Cardiomyopathy Flashcards
Case presentation
19 y/o Male, presents to your office with complaints of palpitations and occasional chest pain especially when he has been practicing for his football team
Fam Hx: Uncle passed away at age 25, Cousin has heart problem
Definition of cardiomyopathy
a heterogeneous group of diseases of the myocardium associated with mechanical &/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are a part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability
Dilated cardiomyopathy
Dilatation and impaired contraction of the left or both ventricles (chamber enlargement)
- Ventricular dilation, hypokinetic left ventricle, and systolic dysfunction
- can be inherited
- congestive presentation
Hypertrophic cardiomyopathy
Left and/or right ventricular hypertrophy, often asymmetrical, which usually involves the interventricular septum.
-Inappropriate myocardial hypertrophy, with or without left ventricular obstruction
Restrictive cardiomyopathy
Restricted filling and reduced diastolic size of either or both ventricles with normal or near-normal systolic function.
-Abnormal ventricular filling with diastolic dysfunction
Arrhythmogenic right ventricular cardiomyopathy
Progressive fibro-fatty replacement of the right ventricle
-Fibroadipose replacement of right ventricle
Common causes of Dilated Cardiomyopathy
- Idiopathic
- Toxin induced (alcohol, anthracycline, cobalt, catecholamines)
- Radiation
- Infection (viral, HIV, parasite)
- Metabolic (starvation, thiamine deficiency like beriberi, thyrotoxicosis)
- Sarcoidosis
- Hemochromatosis
- Peripartum/postpartum
- Genetic
Dilated cardiomyopathy incidence
- most common of all
- myocyte injury and fibrosis
- Third most common cause of heart failure
- Middle age, men > women
- Annual mortality: 12% !
- Most frequent cause of heart transplantation
- complete recovery is rare
S/S of Dilated cardiomyopathy
- Pulmonary congestion-left heart failure: dyspnea (rest, excertional, nocturnal), orthopnea
- Systemic congestion- right heart failure: edema, nausea, abdominal pain, nocturia
- Hypotension (low CO), tachycardia, tachypnea, fatigue and weakness
- Arrhythmia
Atrial fibrillation, conduction delays, complex PVC’s, sudden death
During dilated cardiomyopathy cardiac exam shows
S3, S4 and murmur of TR and/or MR
CXR
shows enlarged heart, CHF
Treatment for dilated cardiomyopathy
- Limit activity based on functional status
- Salt restriction
- Fluid restriction
- Diuretics
- Beta Blockers, ACE/ARB (improve mortality)
- Hydralazine/nitrate combination if cannot tolerate ACE/ARB
- Spironolactone if EF
Dilated cardiomyopathy is the most common indication for ________
cardiac transplantation
Survival after transplant is
80% one year
70% 5 years
-or can do left vent reshaping
People with Hypertrophic Cardiomyopathy (HCM) also have:
- Small LV cavity
- septal hypertrophy (huge)
- systolic anterior motion of the mitral valve leaflet +/- obstruction of left ventricular outflow
- low stroke volume
- elevated EF
Usually patients with Hypertrophic Cardiomyopathy (HCM) are
very young
may lead to MI, mitral regurgitation or arrhythmias
What type of dysfunction is Hypertrophic Cardiomyopathy and what does it lead to?
Diastolic dysfunction (impaired filling, increased filling pressure)
Leads to pulmonary congestion (have LV outflow obstruction and hyper dynamic systolic function bc of so much muscle, especially septum)
“Classic” feature of hypertrophic cardiomyopathy is
Dynamic left ventricular outflow tract obstruction
absent in about half of people and obstruction varies greatly
The severity of LV outflow obstruction increases with
hypertrophic obstructive= HOCM
**exercise
-positive inotropic agents
volume depletion
Anything that decreases filling:
- *sudden assumption of upright posture
- tachycardia
- *Valsalva maneuver
S/S of hypertrophic cardiomyopathy
- Asymptomatic (Echo finding only)
- Dyspnea in 90%
- Angina pectoris in 75% (ISCHEMIC CHEST PAIN)
- Fatigue, pre-syncope, SYNCOPE (incr. risk of SCD)
- Palpitation, PND, CHF, dizziness
- Atrial fibrillation, thromboembolism
- SUDDEN CARDIAC DEATH
In HCM, ejection murmur is heard at
left sternal border
-does NOT radiate to the carotid arteries
HCM, the EKG will show
*LVH (Strain pattern) and *abnormal Q waves
may see giant T wave inversions or LA enlargement, bbb or arrhythmias
In HCM, the valsava will
decrease LV volume and INCREASE THE MURMUR
-standing will do this too
In HCM, squatting will
increase LV volume and DECREASE THE MURMUR
Echo findings in HCM
- Echocardiogram is best test
- Left ventricular hypertrophy > 1.3 cm
- Septal to posterior wall ratio > 1.3:1
- Mitral regurgitation
- Systolic anterior motion of the mitral valve (SAM)
- Premature midsystolic closure of the aortic valve
- Asymmetric septal hypertrophy (ASH)
- Diastolic dysfunction
- Left ventricular outflow tract obstruction (LVOT)
