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Subject: Cardiomyopathy, End Stage
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Cardiomyopathy encompasses a large group of diseases of the myocardium that commonly result in mechanical pump dysfunction. The current classification scheme attempts to differentiate between myocardial diseases confined to the myocardium (primary) and those due to systemic disorders (secondary). Specific causes of myocardial dysfunction due to other cardiovascular disorders are considered a third, separate category (1).
Classification of cardiomyopathy
Hypertrophic cardiomyopathy (HCM)
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)
Left ventricular (LV) noncompaction (LVNC)
Glycogen storage (Danon type, PRKAG2)
Ion channel disorders: long QT syndrome, Brugada, short QT syndrome, catecholaminergic ventricular tachycardia (CVPT), Asian SUNDS
Dilated cardiomyopathy (DCM) (genetic or nongenetic)
Myocarditis, stress cardiomyopathy, peripartum, tachycardia-induced, infants of type 1 diabetic mothers
Secondary (see list below)
Congenital heart disease
Patients with end-stage cardiomyopathy have stage D heart failure or severe symptoms at rest refractory to standard medical therapy.
System(s) affected: cardiovascular; renal
60,000 patients <65 years die each year from end-stage heart disease.
35,000–70,000 people might benefit from cardiac transplant or chronic support.
Ischemic heart disease: most common etiology; up to 66% of patients
Valvular heart disease
Primary genetic causes
Beriberi, pellagra, scurvy, selenium, carnitine, kwashiorkor
Systemic lupus erythematosus
Viral (e.g., HIV, coxsackievirus, adenovirus)
Bacterial and mycobacterial (e.g., diphtheria, rheumatic fever)
Parasitic (e.g., toxoplasmosis, Trypanosoma cruzi)
Glycogen storage disease (type II, Pompe)
Duchenne and Emery-Dreifuss muscular dystrophies
Drugs and chemotherapy: anthracyclines, cyclophosphamide, Herceptin
Heavy metal, chemical agents
Hypereosinophilic syndrome (Loeffler endocarditis)
Coronary artery diease
Excessive alcohol intake
Obstructive sleep apnea
Dyspnea at rest or with exertion
Paroxysmal nocturnal dyspnea
Right upper quadrant pain or bloating
Low pulse pressure
Jugular venous distention
Displaced point of maximal impulse (PMI)
Blowing systolic murmur
Severe pulmonary disease
Primary pulmonary hypertension
Recurrent pulmonary embolism
Some advanced forms of malignancy
ECG: LV hypertrophy, interventricular conduction delay, atrial fibrillation, evidence of prior Q-wave infarction
Mild elevation in troponin
Elevated B-type natriuretic peptide (BNP) or pro-BNP
Elevated liver function tests
Elevated uric acid
Increased vascular markings to the upper lobes
Pleural effusions may or may not be present.
In dilated cardiomyopathy, 4-chamber enlargement and global hypokinesis are present.
In hypertrophic cardiomyopathy, severe LV hypertrophy is present.
Segmental contraction abnormalities of the LV are indicative of previous localized myocardial infarction.
May be useful to characterize certain nonischemic cardiomyopathies
Myocardial stress perfusion imaging (MPI)
Recommended in those with new-onset LV dysfunction or when ischemia is suspected
Helpful to rule out ischemic heart disease
Characterize hemodynamic severity
Pulmonary artery catheters may be reasonable in patients with refractory heart failure to help guide management.
Reduction of filling pressures
Treatment of electrolyte disturbances
Systolic failure syndromes
ACE inhibitors: All considered equally effective; initiate at low doses and titrate as tolerated to target doses (3)[A].
May need to be given IV initially and then orally as patient stabilizes
Furosemide, 40–120 mg/day or TID (3)[A]
Use with caution in acutely decompensated or low–cardiac output states.
Initiate with low doses and titrate as tolerated.
