Pediatric Cardiology ( IF 1.5 ) Pub Date : 2023-03-04 , DOI: 10.1007/s00246-023-03101-4 Justin Robinson 1, 2, 3 , Joseph M Forbess 1, 2 , Michael Slack 1, 2 , Julianne Moss 1, 2 , Alicia Chaves 1, 2
Patients born with obstructed total anomalous pulmonary venous return have a high risk of morbidity and mortality in the neonatal period, which only increases when combined with single ventricle physiology and non-cardiac congenital anomalies such as heterotaxy syndrome. Despite advances in management of congenital heart disease, surgery within the first weeks of life to repair the pulmonary venous connection and establish pulmonary blood flow with a systemic-to-pulmonary shunt has historically led to disappointing outcomes. A multidisciplinary approach with pediatric interventional cardiology and cardiac surgery is required to reduce morbidity and mortality in this extremely high-risk patient population. Extending the time between birth and cardiac surgery can lessen postoperative complications and mortality risk, especially in patients with abnormal thoracoabdominal relationships. Our team was able to successfully utilize transcatheter stent placement in a vertical vein and patent ductus arteriosus to delay and stage cardiac surgeries in an infant born with obstructed total anomalous pulmonary venous return, unbalanced atrioventricular septal defect with pulmonary atresia and heterotaxy, thus reducing the morbidity and mortality associated with this diagnosis.
中文翻译:
肺动脉闭锁和 TAPVR 阻塞的异位单心室新生儿的姑息治疗
出生时肺静脉完全异常回流受阻的患者在新生儿期发病和死亡的风险很高,只有与单心室生理学和非心脏先天性异常(如异位综合征)相结合时,这种风险才会增加。尽管先天性心脏病的治疗取得了进展,但在出生后最初几周内进行手术修复肺静脉连接并通过全身至肺分流建立肺血流,历来都导致了令人失望的结果。需要采用儿科介入心脏病学和心脏手术的多学科方法来降低这一极高危患者群体的发病率和死亡率。延长出生和心脏手术之间的时间可以减少术后并发症和死亡风险,特别是对于胸腹关系异常的患者。我们的团队能够成功地利用经导管在垂直静脉和动脉导管未闭中放置支架来延迟和分期对出生时患有完全性肺静脉回流受阻、房室间隔缺损伴肺动脉闭锁和异位性房室间隔缺损的婴儿进行心脏手术,从而降低发病率以及与该诊断相关的死亡率。