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Causes of dilated cardiomyopathy

Authors
Marilyn Weigner, MD, FACC
James P Morgan, MD, PhD
Section Editor
William J McKenna, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

Dilated cardiomyopathy (DCM) is characterized by dilation and impaired contraction of one or both ventricles [1-5]. Affected patients have impaired systolic function and may or may not develop overt heart failure (HF). The presenting manifestations can include atrial and/or ventricular arrhythmias, and sudden death can occur at any stage of the disease. (See "Definition and classification of the cardiomyopathies".)

DCM is currently responsible for approximately 10,000 deaths and 46,000 hospitalizations each year in the United States. Furthermore, idiopathic DCM is the primary indication for cardiac transplantation [6].

The definition, clinical presentation, and causes of DCM will be reviewed here. The initial evaluation of the patient with suspected HF and the subsequent evaluation to determine the cause are discussed separately. (See "Evaluation of the patient with suspected heart failure".)

DEFINITION

A diagnosis of dilated cardiomyopathy requires evidence of dilation and impaired contraction of the left ventricle or both ventricles (eg, left ventricular ejection fraction <40 percent or fractional shortening less than 25 percent) [1,2]. The disease is considered idiopathic if primary and secondary causes of heart disease (eg, myocarditis and coronary artery disease) are excluded by evaluation including history and physical examination, laboratory testing, coronary angiography (to exclude >50 percent obstruction of one or more coronary arteries), echocardiography, and, endomyocardial biopsy when indicated. (See "Endomyocardial biopsy".)

CLINICAL PRESENTATION

Most patients present between the ages of 20 and 60, but dilated cardiomyopathy can occur in children and older adults [3]. Affected patients can present in a number of different ways [3,7]. Symptoms of heart failure (progressive dyspnea with exertion, impaired exercise capacity, orthopnea, paroxysmal nocturnal dyspnea, and peripheral edema) are most common. Other presentations include the incidental detection of asymptomatic cardiomegaly and symptoms related to coexisting arrhythmia, conduction disturbance, thromboembolic complications, or sudden death [7]. (See "Evaluation of the patient with suspected heart failure", section on 'Clinical presentation'.)

                                

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Literature review current through: Nov 2016. | This topic last updated: Tue Jan 05 00:00:00 GMT 2016.
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