Objectives: Patients with Marfan syndrome are usually not suitable for endovascular repair of the thoracoabdominal aorta. This study was designed to analyze our center's experience with open surgical thoracoabdominal aortic replacement in Marfan patients.
Methods: This was a retrospective study with prospective follow-up. Between January 1995 and September 2021, a total of 648 patients underwent thoracoabdominal aortic replacement at our center. Of these, 60 had Marfan syndrome and were included in this study.
Results: The mean age was 39.5 ± 10.7 years, and 36 (60%) were male. Ten (17%) had aortic aneurysm, 4 (7%) acute/subacute dissection, and 46 (77%) chronic dissection. Patients presented with the following extent of aortic disease according to the Crawford classification: I-17 (28%), II-18 (30%), III-22 (37%), IV-2 (3%), and V-1 (2%). The mean cardiopulmonary bypass time was 173.9 ± 84.7 minutes. Four (7%) patients required stent graft extraction. Postoperatively, 5 (8%) patients required rethoracotomy and 6 (10%) tracheostomy. One (1.7%) patient had permanent paraplegia and 2 (3%) permanent paraparesis. Two (3%) patients had stroke. One (1.7%) patient was discharged with dialysis. The 30-day mortality was 3% (n = 2). Median follow-up time was 21.5 (range, 9.4-33.6) years. The 1-, 5-, and 10-year survival rate was 87%, 80%, and 68%, respectively. There were 16 aortic reinterventions in 9 patients during follow-up.
Conclusions: Thoracoabdominal aortic replacement remains a complex procedure but can be done extremely safely in Marfan patients. Perioperative mortality rates are very low, and the long-term outcomes are enduring. Because endovascular aortic repair is not recommended for patients with connective tissue disease, open surgery remains an important cornerstone of therapy.
Keywords: EVAR, endovascular aortic repair; Marfan syndrome; TEVAR, thoracic endovascular aortic repair; descending aortic repair; thoracoabdominal aortic repair.
© 2022 The Author(s).