Cardiac Manifestations of Marfan Syndrome in Infancy and Childhood

Abstract

The clinical profile and course of 36 infants and children with Marfan syndrome is reviewed. Cardiac abnormalities were present in 61% of the patients and were the major cause of death. Mitral regurgitation, the most frequently encountered cardiac lesion (47%), occurred in both males and females, while aortic regurgitation was present only in males. Arrhythmias were observed in four patients, and congenital heart disease (atrial septal defect) was observed in one. A distinguishing auscultatory feature was an apical mid or late systolic murmur preceded by single or multiple clicks. A characteristic electrocardiographic pattern of S-T segment and T-wave changes in leads II, III, aVF, and left precordial leads was noted in 11 patients. The prognosis of children with Marfan syndrome and isolated mitral regurgitation was usually good, whereas patients with combined mitral and aortic regurgitation had a rapidly deteriorating clinical course. The overall mortality in the group was 14%.