Department of Thoracic and Cardiothoracic Surgery, Cleveland Clinic, Cleveland, Ohio; Case Western Reserve University School of Medicine, Cleveland, Ohio.
Department of Thoracic and Cardiothoracic Surgery, Cleveland Clinic, Cleveland, Ohio.
J Thorac Cardiovasc Surg. 2023 Mar;165(3):1224-1234.e9. doi: 10.1016/j.jtcvs.2022.05.030. Epub 2022 Jun 2.
Shone's syndrome (SS) has a varied anatomic spectrum without consensus on need and timing for mitral valve intervention (MVI). We sought to (1) characterize the anatomic spectrum and treatment pathways; (2) describe long-term outcomes and their determinants; and (3) define the impact of MVI timing on survival.
In total, 121 patients with SS who underwent operation at Cleveland Clinic between 1956 and 2021 were reviewed. Multivariable parametric hazard analyses including time-varying covariables, and modulated renewal to account for repeated events, were performed. End points included time-related survival and reintervention.
Median follow-up was 9.9 years. Mitral stenosis (MS) (98%), coarctation (80%), and aortic stenosis (70%) predominated. The most common combination was MS + aortic stenosis + coarctation (26%). Median initial mean mitral and aortic gradients were 3.6 (15th/85th percentiles: 2.0/6.8) and 9.0 (2.1/46) mm Hg, respectively. Median initial surgery age was 0.041 (0.011/3.2) years. Initial surgeries included coarctation repair (43%), arch repair (18%), and staged biventricular repair (18%). Overall survival was 92% at 20 years. Freedom from reoperation was 66% and 24% at 1 and 20 years. Patients with no MVI or initial MVI (N = 7) tended to be associated with better early survival compared with those with MVI at subsequent operation (N = 29) (P = .06). Risk factors for early reintervention included initial Norwood operation, with younger age and arch hypoplasia increasing later reintervention.
Despite excellent long-term survival, reoperation in SS is frequent and occurs most commonly on left ventricular outflow tract and mitral valve. Although MS is present in most, few require MVI. Delaying MVI may compromise early survival.
Shone 综合征（SS）具有多样化的解剖学谱，对于二尖瓣介入治疗（MVI）的需求和时机尚无共识。我们旨在：（1）描述解剖学谱和治疗途径；（2）描述长期结果及其决定因素；（3）定义 MVI 时机对生存的影响。
共回顾了 1956 年至 2021 年在克利夫兰诊所接受手术的 121 例 SS 患者。进行了包括时变协变量的多变量参数风险分析，以及调制更新以考虑重复事件。终点包括与时间相关的生存和再干预。
中位随访时间为 9.9 年。二尖瓣狭窄（MS）（98%）、缩窄（80%）和主动脉瓣狭窄（70%）为主。最常见的组合是 MS+主动脉瓣狭窄+缩窄（26%）。中位初始平均二尖瓣和主动脉梯度分别为 3.6（15 百分位/85 百分位：2.0/6.8）和 9.0（2.1/46）mmHg。初始手术年龄中位数为 0.041（0.011/3.2）岁。初始手术包括缩窄修复（43%）、弓部修复（18%）和分阶段双心室修复（18%）。20 年时总体生存率为 92%。1 年和 20 年时无再手术的生存率分别为 66%和 24%。与随后手术中进行 MVI 的患者（N=29）相比，无 MVI 或初始 MVI（N=7）的患者早期生存倾向更好（P=0.06）。早期再干预的危险因素包括初始 Norwood 手术，年龄较小和弓部发育不良增加了后期再干预的风险。
尽管长期生存情况良好，但 SS 患者再手术的频率很高，最常见于左心室流出道和二尖瓣。尽管大多数患者存在 MS，但很少需要 MVI。延迟 MVI 可能会影响早期生存。
