Cardiomyopathy
Structural/functional abnormality of heart muscle in the absence of:
- CAD
- HTN
- Valvular disease
- Congenital heart disease
What are the three main structural/functional categories
- Dilated
- Hypertrophic
- Restrictive
What is the most common cardiomyopathy
Dilated
Define Dilated cardiomyopathy
Thin, enlarged ventricular wall, systolic dysfunction
Define hypertrophic cardiomyopathy
Myocardial hypertrophy (thick muscle) in the absence of HTN or aortic stenosis
Define restrictive cardiomyopathy
Nondilated ventricles, impaired filling, reduced diastolic function
Define Heart Failure
- Results from any structural or functional cardiovascular abnormality causing a supply/demand mismatch
- With Systemic perfusion being inadequate to meet the body’s metabolic demand
What is the main cause of dilated cardiomyopathy?
Idiopathic
What are the other non-idiopathic causes for dilated cardiomyopathy?
"ABCD PIG": Alcohol Beriberi (thiamine deficiency) Coxsackie B, Chagas disease Drugs: adriamycin (anthracycline), cocaine Pregnancy Idiopathic, infection Genetic
What is the most common cause of heart failure due to systolic dysfunction?
Ischemic cardiomyopathy
What is a common cause of dilated cardiomyopathy?
Ischemic cardiomyopathy
How is Ischemic cardiomyopathy characterized?
LVEF≤35-40% FROM coronary artery disease
-often occurs after MI
Treatment for ischemic cardiomyopathy
ASA High-intensity statin Beta-blocker ACE-inhibitor Loop diuretic if fluid overload
How is hypertensive cardiomyopathy characterized?
Concentric LVH
What can lead to hypertensive cardiomyopathy?
Uncontrolled and sustained HTN over a long time period
Define alcoholic cardiomyopathy
Excessive alcohol use leads to myocardial dysfunction
Who is at risk for alcoholic cardiomyopathy?
> 90g (7-8 drinks) per day for at least 5 years
When would you see a prolonged QTc (precursor to ventricular arrhythmias)
Alcoholic cardiomyopathy
Define Peripartum Cardiomyopathy
- Development of heart failure late in pregnancy or within 5 months of giving birth
- LVEF<45% with or without dilation
- Most common w/in 1 month postpartum
What are the risk factors for Peripartum Cardiomyopathy?
- age>30
- African descent
- Cocaine abuse
- Multiple fetuses
- Preeclampsia/eclampsia
What is the treatment for Peripartum Cardiomyopathy?
Heart transplant performed in 1/3
Define Takotsubo Cardiomyopathy
- “Stress Cardiomyopathy”
- ->Catecholamine induced
- Characterized by transient LV dysfunction that appears as systolic apical ballooning
Sx’s of Takotsubo Cardiomyopathy
- Substernal chest pain
- Troponin levels often 7x the upper limit of normal
- ECG with ST-elevation is common
- Echocardiography shows apical ballooning pattern, decreased LVEF
What age group does dilated cardiomyopathies occur in?
Age 20-60
Physical exam findings in dilated cardiomyopathy- Vitals
- Hypotensive
- Tachycardic (cardiogenic shock)
- Tachypnea
Signs of left sided HF in dilated cardiomyopathy
Pulmonary congestion:
- Productive cough
- Dyspnea (at rest, exertional, lying flat)
- Crackles or wheezing
Signs of right sided HF in dilated cardiomyopathy
- JVD
- Hepatojugular reflux
- Peripheral edema (pitting)
Diagnostic findings on a CXR in dilated cardiomyopathy
- Enlargement of cardiac silhouette
- Pulmonary vascular congestion
- Pleural effusion (R sided)
- Kerley B lines
Diagnostic findings on an EKG in dilated cardiomyopathy
- LVH
- Conduction delay
- Arrhythmias
What are the effects of ACE inhibitors in treating dilated cardiomyopathy
-Reduce preload and afterload by vasodilation–> BP reduction
What are the effects of Beta Blockers in treating dilated cardiomyopathy
Reduce detrimental effects on the heart from catecholamine stimulation
- Slow HR to increase diastolic perfusion
- Decrease after load
When do we use Beta blockers in treating dilated cardiomyopathy
HFrEF and LVEF ≤40%
Contraindications for beta blockers in dilated cardiomyopathy
HR<50
2nd or 3rd degree AV block
Asthma is a contraindication; COPD is NOT
What are the surgical options for dilated cardiomyopathy
- LVAD
- Cardiac resynchoronization therapy (CRT)
- Automatic implantable cardioverter-defibrillator (AICD)
- Heart transplantation
What is hypertrophic cardiomyopathy caused by?
