Cardiomyopathy

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  • Cardiomyopathy
  • Dilated Cardiomyopathy
    • Left or right ventricular enlargement with loss of contractile function causing congestive heart failure, dysrhythmias, or thrombus formation.
    • Patients typically have symptoms of CHF that is slowly progressive
    • leads to death in approximately 3 years. (MCQ)
    • Etiology
      • Infectious
        • Viral myocarditis (one-third improve, one-third stay the same, one- third get worse)(MCQ)
      • Toxic(MCQ)
        • Reversible—prolonged EtOH abuse
        • Irreversible—doxorubicin (Adriamycin), cocaine, heavy metals (Pb,Hg, Cb)
      • Endocrine(MCQ)
        • Reversible—thyroid disease (hypo or hyper)
        • Irreversible—acromegaly, pheochromocytoma
      • Metabolic(MCQ)
        • Reversible—hypocalcemia, hypophosphatemia, thiamine deficiency (wet beriberi), selenium deficiency
      • Genetic: 20% of cases have positive family histories
      • Pregnancy: Similar prognosis as viral
      • Other:
        • Neuromuscular disease (usually irreversible)
        • idiopathic (usuallyirreversible)
      • Mechanical
        • Dysrhythmias
        • Valvular disease
    • Signs and symptoms
      • Symptoms of heart failure
      • Angina due to increased O2 demands of enlarged ventricles
      • Neurologic deficits from thrombus emboli
    • Diagnosis
      • Auscultation(MCQ)
        • S3/S4 gallop murmurs (stiffened ventricular walls)
        • regurgitant valves, rales
      • ECG
        • ventricular hypertrophy
        • bundle branch blocks
        • nonspecific ST segment/T wave changes,
        • dysrhythmias (atrial fibrillation most common)
      • CXR
        • enlarged cardiac silhouette, pulmonary venous congestion
      • Echocardiography
        • Enlargedventricles/atria,regurgitantvalves
        • lowejection fractions(MCQ)
    • Treatment
      • ACE inhibitorsand diuretics reduce mortality)(MCQ)
      • Anticoagulation with Coumadin (even if no evidence of thrombus)
      • Implanted automatic defibrillator for patients with life-threatening dysrhythmias(MCQ)
      • Heart transplant(MCQ)

 

  • Restrictive cardiomyopathy
    • Scarring and infiltration of the myocardiumcausing decreased right or left ventricular filling
    • Etiology(MCQ)
      • Amyloidosis ,Endomyocardial fibrosis
      • Hemochromatosis ,Sarcoidosis
      • Carcinoid heart disease
      • Congenital: Gaucher, Hurler, and glycogen storage diseases
    • Signs and symptoms
      • Signs of left/right heart failure, right failure usually predominates
      • Exercise intolerance is a common presenting symptom
    • Diagnosis
      • Auscultation
        • S3and/or S4gallopmurmurs(MCQ)
        • Occasionalmitralortricuspid regurgitation
      • ECG—
        • low voltages, conduction abnormalities
        • nonspecific ST seg- ment/T wave changes
        • leftbundle branch block(MCQ)
      • CXR—
        • normalcardiac silhouette or enlarged atria(MCQ)
        • pulmonary venous congestion
      • Echocardiography—
        • normal-sized ventricles, large atria
        • thickened ventricular walls
        • mitral/tricuspid regurgitation
        • typically has a speckled appearance if amyloid is cause
      • Endomyocardial biopsy(MCQ)
        • may detect eosinophilic infiltration ormyocardial fibrosis.
    • Treatment
      • No specific treatment or cure
      • Mainstay is to treat resulting heart failure.
      • Anticoagulate and rate control atrial fibrillation if present.
      • Treat underlying cause.
      • Permanent pacemaker for complete heart block(MCQ)
      • Heart transplant for refractory cases
    • Restrictive cardiomyopathy is often difficult to distinguish from constrictive pericarditis—biopsy can usually confirm.(MCQ)


Cardiomyopathy (CMP)
What is a cardiomyopathy?
Cardiomyopathies are instrinsic diseases of the heart. Here we see a defect in the cardiac muscle fibers. Cardiomypathies are rare heart diseases and these are more commonly used for differential diagnosis (eg inflammatory, valvular, vascular, congenital etc)
There are many types of cardiomyopathies but the common ones include dilated, hypertrophic and restrictive.
Which are types of cardiomyopathies do we have?
The most common types is the dilated cardiomyopathy that account for more than 90% and here we see features such as systolic failure, s3 gallops, annular dilation, risk of thromboemblosim and progressive heart failure. The other type is Hypertrophic cardiomyopathy where there is an genetical error in the myocardial fibers and their proteins, here we see the classical case of an young athlete that may suddenly fall and die. The third type of cardiomyopathy is the restrictive cardiomyopathy where there is a problem with infiltration of the myocardial fibers.
Cardiomyopathy signs and symptoms
Dilated cardiomyopathy: Pathophysiology and diagnosis
Hypertrophic cardiomyopathy: Pathophysiology and diagnosis
Cardiomyopathy
Hypertrophic Cardiomyopathy Overview – Mayo Clinic
Dr. Steve Ommen, a cardiologist at Mayo Clinic, discusses Hypertrophic Cardiomyopathy.
Restrictive cardiomyopathy: Pathophysiology and diagnosis
Surgery for Hypertrophic Cardiomyopathy
Hiroo Takayama, MD, PhD, surgeon at NewYork-Presbyterian Hospital/Columbia University Medical Center, narrates a surgery video of a hypertrophic cardiomyopthy procedure.
Cardiomyopathy treatment