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Pediatric Cardiomyopathy

Abstract

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NORD is very grateful to Steve D. Colan, MD, Associate Professor of Pediatrics at Harvard Medical School and Chief, Division of Noninvasive Cardiology, Boston Children's Hospital, and the Children's Cardiomyopathy Foundation, for assistance in the preparation of this report.

Synonyms of Pediatric Cardiomyopathy

  • arrhythmogenic right ventricular cardiomyopathy (ARVC)
  • asymmetrical septal hypertrophy
  • familial congestive cardiomyopathy
  • familial dilated cardiomyopathy (FDC)
  • hypertrophic obstructive cardiomyopathy (HOCM)
  • idiopathic dilated cardiomyopathy
  • idiopathic hypertrophic subaortic stenosis
  • non-obstructive hypertrophic cardiomyopathy

Disorder Subdivisions

  • arrhythmogenic right ventricular dysplasia (ARVD)
  • dilated cardiomyopathy
  • hypertrophic cardiomyopathy (HCM)
  • restrictive cardiomyopathy

General Discussion

Pediatric cardiomyopathy is a rare heart condition that affects infants and children. Specifically, cardiomyopathy means disease of the heart muscle (myocardium). Several different types of cardiomyopathy exist and the specific symptoms vary from case to case. In some cases, no symptoms may be present (asymptomatic); in many cases, cardiomyopathy is a progressive condition that may result in an impaired ability of the heart to pump blood; fatigue; heart block; irregular heartbeats (tachycardia); and, potentially, heart failure and sudden cardiac death.

Cardiomyopathy may be termed ischemic or nonischemic. Ischemic cardiomyopathy refers to cases that occur due to a lack of blood flow and oxygen (ischemia) to the heart. Such cases often result from hardening of the arteries (coronary artery disease). Nonischemic cardiomyopathy refers to cases that occur due to structural damage or malfunction of the heart muscle. Nearly all cases of pediatric cardiomyopathy are nonischemic. This report deals with nonischemic pediatric cardiomyopathy.

Cardiomyopathy may also be termed primary or secondary. Primary cardiomyopathy refers to cases where cardiomyopathy occurs by itself or for unknown reasons (idiopathic). Secondary cardiomyopathy refers to cases where the disease occurs secondary to a known cause such as heart muscle inflammation (myocarditis) caused by viral or bacterial infections; exposure to certain toxins such as heavy metals or excessive alcohol use; or certain disorders that affect the heart and/or additional organs systems. According to the Pediatric Cardiomyopathy Registry, approximately 79 percent of pediatric cardiomyopathy cases occur for unknown reasons (idiopathic).

Nonischemic cardiomyopathy may be further divided into four subtypes based upon the specific changes within the heart. These subtypes are: dilated, hypertrophic, restrictive and arrhythmogenic right ventricular dysplasia.

Organizations related to Pediatric Cardiomyopathy

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