Giant Pulmonary Artery Aneurysm in a Patient With Marfan Syndrome and Pulmonary Hypertension

Aneurysmal dilatation of the pulmonary artery is rare.1 Management considerations, including indications for surgery, are not well defined.2,3 We present a case of giant pulmonary artery aneurysm (PAA) in a patient with pulmonary hypertension (PH) and connective tissue disorder.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 ventricular systolic pressure of 83 mm Hg and 2+ pulmonary regurgitation. Mitral regurgitation was mild; neither aortic regurgitation nor aortic root dilatation was present. Over the next 4 years, the MPA progressed to 7.9 cm despite initiating sildenafil therapy for idiopathic pulmonary arterial hypertension. Operative repair was deferred because of the poor control of PH. Switching to amlodipine and ambrisentan improved her symptoms. Right ventricular systolic pressure fell to 30 mm Hg, and the MPA stabilized in size for 6 years, but cardiac magnetic resonance in …