title: A case of pediatric cardiomyopathy with severely restrictive physiology.
abstract: A rare case of a 6-year-old male with idiopathic familial cardiomyopathy manifesting severely restrictive physiology is reported. The patient showed congestive heart failure with dilatation of both atria with a normal ventricular cavity. A square-root configuration was revealed in the ventricular pressure tracings. His elder brother had died of hypertrophic cardiomyopathy at the age of 3 years. Endomyocardial biopsy disclosed marked disorganization of muscle bundles with hypertrophy of the myocytes and interstitial fibrosis. The patient died suddenly during hospitalization. Autopsy revealed diffuse hypertrophy of both the ventricular walls and the ventricular septum with extensive myocardial disorganization and interstitial fibrosis. These advanced myopathic changes in the myocardium may have been related to the restrictive physiology in this case.
authors: Department of Pathology, Tokyo Women's Medical College, Japan.
authors: T
authors: T
authors: Nishikawa
authors: Y
authors: Y
authors: Tanaka
authors: Y
authors: Y
authors: Sasaki
authors: M
authors: M
authors: Kawataki
authors: Y
authors: Y
authors: Miyazawa
authors: S
authors: S
authors: Yasui
authors: M
authors: M
authors: Takarada
authors: T
authors: T
authors: Kasajima
mesh_terms: D002037:Bundle-Branch Block / Q000473:pathology / Q000503:physiopathology; D002312:Cardiomyopathy, Hypertrophic / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D002313:Cardiomyopathy, Restrictive / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D003937:Diagnosis, Differential; D004562:Electrocardiography; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D008297:Male; D009200:Myocardial Contraction / Q000502:physiology*; D009206:Myocardium / Q000473:pathology