Neonatal Marfan Syndrome: Improving the Bad Prognosis with a Strict Conservative Treatment with Carvedilol?

Abstract

We report about a successful heart failure therapy with carvedilol in two children with neonatal Marfan syndrome (nMFS). As shown in Case 1, double valve replacement in an infant with neonatal Marfan syndrome is feasible but its benefit on long term is uncertain. Excluding our patient, 3 infants with nMFS from the literature died early after cardiac surgery. Our second case is a unique patient who survives nMFS despite diaphragmatic herniae, dilated neonatal cisterna magna and severe atrioventricular valve insufficiencies. Treated with 0.7 mg/kg/day Carvedilol since his seventh month of life, he never developed severe heart failure. However despite his good health status at the age of 9 years, a progressive aortic root dilatation and left conornary aneurysm are still waiting for surgical repair.