Heart failure definitions have changed over the years and patients with a dilated cardiomyopathy are generally placed into the category of heart failure with reduced ejection fraction where the LVEF is defined as less than or equal to 40%. In about half of the patients in this category, there is LV enlargement and it is this group that defines dilated cardiomyopathy. This is a large group of heterogeneous myocardial disorders characterized by reduced myocardial contractility in the absence of abnormal loading conditions such as with hypertension or valvular disease. The prevalence averages 36 cases/100,000 in the United States and accounts for approximately 10,000 deaths annually. Blacks are afflicted three times as often as whites. The prognosis is poor with 50% mortality at 5 years once symptoms emerge.
The causes are multiple and diverse. Up to 20–35% have a familial etiology. A large proportion is idiopathic. Endocrine, inflammatory, and metabolic causes include obesity, diabetes, thyroid disease, celiac disease, systemic lupus erythematosus, acromegaly, and growth hormone deficiency. Toxic, drug-induced, and inflammatory causes are listed in the prior section. Nutritional diseases such as deficiency of thiamine, selenium, and carnitine have also been documented. Dilated cardiomyopathy may also be caused by prolonged tachycardia either from supraventricular arrhythmias, from very frequent PVCs (more than 15% of heart beats), or from frequent RV pacing. Dilated cardiomyopathy is also associated with HIV, Chagas disease, rheumatologic disorders, iron overload, sleep apnea, amyloidosis, sarcoidosis, chronic alcohol usage, end-stage kidney disease, or cobalt exposure (“Quebec beer-drinkers cardiomyopathy”). Peripartum cardiomyopathy and stress-induced disease (tako-tsubo) are discussed separately.