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.
Neonatal Marfan syndrome - heart failure - pediatric heart transplantation
