Marfan's syndrome in mitral valve disease.

We read with interest the report by Marlow et al on an infant with Marfan's syndrome and mitral valve dysfunction who died after acute illness with gross cardiac failure.' The authors stated that 'disease affecting the mitral valve is usually more benign than that of the aortic valve ....' This statement, however, may not be applicable to infants and young children with Marfan's syndrome. We have recently reported on the clinical course and the echocardiographic findings in 25 children with this syndrome.2 Prolapse of the mitral valve leaflets was shown by echocardiography in all the patients and aortic pathology in 80% of the cases. Five patients died during the follow up period (mean 5±4 5 years); two of them were infants with severe mitral insufficiency and progressive congestive heart failure. The other three patients died during the second decade of life from complications of the aortic pathology. Another series reported by Sisk et al showed that among 15 patients with Marfan's syndrome diagnosed before 4 years of age, mitral valve dysfunction was the leading cause of cardiovascular morbidity and mortality.3 These data indicate that mitral valve disease is the most common cause of congestive heart failure and death in infants and young children with Marfan's syndrome.