Mingke D, Dresler C, Pethig K, Heinemann M, Borst H G
Department of Cardiovascular and Thoracic Surgery, Hannover Medical School, Germany.
J Cardiovasc Surg (Torino). 1998 Feb;39(1):65-74.
The authors retrospectively analyzed early and late results of surgical treatment for 79 Marfan patients with aneurysms and dissection of the proximal aorta.
From September 1979 to February 1996, 79 patients with Marfan syndrome underwent aortic root replacement using composite grafts (n=68, Bentall-technique 63, button-technique 5), and ascending aortic replacement with a valve-sparing procedure (n=11). There were 12 patients (15.2%) who simultaneously received partial or total arch replacement. 55 patients (69.6%) were male, and 24 female (30.4%). The average age was 33.8 years. Forty-one patient (51.9%) had non-dissecting aneurysms while the remaining 38 patients suffered from either acute (24.0%) or chronic aortic dissection (24.0%). The aortic valve was involved in 97.5% of all cases.
The total early mortality (< or =30 days) was 3.8%, 10.5% for acute aortic dissection and 2.4% for non-dissecting aneurysms. There were no early postoperative deaths in patients after valve-sparing operation and in those with chronic aortic dissection. The follow-up rate was 98.7%. During a mean follow-up of 68+/-25 months 10 patients (13.3%) died and cardiac complications were a common cause of the late deaths. There was no late mortality in the valve-sparing operations during a mean follow-up period of 8+/-6 months, however, 1 required valve replacement. 19 (25.3%) of the 75 patients surviving late have undergone 25 secondary operations on the cardiovascular system. Reoperations at aortic sites distant from the original were much more frequent after primary repair for acute and chronic dissection when compared to non-dissecting aneurysms (p<0.005). Actuarial survival rate of all patients with composite graft replacement including early deaths was 91.2% at 1 year, 84.4% at 5 years and 75.2% at 10 years.
Composite graft insertion has become the gold standard for treating Marfan-patients with non-dissecting and dissecting aneurysms of the aortic root. Our early experience in 11 patients with valve-sparing procedures indicated that this,variant may be the better choice in selected patients.
作者回顾性分析了79例患有主动脉近端动脉瘤和夹层的马凡综合征患者的手术治疗早期和晚期结果。
1979年9月至1996年2月，79例马凡综合征患者接受了复合移植物主动脉根部置换术（n = 68，Bentall技术63例，纽扣技术5例），以及保留瓣膜的升主动脉置换术（n = 11）。12例患者（15.2%）同时接受了部分或全弓置换。55例患者（69.6%）为男性，24例为女性（30.4%）。平均年龄为33.8岁。41例患者（51.9%）患有非夹层动脉瘤，其余38例患者患有急性（24.0%）或慢性主动脉夹层（24.0%）。97.5%的病例主动脉瓣受累。
早期总死亡率（≤30天）为3.8%，急性主动脉夹层为10.5%，非夹层动脉瘤为2.4%。保留瓣膜手术患者和慢性主动脉夹层患者术后无早期死亡。随访率为98.7%。在平均68±25个月的随访期间，10例患者（13.3%）死亡，心脏并发症是晚期死亡的常见原因。在平均8±6个月的随访期内，保留瓣膜手术无晚期死亡，但有1例需要更换瓣膜。75例晚期存活患者中有19例（25.3%）接受了25次心血管系统二次手术。与非夹层动脉瘤相比，急性和慢性夹层初次修复后，远离原发部位的主动脉再次手术更为频繁（p<0.005）。包括早期死亡在内的所有接受复合移植物置换患者的精算生存率1年时为91.2%，5年时为84.4%，10年时为75.2%。
复合移植物植入已成为治疗马凡综合征患者主动脉根部非夹层和夹层动脉瘤的金标准。我们对11例保留瓣膜手术患者的早期经验表明，对于选定患者，这种术式可能是更好的选择。
