title: Clinical spectrum of restrictive cardiomyopathy in children.
abstract: We reviewed the clinical spectrum and possible prognostic factors in 14 children with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.
authors.affiliation: Saint Louis University School of Medicine, Department of Pediatrics and Cardinal Glennon Children's Hospital, 1465 South Grand Blvd., St. Louis, MO 63104-1095, USA.
authors.forename: S C
authors.initials: SC
authors.lastname: Chen
authors.forename: I C
authors.initials: IC
authors.lastname: Balfour
authors.forename: S
authors.initials: S
authors.lastname: Jureidini
mesh_terms: D000293:Adolescent; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D001706:Biopsy; D006328:Cardiac Catheterization; D002313:Cardiomyopathy, Restrictive / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D018450:Disease Progression; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D009200:Myocardial Contraction; D011379:Prognosis; D013902:Radiography, Thoracic; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D004232:Diuretics; D014665:Vasodilator Agents