Beyond the aortic root: Staged open and endovascular repair of arch and descending aorta in patients with connective tissue disorders

Background Improvements in care have prolonged survival of patients with connective tissue disorders (CTDs), but their entire native aorta remains at risk. Little data are available to guide treatment. Objectives were to characterize patients, describe repair methods, and assess outcomes. Methods From 1996 to 2012, 527 patients with CTDs underwent cardiovascular operations. Beyond the root, arch and descending repair was performed in 121 patients (23%) for aneurysm (n = 17), acute complicated dissection (n= 5), or chronic dissection with aneurysmal degeneration (n = 99). CTD diagnoses included Marfan (n = 107), marfanoid (n = 7), Ehlers-Danlos (n = 4), and Loeys-Dietz (n = 3) syndromes. Eighty-seven (72%) had a previous ascending aorta repair, including 51 (57%) for type A dissection. Median interval to distal operation was 8.4 years. Index procedures for repair beyond the root were elephant trunk (ET) stage I (n = 63), open descending repair (n = 26), thoracoabdominal repair (n = 13), total arch replacement (n = 13), and stent-grafting (n = 6: frozen ET 3, thoracic endovascular aortic repair [TEVAR] 3). Median follow-up was 4.4 years. Results Operative mortality was 2.5% (3 of 121). No paralysis occurred, but 3 patients (2.5%) had nonpermanent stroke, 4 (3.3%) required dialysis, 12 (10%) required tracheostomy, and 13 (11%) underwent reoperation for bleeding. During follow-up, 67 patients underwent 85 additional distal aortic procedures (58 open, 27 endovascular, 49 of which were stage II ET). By 10 years, probability of at least 1 reintervention was 61%. At 1, 5, and 10 years, estimated survival was 91%, 79%, and 62%, and event-free survival was 52%, 35%, and 24%, respectively. Conclusions Most patients with CTDs who require operations beyond the aortic root have aortic dissection and require multiple reinterventions. Staged repair strategies, including open repair in combination with TEVAR, are feasible, and benefits outweigh risks. These patients require lifelong imaging surveillance.