Restrictive cardiomyopathy in childhood.
Depending on the part of the world one lives in, restrictive cardiomyopathy is either one of the rarest forms of cardiomyopathy in childhood, with no cause usually identified, or it is secondary to a poorly understood disease, endomyocardial fibrosis, that is endemic in some populations. Regardless of the underlying cause, the outcome is poor once symptoms develop. This article reviews the definitions, epidemiology, etiologies, genetics, "overlap" phenotypes, clinical presentation, diagnostic evaluation, outcome, and management of pediatric patients with restrictive cardiomyopathy.
Denfield
Susan W
SW
Lillie Frank Abercrombie Division of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin MC-19345C, Houston, TX 77030, USA. swdenfie@texaschildrenshospital.org
Webber
Steven A
SA
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D002313:Cardiomyopathy, Restrictive / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy*; D002648:Child; D003645:Death, Sudden / Q000209:etiology; D003935:Diagnostic Techniques, Cardiovascular; D004719:Endomyocardial Fibrosis / Q000150:complications; D005838:Genotype; D016027:Heart Transplantation; D006801:Humans; D009154:Mutation; D010641:Phenotype; D011379:Prognosis; D012518:Sarcomeres / Q000235:genetics
D000319:Adrenergic beta-Antagonists; D000925:Anticoagulants