Genetic disease of heart muscle (myocardium)
- Autosomal dominant
- Mutation in sarcomere protein genes
Define hypertrophic cardiomyopathy
Unexplained LV hypertrophy without dilation of the ventricles or a cardiovascular disease that could cause the degree of hypertrophy seen
What is diagnostic of hypertrophic cardiomyopathy
> 15 mm LV wall thickness
What are the two classifications of hypertrophic cardiomyopathy
- Obstructive
2. Non-obstructive
Define obstructive hypertrophic cardiomyopathy
midsystolic obstruction of flow through the LV outflow tract as a result of systolic anterior motion of the mitral valve (SAM) toward the septum
What is the number one risk for sudden cardiac death?
History of syncope and/or a family history of sudden death
Hypertrophic cardiomyopathy sx’s
Fatigue Dyspnea Angina Palpitations Presyncope or syncope Orthopnea and paroxysmal nocturnal dyspnea (PND) Dizziness
What is a systolic ejection crescendo-decrescendo murmur indicate?
Hypertrophic cardiomyopathy
What is diagnostic of Hypertrophic cardiomyopathy
Transthoracic echocardiogram
What is the treatment for pt’s with symptomatic arrhythmias in hypertrophic cardiomyopathy
beta-blocker or anti-arrhythmic
What is the treatment for pt’s with arrhythmias sustaining frequent shocks from ICD with hypertrophic cardiomyopathy
Antiarrhythmic therapy:
- Sotalol
- Amiodarone
What is the treatment for pt’s with HCM and Atrial Fibrillation, despite CHADS2VASc score
Anticoagulation
Non-pharmacologic treatment for hypertrophic cardiomyopathy
Surgical septal myectomy
Alcohol septal ablation
How is restrictive cardiomyopathy characterized
Non-dilated, non-hypertrophied ventricles with impaired LV filling
=Diastolic dysfunction
What is the most common cause of secondary restrictive cardiomyopathy in the US?
Amyloidosis
What diseases cause secondary restrictive cardiomyopathy
Sarcoidosis
Scleroderma (Progressive Systemic Sclerosis)
Metastatic malignancy
Radiation induced
Drug induced (chloroquine, hydroxychloroquine)
Hemochromatosis
Clinical presentation of restrictive cardiomyopathy
Progressive exercise intolerance and SOB Fatigue Orthopnea Palpitations (frequently causes atrial fibrillation) Thromboembolic complications Orthostatic hypotension and syncope
Amyloidosis systemic signs
easy bruising
periorbital purpura macroglossia
+ Kussmaul Sign
JVP fails to fall during inspiration, and actually rises
Define cardiac amyloidsosis
- Multisystem deposition of amyloid fibrils
- Amyloidosis is a systemic disease, with cardiac infiltration being common
Myocarditis
-An inflammatory infiltrate of the myocardium with necrosis and/or degeneration of adjacent myocytes not typical of the ischemic damage associated with coronary artery disease
What is the most common cause of myocarditis in developed countries?
Viral infections
In developing countries, what are causes of myocarditis
- Rheumatic carditis
- Chagas disease
- Complications of HIV are more common
What is the gold standard diagnostic test for myocarditis
Endomyocardial Biopsy
When is Endomyocardial Biopsy indicated?
- New onset fulminant HF within 2 weeks with hemodynamic compromise
- New onset HF with failure to respond to treatment