Cardiac transplantation in neonatal Marfan syndrome -- a life-saving approach.

Krasemann T, Kotthoff S, Kehl HG, Debus V, Tjan TD, Schmid C, Vogt J, Scheld HH

Abstract

Marfan syndrome is a connective tissue disease with typical clinical signs and cardiac involvement. Its appearance in the neonatal period has a bad prognosis due to incompetence of all cardiac valves with subsequent congestive heart failure. Conservative management usually fails, the children die during their first year of life. We report on a girl with neonatal Marfan syndrome who suffered from regurgitance of all cardiac valves, enlarged ventricles, and dilated great arteries. She was NYHA class IV. At the age of six months she underwent heart transplantation. To prevent aneurysm formation and dissection of the great vessels, the whole aortic arch and pulmonary trunk were replaced as well.