Treatment strategy selection of Marfan syndrome combined with thoracoabdominal aortic diseases / 中国胸心血管外科临床杂志

ABSTRACT

@#Objective    To summarize the surgical treatment plan and experience of patients with Marfan syndrome complicated with Stanford type B aortic dissection, and to explore the treatment strategy selection. Methods    A retrospective analysis was conducted on 27 patients with Marfan syndrome complicated with thoracoabdominal aortic diseases who were treated in the department of cardiovascular surgery of our hospital from January 2013 to June 2019, including 13 males and 14 females, with an average age of 32.2±8.6 years. According to the patients' conditions, 19 of them received single pump-assisted blood transfusion combined with total thoracoabdominal aortic replacement (TAAAR), and 8 received thoracic endovascular aortic repair (TEVAR) in critically ill and pregnant patients. The patients were followed up in the outpatient clinic, and the thoracoabdominal aortic CT angiography was reexamined at 3 months, 6 months, 12 months and annually. The outcome of surgery, the incidence of intermediate cardiovascular adverse events, defined as the reoperation due to aortic or cardiac diseases, and intermediate survival rate were studied. Results    All 27 patients successfully completed the operation, the operation time was 60-852 (395.10±222.60) min, the spinal cord ischemia time was 14-26 (19.33±3.44) min, and the abdominal viscera ischemia time was 16-23 (19.83±1.94) min. Eight patients of TEVAR were all operated in acute phase and 19 patients of TAAAR in chronic phase. Two early postoperative deaths occurred in TEVAR patients. One died of puerperal infection and multiple organ dysfunction after cesarean section at the same time. After TEVAR, type A dissection re-ocurred in one patient. The family member gave up the treatment, and the patient died of the dissection ruptured after cesarean section. During the average follow-up of 47.6±36.7 months, 1 patient died of cerebrovascular accident and 9 patients were reoperated for adverse cardiovascular events, including 4 in TEVAR and 5 in TAAAR. Conclusion    TAAAR is the first choice for the treatment of Marfan syndrome combined with thoracoabdominal aortic diseases. TEVAR is easy to operate, with a low incidence of early mortality and complications, but has the risk of internal leakage and avulsion, and a high reoperation rate in the middle stage, so it can be used for high-risk elderly patients not suitable for open surgery, or as a bridge therapy for emergency patients before open surgery.