PATIENTS

  • first-line -> beta-blockers
  • second-line -> nondihydropyridine CCB
  • surgery -> septal ablation or myomectomy OR heart transplant
17
Q

Myocardial distortion due to proliferation of abnormal tissue or deposition of abnormal compounds will cause?

A

Restrictive cardiomyopathy -> marked diastolic dysfunction -> HF in over 80%

18
Q

What are the causes of restrictive cardiomyopathy?

A
  • amyloidosis
  • sarcoidosis
  • hereditary hemochromatosis
  • congenital enzyme abnormalities (Gaucher’s disease)
  • hypereosinophilia (Loffler endocarditis)
  • endocardial fibroelastosis
  • radiotherapy
  • cardiac surgery
19
Q

What is the pathophysiology of restrictive endocarditis?

A

1- infiltration or proliferation of connective or fibrotic tissue
2- decrease elasticity of myocardium
3- decreased ventricular compliance -> severe diastolic dysfunction
4- decrease ventricular filling in diastole
5- increase left & right sided filling pressures
6- increase in atrium size
7- increase pulmonary & systemic venous congestion

20
Q

What are the clinical features of restrictive cardiomyopathy?

A
  • dyspnea on exertion
  • leg swelling
  • palpitations
  • syncope
  • sudden cardiac death
  • signs of heart failure
  • features of underlying disease
21
Q

What are the best initial & diagnostic tests for restrictive cardiomyopathy?

A

Initial -> echo
diagnostic -> cardiac MRI (CMR)

X-ray -> signs of pulmonary congestion & could reveal signs of underlying disease

22
Q

What will an ECHO show inn restrictive cardiomyopathy?

A
  • LV cavity size -> decreased
  • EF -> normal or increased
  • wall thickness -> increased
23
Q

How should restrictive cardiomyopathy be treated?

A
  • symptomatic treatment of HF
  • screen & treat associated diseases
  • treat underlying cause
  • heart transplant incase of refractory symptoms