St Louis James D, McCracken Courtney E, Turk Elizabeth M, Hancock Hayley S, Menk Jeremiah S, Harvey Brian A, Vinocur Jeffrey M, Oster Matthew E, Moller James H, Spector Logan G, Kochilas Lazaros K
Department of Surgery, University of Missouri-Kansas City School of Medicine, Kansas City, Missouri.
Department of Pediatrics, Emory University School of Medicine and Children's Health Care of Atlanta, Atlanta, Georgia.
Ann Thorac Surg. 2018 Jan;105(1):186-192. doi: 10.1016/j.athoracsur.2017.05.052. Epub 2017 Aug 25.
Long-term survival, risk of transplantation, and causes of death after repair of total anomalous pulmonary venous connection (TAPVC) remain unknown. By linking the Pediatric Cardiac Care Consortium with the National Death Index and the United Network for Organ Sharing, we evaluated long-term transplant-free survival in children undergoing repair of TAPVC.
We identified 777 infants within the Pediatric Cardiac Care Consortium who underwent TAPVC repair (median 21 days; interquartile range, 5 to 80) and had sufficient personal identifiers for linkage with the National Death Index and United Network for Organ Sharing. Sixty-six deaths, ten cardiac transplantations, and one bilateral lung transplantation had occurred by the end of 2014. Data collected included age and weight at time of procedure, TAPVC type, associated cardiac lesions, and postoperative length of stay. The study cohort was divided into simple and complex TAPVC based on the presence of an associated cardiac lesion. Parametric survival plots were constructed, and risk factor analyses were performed to identify demographic and clinical characteristics associated with long-term outcomes.
Mortality or need for transplantation was 9.7% with a median follow-up of 18.4 years and a median age of death or transplant of 0.74 years. The risk of mortality and transplant after TAPVC repair was highest during the first 18 months after hospital discharge. Cardiac causes accounted for the majority of deaths. Multivariate regression models for transplant-free survival demonstrated that complex TAPVC, mixed TAPVC, and postoperative length of stay were associated with increased risk of death/transplant.
Transplant-free survival after TAPVC repair is excellent, with most deaths or transplant events occurring early. Factors associated with the worst long-term outcomes included complex TAPVC, mixed TAPVC, and prolonged postoperative length of stay.
完全性肺静脉异位连接（TAPVC）修复术后的长期生存率、移植风险及死亡原因仍不清楚。通过将儿童心脏护理联盟与国家死亡指数和器官共享联合网络相联系，我们评估了接受TAPVC修复术儿童的长期无移植生存率。
我们在儿童心脏护理联盟中确定了777例接受TAPVC修复术的婴儿（中位年龄21天；四分位间距，5至80天），他们有足够的个人标识符以便与国家死亡指数和器官共享联合网络相联系。到2014年底，已发生66例死亡、10例心脏移植和1例双侧肺移植。收集的数据包括手术时的年龄和体重、TAPVC类型、相关心脏病变及术后住院时间。根据是否存在相关心脏病变，将研究队列分为单纯性和复杂性TAPVC。构建参数生存曲线，并进行危险因素分析以确定与长期结局相关的人口统计学和临床特征。
中位随访18.4年，中位死亡或移植年龄为0.74岁，死亡率或移植需求为9.7%。TAPVC修复术后死亡和移植风险在出院后的前18个月最高。心脏原因占死亡的大多数。无移植生存的多变量回归模型表明，复杂性TAPVC、混合型TAPVC和术后住院时间与死亡/移植风险增加相关。
TAPVC修复术后的无移植生存率良好，大多数死亡或移植事件发生在早期。与最差长期结局相关的因素包括复杂性TAPVC、混合型TAPVC和术后住院时间延长。
