Podolec P, Tracz W, Kostkiewicz M, Sadowski J, Hlawaty M, Olszowska M, Leśniak A, Andres J, Marek G, Pfitzner R, Dziatkowiak A
Department of Cardiovascular Diseases, Collegium Medicum, Jagiellonian University Cracow, Poland.
Int J Cardiol. 1997 Jun 27;60(1):41-7. doi: 10.1016/s0167-5273(97)02964-1.
The aim of the study was to assess the long-term results of surgical treatment with homogenic aortic grafts (HAGs) implantation in patients with Marfan syndrome. There were 31 patients with Marfan syndrome and aortic aneurysm who were operated on between 1980 and 1996. Aortic dissection was diagnosed in 14 patients, DeBakey Type I in six patients and Type II in eight patients. Four patients had to be operated urgently in cardiogenic shock with cardiac tamponade. Sealing up and reinforcement with strip of felt or Gore-Tex has been applied in 22 patients. The surgical modifications mentioned above have been applied since 1987 in all patients with the diameter of the aortic ring exceeding 30 mm or with active infective endocarditis or during reoperation. In 16 patients the space between the aortic homograft and patients own aortic wall was joined to the right atrial auricle. Patients were followed up for 12-179 months (average: 94.6 +/- 499). Three patients died in the early postoperative period and four patients died in the late postoperative period. Rethoracotomy because of bleeding complications was necessary in five patients. HAG damage was responsible for six other reoperations-new HAGs have been implanted in three patients and artificial prostheses were implanted in the other three patients. In the late follow-up period significant improvement in cardiac performance was observed in 24 patients (NYHA I or II). Survival probability of 15 years for the whole group was 80%. The lowest survival probability has been shown in the group of patients with DeBakey Type I aortic dissection (35% survived 15 years after operation). Echocardiographic follow-up has shown that the pressure gradient in HAG was low (7.4 +/- 6.2 mmHg). Only in two patients did the HAG gradient exceeded 20 mmHg. There were no significant differences concerning aortic ring diameters, dimensions of HAG and echocardiographic parameters between the group with surgical modifications, i.e. sealing up and reinforcement with strip of felt or Gore-Tex applied and the group in which these modifications were not applied. Homogenic aortic graft implantation as a method of surgical treatment of aortic aneurysm in patients with Marfan syndrome avoids postoperative anticoagulation, results in substantial improvement of cardiac performance and prolongs life. Surgical treatment should be considered in asymptomatic patients with large aneurysms (exceeding 55-65 mm) in patients with Marfan syndrome because there is a high risk of death in this group of patients in the case of dissection.
本研究的目的是评估马凡综合征患者植入同种异体主动脉移植物（HAGs）进行手术治疗的长期效果。1980年至1996年间，对31例患有马凡综合征和主动脉瘤的患者进行了手术。14例患者被诊断为主动脉夹层，其中6例为DeBakey I型，8例为II型。4例患者因心源性休克合并心脏压塞而紧急手术。22例患者采用毡条或戈尔特斯（Gore-Tex）条带进行封闭和加固。自1987年起，上述手术改良方法应用于所有主动脉环直径超过30mm或患有活动性感染性心内膜炎的患者或再次手术时。16例患者将主动脉同种异体移植物与患者自身主动脉壁之间的间隙与右心耳相连。对患者进行了12 - 179个月的随访（平均：94.6±49.9）。3例患者在术后早期死亡，4例患者在术后晚期死亡。5例患者因出血并发症需要再次开胸手术。6例再次手术是由于HAG损坏，3例患者植入了新的HAG，另外3例患者植入了人工假体。在晚期随访期间，24例患者（纽约心脏协会I级或II级）的心功能有显著改善。整个组15年的生存率为80%。DeBakey I型主动脉夹层患者组的生存率最低（术后15年35%存活）。超声心动图随访显示，HAG中的压力梯度较低（7.4±6.2mmHg）。仅2例患者的HAG梯度超过20mmHg。在应用毡条或戈尔特斯（Gore-Tex）条带进行封闭和加固的手术改良组与未应用这些改良方法的组之间，主动脉环直径、HAG尺寸和超声心动图参数方面无显著差异。同种异体主动脉移植物植入作为马凡综合征患者主动脉瘤的手术治疗方法，避免了术后抗凝，显著改善了心功能并延长了寿命。对于马凡综合征无症状且动脉瘤较大（超过55 - 65mm）的患者应考虑手术治疗，因为该组患者发生夹层时死亡风险很高。
