Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan.
Ann Thorac Surg. 2011 Nov;92(5):1646-9; discussion 1649-50. doi: 10.1016/j.athoracsur.2011.06.090. Epub 2011 Oct 31.
This study determined the midterm outcome of valve-sparing aortic root replacement for patients with inherited connective tissue disorders.
From 1993 to 2008, 94 patients underwent valve-sparing aortic root replacement. Sixty patients (64%), average age 33 years (range, 15 to 61 years), had inherited connective tissue disorders: Marfan syndrome, 54 (92%); Loeys-Dietz syndrome, 5 (8%); and smooth muscle α-actin (ACTA2) mutation in 1. Median preoperative sinus diameter was 52 mm (range, 42 to 76 mm), and moderate/severe aortic regurgitation was present in 14 (23%). Seven (12%, 1993 to 1999) underwent remodeling procedures, and 53 had reimplantation procedures. Cusp repair was performed in 4. Median follow-up was 55 months (range, 1 to 149 months). There were 15 patients in the early term (1993 to 2000) and 45 in the late term (2001 to 2008).
Four late deaths occurred (cardiac, 3; aortic, 1), with 10-year survival of 86%. Rates of freedom from aortic valve replacement at 5 and 10 years were 85% and 58% in remodeling and 96% and 58% in reimplantation. Risk factors for reoperations were postprocedure intraoperative aortic insufficiency greater than mild (p = 0.046), remodeling procedure (p = 0.016), and early term (p = 0.0002). One patient (2%) with none/trivial postprocedure aortic insufficiency required aortic valve replacement. Freedom from reoperation in patients with none/trivial postprocedure aortic insufficiency at 5 and 10 years was 100% and 67%.
Meticulous control of aortic insufficiency during operation would bring favorable midterm durability in valve-sparing aortic root replacement using a reimplantation technique, even in patients with inherited connective tissue disorders.
本研究旨在评估心脏瓣膜保留型主动脉根部替换术治疗遗传性结缔组织疾病患者的中期疗效。
1993 年至 2008 年，94 例行心脏瓣膜保留型主动脉根部替换术患者纳入本研究。其中 60 例（64%）患者患有遗传性结缔组织疾病，包括马凡综合征 54 例（92%）、Loeys-Dietz 综合征 5 例（8%）和平滑肌α-肌动蛋白（ACTA2）突变 1 例。术前窦管交界直径中位数为 52mm（范围 42-76mm），14 例（23%）存在中重度主动脉瓣关闭不全。7 例行主动脉根部塑形术，53 例行主动脉瓣叶重新植入术。4 例行瓣叶修复术。中位随访时间为 55 个月（范围 1-149 个月）。其中 15 例患者为早期组（1993 年至 1999 年），45 例为晚期组（2001 年至 2008 年）。
共有 4 例患者术后晚期死亡（心脏源性 3 例，主动脉源性 1 例），10 年生存率为 86%。主动脉瓣再次置换的 5 年和 10 年无事件生存率在主动脉根部塑形组分别为 85%和 58%，在主动脉瓣叶重新植入组分别为 96%和 58%。再次手术的危险因素包括术后中重度主动脉瓣关闭不全（p=0.046）、主动脉根部塑形术（p=0.016）和早期组（p=0.0002）。1 例（2%）术后无/轻度主动脉瓣关闭不全患者需行主动脉瓣置换术。术后无/轻度主动脉瓣关闭不全患者的 5 年和 10 年无事件生存率分别为 100%和 67%。
采用主动脉瓣叶重新植入技术行心脏瓣膜保留型主动脉根部替换术，术中精细控制主动脉瓣关闭不全可获得良好的中期效果，即使是遗传性结缔组织疾病患者也是如此。
