Diagnosis of a postsurgical aortic pseudoaneurysm in Shone syndrome by cardiovascular magnetic resonance.

A 34-year-old male was admitted with increasing dyspnea on exertion for the past 3 years. He was born with shone syndrome. Shone syndrome was first described by Shone et al. in 1963 and consists of four obstructive, or potentially obstructive conditions of the heart. These are a “parachute” deformity of the mitral valve, supravalvular ring of the left atrium, subaortic stenosis, and aortic coarctation. He had repair of a coarctation of aorta within the first year of his life. At 7, he underwent mitral and aortic valvuloplasty and at 15 had aortic valvulotomy and aortoplasty. Five years later, he underwent repair of an ascending aortic aneurysm with a Dacron graft, reimplantation of the coronary ostia by a button technique, and valve replacements with a Bjork–Shiley mitral valve prosthesis and St. Jude aortic valve prosthesis. He was asymptomatic until age 31 when he was admitted with hemoptysis but discharged after negative bronchoscopy and V/Q scan. He continued to become progressively more dyspneic on exertion. He underwent right and left heart catheterization and transthoracic echocardiography, which showed pulmonary hypertension (Mean PA 1⁄4 46 mm Hg), increased right ventricular pressure and the presence of an aortic pseudoaneurysm (PSA) extending from the proximal ascending aorta and compressing the left atrium. On angiography it was noted that the left circumflex originated from the right coronary artery but it was not possible to visualize left main or left anterior descending artery. Cardiovascular MR (CMR) was performed including MR angiography with gadolinium DTPA. This showed the previously placed tube graft in the ascending aorta connected to a large PSA