关键词: Congenital heart disease Double-outlet ventricle Malposition of great arteries Paediatric cardiac surgery Réparation à Transposition of great arteries l’étage ventriculaire

Mesh : Humans Infant Transposition of Great Vessels / surgery Double Outlet Right Ventricle / surgery Ventricular Outflow Obstruction, Left Cardiac Surgical Procedures / methods Treatment Outcome Pulmonary Valve Stenosis / surgery Heart Septal Defects, Ventricular / surgery Arteries

来  源:   DOI:10.1093/ejcts/ezad409

Abstract:
OBJECTIVE: The purpose of this study is to describe the long-term results of the \'réparation à l\'étage ventriculaire\' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS).
METHODS: Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%).
RESULTS: Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years.
CONCLUSIONS: The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.
摘要:
目的:本研究的目的是描述“脑室分离”(REV)技术用于右心室双出口(DORV)和大动脉转位的长期结果(TGA)伴有肺动脉狭窄(PS)。
方法:在1980年至2021年之间,157例患者接受了REV手术(中位年龄和体重:20.8个月和7.7kg)。最常见的解剖学表现是TGA之间的关联,室间隔缺损(VSD),PS(n=116,73.9%)。
结果:67例患者(42.7%)接受了Rashkind手术,67例患者(42.7%)曾接受过手术缓解(包括62例全身至肺动脉分流)。109例患者(69.4%)进行了圆锥隔膜切除术和/或VSD扩大。13名患者(8.3%)死亡,包括术后第一个月的4例和心脏移植后的2例。40年总生存率为89.3%。37例患者(23.6%)需要对右心室流出道(RVOT)进行68次再干预,包括49次再次手术,REV后的中位延迟为9年(8个月-27年)。20例患者(12.7%)接受了RVOT瓣膜置换术(16例手术和4例介入)。40年RVOT再干预和再手术的发生率分别为60.3%和62.6%。4例患者(2.5%)因左心室流出道梗阻(LVOTO)需要再次手术,平均延迟4.8年。
结论:对于PS患者的TGA和DORV,REV程序是一个很好的选择。只有四分之一的患者需要在RVOT上进行重做手术。LVOTO的再操作很少。
公众号