Coronary ostial aneurysms following aortic root replacement in patients with familial aortic aneurysm are common and support the need for long-term surveillance

Abstract

BackgroundFollowing the successful aortic root replacement (ARR) in patients with familial forms of aortic aneurysms (FFAA), few studies have defined the long-term outcomes of the re-implanted coronary arteries. The goal of the study was to describe late coronary complications following ARR in patients with FFAA. Methods40 patients with a genetically confirmed form of FFAA S/P ARR whom had undergone at least 1 CT scan or cardiac MRI study following ARR were identified. All studies were reviewed retrospectively and the coronary ostium were measured offline by two independent observers. ResultsThe majority of patients had Marfan Syndrome (80%), 15% had Loeys-Dietz Syndrome (TGFBR1 or TGFBR2), and 2 patients a MYLK or ACTA2 mutation. Mean age at time of ARR was 30.7 years, range 10–65. Mean length of follow-up from ARR 6.2 years, range 0.5–19. At last follow-up, 55% had developed at least 1 coronary complication. Nineteen (48%) had developed a coronary ostium aneurysm and 3 patients coronary ostium stenosis. All patients were alive at last follow-up. No patient had developed a coronary button rupture or dissection. Patients with aneurysms were more likely to have a mutation other than Marfan Syndrome (p < .05) and were more likely to have undergone emergent ARR following dissection (p = .008). Progressive coronary ostium dilation was rare. Two patients however did develop severe dilation and required button revision, both with TGFBR2 mutation. ConclusionCoronary ostium dilation is common following ARR in patients with FFAA. Progressive dilation and risk for coronary button rupture appears to be low. Patients with non-fibrillin 1 mutations, are at higher risk for severe dilation and some may require intervention. Long term surveillance in particular in patients with non-fibrillin1 forms of familial aortic aneurysm and dissection syndrome should be considered.