Pulomonary Arterial Hypertension and Aneurysm in Patient with Marfan Syndrome


  • Author- Tariq Waseem, Aasma Riaz, Shahzad Latif, Wafa Qaisar, Hina Latif


Cardiovascular involvement in Marfan syndrome is considered major criteria for its diagnosis and typically includes mitral valve prolapse, aortic root dilatation and aortic dissection. Pulmonary arterial trunk involvement in Marfan syndrome has rarely been reported. We report a case of a young Pakistani male presenting with dyspnea and fever who had morphological features of Marfan syndrome. On chest X-ray a left hilar mass was reported. Echocardiography of the patient revealed dilatation of pulmonary artery and moderate pulmonary hypertension. An aneurysm involving pulmonary artery and its left main trunk was confirmed on CT chest with IV contrast and on cardiac catheterization. Aneurysm of pulmonary trunk should be considered in the differential diagnosis of mediastinal masses in patients having Marfan syndrome.


Marfan's Syndrome; Pulmonary Artery Aneurysm; Pulmonary hypertension.