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Flashcards in Cardiomyopathy Deck (50)
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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

1. *Idiopathic*
2. Toxin induced (alcohol, anthracycline, cobalt, catecholamines)
3. Radiation
4. Infection (viral, HIV, parasite)
5. Metabolic (starvation, thiamine deficiency like beriberi, thyrotoxicosis)
6. Sarcoidosis
7. Hemochromatosis
8. Peripartum/postpartum
9. 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

1. Pulmonary congestion-left heart failure: dyspnea (rest, excertional, nocturnal), orthopnea

2. Systemic congestion- right heart failure: edema, nausea, abdominal pain, nocturia

3. Hypotension (low CO), tachycardia, tachypnea, fatigue and weakness

4. 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



shows enlarged heart, CHF


Treatment for dilated cardiomyopathy

-Limit activity based on functional status
-Salt restriction
-Fluid restriction
-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

-positive inotropic agents
volume depletion

Anything that decreases filling:
**sudden assumption of upright posture
**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


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)


Recommendation when there is a family history of HCM

Serial ECHOs are recommended up to the age of 20 yr where there is a family history of HCM

-LVH usually develops between 5-15 y/o


Accounts for 36% of deaths in



Management of HCM

-careful family history focused on sudden cardiac death
-counseling regarding avoidance of strenuous exercise, avoidance of dehydration
-all first-degree family members should be periodically screened with an echo


HCM treatment

1. FIRST- Beta blockers
-Anti-arrhythmics – Amiodarone, disopyramide
-Pacemakers (ICD) / Defibrillators (AICD)
***Myomectomy (resection of septum)
***Alcohol septal ablation (controlled MI through septal perforator perfusing basal septum), leads to wall thinning which decreases LV outflow obstruction


Beta blockers help HMC by

*NEGATIVE inotropic effect decreases outflow gradient
-decreased myocardial demand results in reduced
-prolonged diastolic filling time results in improved LV
filling as well as improved coronary perfusion
-may have an antiarrhythmic effect


IN HCM, usually, signs and symptoms _____ with
Leading cause of death: _____________


sudden death in asymptomatic patients