The Fetal Heart Program at the Cardiac Center at the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; The University of Pennsylvania School of Medicine, Department of Pediatrics, Division of Cardiology, Philadelphia, Pennsylvania.
The Fetal Heart Program at the Cardiac Center at the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Ann Thorac Surg. 2014 Jul;98(1):152-8. doi: 10.1016/j.athoracsur.2014.03.002. Epub 2014 May 1.
We sought to describe current outcomes and risk factors for mortality for fetuses diagnosed with absent pulmonary valve syndrome (APV). Fetuses with APV were divided into two cohorts, those with underlying tetralogy of Fallot (TOF/APV) and those without underlying TOF and either an intact ventricular septum or small ventricular septal defect (APV/IVS).
The fetal echocardiographic database was reviewed from January 1, 2001, until June 1, 2010, and all subjects with a diagnosis of APV were included. Multiple clinical and fetal echocardiographic measurements were recorded. Statistical analysis was performed by χ2 analysis and t tests. Survival analysis was performed by Kaplan-Meier analysis. Significant relationships between variables were explored by regression analysis. Significance was set at p=0.05.
The cohort consisted of 15 fetuses with TOF/APV and 6 fetuses with APV/IVS. There were no fetal demises in either cohort. Survival to birth was 71% in the TOF/APV cohort and 83% in the APV/IVS cohort (p=0.62). Of subjects born alive, survival was 80% for both cohorts (p=0.95). However, in the APV/IVS cohort, transplantation-free survival was only 20%. Underlying single-ventricle physiology strongly predicted those who underwent heart transplantation (p=0.003, R2=0.50). For the entire APV cohort, left ventricular dysfunction (p=0.005, R2=0.41) and a higher pulmonary artery valve-to-aortic valve ratio (p=0.02, R2=0.34) predicted mortality.
Postnatal outcomes continue to improve for fetuses with APV syndrome. Left ventricular dysfunction and higher pulmonary artery valve-to-aortic valve ratio accurately predict postnatal mortality for fetuses with APV.
我们旨在描述诊断为肺动脉瓣缺如综合征（APV）的胎儿的当前结局和死亡率的相关风险因素。APV 胎儿分为两个队列，一组为伴有法洛四联症（TOF/APV）的胎儿，另一组为不伴有 TOF 且伴有完整室间隔或小室间隔缺损（APV/IVS）的胎儿。
从 2001 年 1 月 1 日至 2010 年 6 月 1 日，回顾胎儿超声心动图数据库，纳入所有诊断为 APV 的患者。记录多项临床和胎儿超声心动图测量值。采用卡方检验和 t 检验进行统计学分析。采用 Kaplan-Meier 分析进行生存分析。采用回归分析探索变量之间的显著关系。p=0.05 为差异有统计学意义。
该队列包括 15 例 TOF/APV 胎儿和 6 例 APV/IVS 胎儿。两个队列中均无胎儿死亡。TOF/APV 队列的出生存活率为 71%，APV/IVS 队列为 83%（p=0.62）。在出生存活的患者中，两个队列的存活率均为 80%（p=0.95）。然而，APV/IVS 队列中，无心脏移植的存活率仅为 20%。单心室生理学是预测需要进行心脏移植的重要因素（p=0.003，R2=0.50）。对于整个 APV 队列，左心室功能障碍（p=0.005，R2=0.41）和肺动脉瓣至主动脉瓣比值升高（p=0.02，R2=0.34）可准确预测胎儿的死亡率。
APV 综合征胎儿的出生后结局持续改善。左心室功能障碍和肺动脉瓣至主动脉瓣比值升高可准确预测 APV 胎儿的出生后死亡率。
