Cardiovascular anomalies in Marfan's syndrome: the role of echocardiography and beta-blockers.
Six patients with Marfan's syndrome were included in a study to determine the effect of beta-blockers on progressive aortic dilation. Patients' histories and results of physical examination, chest radiogram, electrocardiogram, and echocardiogram are reported. Patients ranged in age from 5 years 8 months to 14 years; one was black, five were white, and four were male. The study included a brother and sister whose mother had had Marfan's syndrome and died at age 32. All six patients had aortic dilatation documented by echocardiography; two also had mitral valve prolapse. Auscultatory findings were unrevealing, but patients showed easy fatigability. During the prospective, randomized study, half of the patients were given beta-blockers; the other half formed a control group. Findings at short to midterm follow-up suggest that beta-blockers are beneficial in arresting progressive dilatation of the aorta.
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Division of Pediatric Cardiology, Stormont-Vail Regional Medical Center, Topeka, Kansas 66604-1353.
D000293:Adolescent; D001018:Aortic Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology; D001314:Auscultation; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic; D004452:Echocardiography / Q000379:methods / Q000592:standards*; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology; D006347:Heart Sounds; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008945:Mitral Valve Prolapse / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology; D011433:Propranolol / Q000008:administration & dosage / Q000627:therapeutic use*; D011446:Prospective Studies
D011433:Propranolol