AME case reports · 2026
Urgent sutureless repair of supracardiac total anomalous pulmonary venous return coexisting with Ebstein's anomaly in a female neonate: a rare case report.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Getachew Abel, Muralidaran Ashok, Likes Maggie L, Ootaki Yoshio
Аннотация
BACKGROUND: Total anomalous pulmonary venous return (TAPVR) and Ebstein's anomaly are both rare congenital cardiac defects, and their coexistence is exceptionally uncommon, particularly in neonates. When present together, these defects create a unique hemodynamic conflict: TAPVR imposes a significant pulmonary venous volume load on the right heart, while Ebstein's anomaly limits right ventricular (RV) compliance and functional capacity, compromising the ability to accommodate this increased preload and predisposing affected neonates to early hypoxemia and hemodynamic instability.
CASE DESCRIPTION: We present a full-term female neonate with supracardiac TAPVR and Ebstein's anomaly who exhibited persistent hypoxemia with oxygen saturations of 77% despite maximal medical support. Echocardiography and computed tomography confirmed supracardiac TAPVR draining via a vertical vein to the innominate vein, a large secundum atrial septal defect (ASD), patent ductus arteriosus (PDA), and Ebstein's anomaly with mild RV hypoplasia but no significant tricuspid regurgitation. On day 2 of life, surgical repair was performed using a sutureless anastomosis of the pulmonary venous confluence to the left atrium, PDA ligation, and partial ASD closure with a 3-mm fenestrated patch. Postoperative management included delayed sternal closure and inhaled nitric oxide therapy. The patient recovered well and maintained stable oxygen saturations at discharge.
CONCLUSIONS: This case demonstrates that primary sutureless repair of supracardiac TAPVR combined with intentional atrial fenestration is a viable strategy for managing the complex RV hemodynamics associated with the coexistence of TAPVR and Ebstein's anomaly in neonates. This physiologically guided approach allows effective relief of pulmonary venous obstruction while preserving RV decompression and may optimize early outcomes in this rare and challenging clinical scenario.
CASE DESCRIPTION: We present a full-term female neonate with supracardiac TAPVR and Ebstein's anomaly who exhibited persistent hypoxemia with oxygen saturations of 77% despite maximal medical support. Echocardiography and computed tomography confirmed supracardiac TAPVR draining via a vertical vein to the innominate vein, a large secundum atrial septal defect (ASD), patent ductus arteriosus (PDA), and Ebstein's anomaly with mild RV hypoplasia but no significant tricuspid regurgitation. On day 2 of life, surgical repair was performed using a sutureless anastomosis of the pulmonary venous confluence to the left atrium, PDA ligation, and partial ASD closure with a 3-mm fenestrated patch. Postoperative management included delayed sternal closure and inhaled nitric oxide therapy. The patient recovered well and maintained stable oxygen saturations at discharge.
CONCLUSIONS: This case demonstrates that primary sutureless repair of supracardiac TAPVR combined with intentional atrial fenestration is a viable strategy for managing the complex RV hemodynamics associated with the coexistence of TAPVR and Ebstein's anomaly in neonates. This physiologically guided approach allows effective relief of pulmonary venous obstruction while preserving RV decompression and may optimize early outcomes in this rare and challenging clinical scenario.