Bobylev Dmitry, Boethig Dietmar, Breymann Thomas, Mathoni Annika, Horke Alexander, Ono Masamichi
Department of Cardiothoracic, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany.
Thorac Cardiovasc Surg. 2015 Aug;63(5):388-96. doi: 10.1055/s-0034-1376203. Epub 2014 Jul 4.
This study we evaluate our results for surgical treatment of thoracic aortic aneurysms in patients with congenital heart disease.
Fifty patients aged between 12 and 71 years were treated for 51 thoracic aortic aneurysms. Forty-four of the patients developed aneurysms in the ascending aorta and seven developed aneurysms in the descending aorta. The underlying diseases for ascending aortic aneurysms included 16 congenital aortic stenosis, 16 bicuspid aortic valves, 4 transposition of the great arteries, 3 tetralogy of Fallot, 2 truncus arteriosus communis, and 3 other diseases. Patients with connective tissue disorders were excluded. All descending aortic aneurysms developed late after coarctation repair. The time interval between the correction and aneurysm operations was 15 years in ascending aneurysms and 28 years in descending aneurysms.
The operative procedures for 44 ascending aortic aneurysms included 38 ascending aortic replacements, with or without aortic valve replacement (including 31 conduits), and 6 David operations. Graft replacement was undertaken for the seven descending aortic aneurysms. There were two hospital deaths. Forty-eight hospital survivors were in New York Heart Association functional class II or less at follow-up, which was conducted up to a maximum of 8 years after the procedure. All patients were free from reoperation and thromboembolic events. Aortic valve function was good in all six patients after the valve-sparing operation.
Thoracic aortic aneurysms in patients with congenital heart disease is highly associated with bicuspid aortic valve and aortic coarctation. For ascending aortic aneurysms, conduit replacement is the method of choice. The David procedure provides good results in selected patients. For descending aortic aneurysms, graft replacement is the preferred procedure. The relatively late development of thoracic aortic aneurysms indicates that long-term follow-up in patients with congenital heart disease, especially in patients with bicuspid aortic valve and aortic coarctation, is essential.
本研究评估我们对先天性心脏病患者胸主动脉瘤的手术治疗结果。
50例年龄在12至71岁之间的患者接受了51例胸主动脉瘤的治疗。44例患者升主动脉出现动脉瘤，7例患者降主动脉出现动脉瘤。升主动脉瘤的潜在疾病包括16例先天性主动脉狭窄、16例二叶式主动脉瓣、4例大动脉转位、3例法洛四联症、2例共同动脉干以及3例其他疾病。排除患有结缔组织疾病的患者。所有降主动脉瘤均在缩窄修复术后晚期出现。升主动脉瘤矫正与动脉瘤手术之间的时间间隔为15年，降主动脉瘤为28年。
44例升主动脉瘤的手术方式包括38例升主动脉置换术，伴或不伴主动脉瓣置换术（包括31例使用人工血管），以及6例David手术。7例降主动脉瘤进行了人工血管置换术。有2例住院死亡。48例住院幸存者在随访时纽约心脏协会心功能分级为II级或更低，随访在手术后最长8年进行。所有患者均未再次手术且无血栓栓塞事件。保留瓣膜手术后的6例患者主动脉瓣功能良好。
先天性心脏病患者的胸主动脉瘤与二叶式主动脉瓣和主动脉缩窄高度相关。对于升主动脉瘤，人工血管置换是首选方法。David手术在部分患者中效果良好。对于降主动脉瘤，人工血管置换是首选手术方式。胸主动脉瘤相对较晚出现表明对先天性心脏病患者，尤其是二叶式主动脉瓣和主动脉缩窄患者进行长期随访至关重要。
