All issues > Volume 42(11); 1999

Case Report

J Korean Pediatr Soc. 1999;42(11):1583-1588. Published online November 15, 1999.

A Case of Infantile Marfan Syndrome

Sun Young SY Kim¹, Hee Ju HJ Park¹

¹Department of Pediatrics, Pusan National University, College of Medicine, Pusan, Korea

Correspondence Sun Young SY Kim ,Email: 1

Abstract

Marfan syndrome is infrequently diagnosed early in infancy. The morphologic characteristics and prognosis in infantile Marfan syndrome may be quite different from those in older patients. Characteristic cardiac findings in early life include mitral valve prolapse, valvular regurgitation, and aortic root dilation. Morbidity and mortality may be high for infants diagnosed with Marfan syndrome. Cardiac surgery for cardiovascular complication in infants often resulted in mortality due to the patient's young age and small size of valve. But recently, some reports say that operations for cardiovascular complications of Marfan syndrome can be performed in children with low operative morbidity and mortality. Echocardiographic findings of this patient were mitral valve prolapse, severe mitral regurgitation and aortic root dilatation. She underwent mitral valve replacement due to severe mitral valve prolapse and mitral regurgitation, and will have an aortic root and valve replacement in the near future. We experienced a case of infantile Marfan syndrome diagnosed by echocardiographic findings, and reported the case with associated literature.

Keywords :Infantile Marfan syndrome