Metoprolol succinate, 12.5–200 mg/day; carvedilol, 3.125–25 mg BID; or bisoprolol, 1.25–10 mg/day (3)[A]
Patients with New York Heart Association (NYHA) II–IV heart failure, ejection fraction (EF) <35%, on standard therapy: aldosterone antagonists: spironolactone or eplerenone (3)[A]
Digoxin, 0.125–0.25 mg/day for symptomatic patients on standard therapy (3)[B]
Combination hydralazine/isosorbide dinitrate is 1st-line treatment in African American patients with class III–IV symptoms already on standard therapy and for all patients with reduced EF and symptoms incompletely responsive to ACE inhibitor and β-blocker (3)[A].
β-Blockers: low cardiac output, 2nd- or 3rd-degree heart block
Avoid use of diltiazem and verapamil in patients with systolic dysfunction.
Aldosterone antagonists: oliguria, anuria, renal dysfunction
Loop diuretics: hypokalemia, hypomagnesemia
ACE inhibitors: pregnancy, angioedema
In patients with chronic kidney disease, digoxin dosage should be ≤0.125 mg/day and drug levels followed carefully to avoid toxicity.
Closely monitor electrolytes.
ACE inhibitors: Initiate with care if BP is low. Begin with low-dose captopril, such as 6.25 mg TID.
β-Blockers: Avoid in patients with evidence of poor tissue perfusion; they may further depress systolic function.
Milrinone, dobutamine: long-term use associated with increased mortality
Medications TO AVOID
Angiotensin receptor blockers as an alternative to ACE inhibitors
Inotropic therapy (e.g., dobutamine or milrinone) for cardiogenic shock and support prior to surgery or cardiac transplantation (3)[B]
Continuous inotrope infusion may be considered in stage D outpatients for symptom control in those who are not eligible for transplantation or mechanical circulatory support (3)[B].
Prophylactic implantable cardioverter defibrillator (ICD) should be considered for patients with a left ventricular ejection fraction (LVEF) <35% and mild to moderate symptoms (3)[A].
Cardiac resynchronization therapy (CRT) is recommended and should be considered for patients in sinus rhythm with a QRS >150 msec, LVEF <35%, in FC I–III and ambulatory FC IV patients (3)[A].
Patients with severe, refractory heart failure with no reasonable expectation of improvement should not be considered for an ICD.
Consideration of an LV assist device as “permanent” or destination therapy or cardiac transplantation is reasonable in selected stage D patients.
I42.9 Cardiomyopathy, unspecified
I42.2 Other hypertrophic cardiomyopathy
I42.1 Obstructive hypertrophic cardiomyopathy
I43 Cardiomyopathy in diseases classified elsewhere
I42.4 Endocardial fibroelastosis
I42.5 Other restrictive cardiomyopathy
I42.8 Other cardiomyopathies
I42.6 Alcoholic cardiomyopathy
I42.3 Endomyocardial (eosinophilic) disease
I42.7 Cardiomyopathy due to drug and external agent
I42.0 Dilated cardiomyopathy
425.4 Other primary cardiomyopathies
425.18 Other hypertrophic cardiomyopathy
425.11 Hypertrophic obstructive cardiomyopathy
425.8 Cardiomyopathy in other diseases classified elsewhere
425.5 Alcoholic cardiomyopathy
425.7 Nutritional and metabolic cardiomyopathy
425.9 Secondary cardiomyopathy, unspecified
85898001 Cardiomyopathy (disorder)
233873004 Hypertrophic cardiomyopathy (disorder)
45227007 Hypertrophic obstructive cardiomyopathy (disorder)
195029002 Cardiomyopathy associated with another disorder (disorder)
83521008 Dilated cardiomyopathy secondary to alcohol (disorder)
399020009 Congestive cardiomyopathy (disorder)
415295002 Restrictive cardiomyopathy (disorder)
195023001 Nutritional and metabolic cardiomyopathies (disorder)
89461002 Primary cardiomyopathy
Cardiomyopathy represents the end-stage of a large number of disease processes involving the heart muscle.
Ischemic, hypertensive, postviral, familial, alcoholic, and incessant tachycardia-induced are the most common cardiomyopathy varieties seen in the United States.
Core therapy for heart failure applies: salt restriction, diuretics, ACE inhibitors, β-blockers, digoxin, and electrical treatments, such as cardiac resynchronization and implantable defibrillators, as appropriate.