Case Presentation This is a 58-year-old woman with Marfan syndrome, FBN1 mutation c.6423delG, who, in 2004, was found to have a dilated main pulmonary artery (MPA) on chest x-ray performed for cough and wheezing. The MPA was 4.9 cm on computed tomographic angiogram; mean pulmonary artery (PA) pressure was 33 mm Hg on right heart catheterization. Transthoracic echocardiogram showed a right ventr...
