Department of Surgery, Weill Cornell New York Presbyterian, New York, NY.
Division of Biostatistics and Epidemiology, Department of Pediatrics, Cincinnati Children's Hospital, University of Cincinnati College of Medicine, Cincinnati, Ohio.
J Thorac Cardiovasc Surg. 2021 Aug;162(2):385-393. doi: 10.1016/j.jtcvs.2020.10.053. Epub 2020 Oct 22.
We investigated the incidence and predictors of failure to undergo the Fontan in children with hypoplastic left heart syndrome who survived superior cavopulmonary connection.
The cohort consists of all patients with hypoplastic left heart syndrome who survived to hospital discharge after superior cavopulmonary connection between 1988 and 2017. The primary outcome was attrition, which was defined as death, nonsuitability for the Fontan, or cardiac transplantation before the Fontan. Subjects were excluded if they were awaiting the Fontan, were lost to follow-up, or underwent biventricular repair. The study period was divided into 4 eras based on changes in operative or medical management. Attrition was estimated with 95% confidence intervals, and predictors were identified using adjusted, logistic regression models.
Of the 856 hospital survivors after superior cavopulmonary connection, 52 died, 7 were deemed unsuitable for Fontan, and 12 underwent or were awaiting heart transplant. Overall attrition was 8.3% (71/856). Attrition rate did not change significantly across eras. A best-fitting multiple logistic regression model was used, adjusting for superior cavopulmonary connection year and other influential covariates: right ventricle to pulmonary artery shunt at Norwood (P < .01), total support time at superior cavopulmonary connection (P < .01), atrioventricular valve reconstruction at superior cavopulmonary connection (P = .02), performance of other procedures at superior cavopulmonary connection (P = .01), and length of stay after superior cavopulmonary connection (P < .01).
In this study spanning more than 3 decades, 8.3% of children with hypoplastic left heart syndrome failed to undergo the Fontan after superior cavopulmonary connection. This attrition rate has not decreased over 30 years. Use of a right ventricle to pulmonary artery shunt at the Norwood procedure was associated with increased attrition.
我们研究了在接受上腔静脉-肺动脉吻合术（ superior cavopulmonary connection ）后存活下来的左心发育不全综合征患儿中未能进行 Fontan 手术的发病率和预测因素。
该队列包括 1988 年至 2017 年间接受上腔静脉-肺动脉吻合术治疗后存活至出院的所有左心发育不全综合征患儿。主要结局是淘汰，定义为死亡、不适合 Fontan 手术或在进行 Fontan 手术前进行心脏移植。如果患者等待 Fontan 手术、失去随访或接受双心室修复，则将其排除在外。研究期间根据手术或医疗管理的变化分为 4 个时期。使用调整后的逻辑回归模型确定淘汰率和预测因素。
在接受上腔静脉-肺动脉吻合术的 856 例住院幸存者中，52 例死亡，7 例被认为不适合 Fontan 手术，12 例接受或正在等待心脏移植。总淘汰率为 8.3%（71/856）。各时期淘汰率无显著变化。使用最佳拟合多因素逻辑回归模型，调整上腔静脉-肺动脉吻合术年份和其他有影响的协变量：Norwood 手术时的右心室-肺动脉分流（P<0.01）、上腔静脉-肺动脉吻合术时的总支持时间（P<0.01）、上腔静脉-肺动脉吻合术时的房室瓣重建（P=0.02）、上腔静脉-肺动脉吻合术时的其他手术（P=0.01）和上腔静脉-肺动脉吻合术之后的住院时间（P<0.01）。
在这项跨越 30 多年的研究中，8.3%的左心发育不全综合征患儿在上腔静脉-肺动脉吻合术后未能进行 Fontan 手术。30 多年来，这种淘汰率并未降低。在 Norwood 手术中使用右心室-肺动脉分流与淘汰率增加相关。
