目的:对接受了Shone综合征双心室修复手术的患者进行中期检查,并对死亡率和再手术危险因素进行评估。
方法:这项回顾性研究包括2005年至2020年期间接受二尖瓣(MV)手术的34例Shone's复合体患者。
结果:共有19例患者(56%)出现缩窄,10例(29%)主动脉瓣下狭窄,9例(26.5%)患者主动脉弓发育不良(AA),9例(26.5%)患者发生主动脉瓣(AV)狭窄。24例(70.6%)患者患有双叶AV。相关的左侧流入狭窄病变包括19例(56%)患者的降落伞MV和18例(53%)的二尖瓣上环。估计第6个月的再手术自由率,术后1年和2年为84.4%,79.5%,71.5%,分别。总死亡率为20.6%(7例),中位随访时间为10个月(0-41)。估计第6个月的生存率,1年,术后3年占83.8%,79.4%,和79.4。二叶主动脉瓣(p=.017)(HR(95%CI)=0.130(0.025-0.695)和吊床二尖瓣(p=.038)(HR(95%CI)=11,008(1,146->100)与死亡率相关。
结论:双叶主动脉瓣的存在可能会对结果产生负面影响。
OBJECTIVE: The midterm results of patients who underwent biventricular repair surgery for Shone\'s complex were examined, and mortality and reoperation risk factors were evaluated.
METHODS: This retrospective study included 34 patients with Shone\'s complex who underwent mitral valve (MV) surgery between 2005 and 2020.
RESULTS: A total of 19 patients (56%) had coarctation, 10 (29%) patients had subaortic stenosis, 9 (26.5%) patients had a hypoplastic aortic arch (AA), and 9 (26.5%) patients had aortic valve (AV) stenosis. Twenty-four (70.6%) patients had bileaflet AV. Associated left-sided in-flow stenotic lesions included parachute MV in 19 (56%) patients and supramitral ring in 18 (53%) patients. The estimated freedom from reoperation rate on the 6th month, 1 year and 2 years after surgery was 84.4%, 79.5%, and 71.5%, respectively. The overall mortality rate was 20.6% (seven patients) with a median follow-up of 10 months (0-41). The estimated survival rate on the 6th month, 1 year, and 3 years after surgery was 83.8%, 79.4%, and 79.4 respectively. Bicuspid aortic valve (p = .017) (HR (95% CI) = 0.130 (0.025-0.695) and hammock mitral valve (p = .038) (HR (95% CI) = 11,008 (1,146->100) were associated with mortality.
CONCLUSIONS: The presence of a bicuspid aortic valve hammock mitral valve might have an effect on negative effect on the outcome.