Universitätsklinikum Erlangen, Erlangen, Bayern, Germany.
Department of Pediatric Cardiology, Erlangen University Hospital, Erlangen, Germany.
Thorac Cardiovasc Surg. 2022 Dec;70(S 03):e7-e14. doi: 10.1055/s-0042-1749098. Epub 2022 Jun 25.
The aim of this study was to evaluate the long-term outcome and freedom from pulmonary valve replacement (PVR) after initial repair of tetralogy of Fallot (TOF).
The cohort of 306 patients treated between 1980 and 2017 was divided into anatomical subgroups according to the diagnosis of TOF-pulmonary stenosis, TOF-pulmonary atresia and TOF-double outlet right ventricle. Patients were treated with transannular patch (TAP), valve sparing repair (VSR), or conduits from the right ventricle to the pulmonary arteries (RVPA conduits).
There were 21 deaths (6.9%), 14 being hospital deaths (4.6%) after primary correction and four deaths (1.3%) occurred after PVR. One patient died after a non-cardiac operation (0.3%). There were two late deaths (0.7%). During the past 12 years no early mortality has been observed. Ninety-one patients (30.4%) received PVR after a median of 12.1 ± 7.0 years with an early mortality of 4.4% ( = 4) and no late mortality. A significant difference in freedom from reoperation after TAP, VSR, and RVPA-conduits could be identified. Multivariate analysis displayed transannular repair ( = 0.016), primary palliation ( <0.001), the presence of major aortopulmonary collateral arteries (MAPCA; = 0.023), and pulmonary valve -scores < - 4.0 ( = 0.040) as significant risk factors for PVR.
TOF repair has a beneficial long-term prognosis with low morbidity and mortality. Pulmonary valve -scores < - 4.0, transannular repair, and presence of MAPCAs are associated with earlier PVR. Non-VSRs and TOF-pulmonary atresia lead to earlier reoperation but have no negative impact on survival.
本研究旨在评估法洛四联症(TOF)初次修复后长期的预后和免于肺动脉瓣置换(PVR)的情况。
1980 年至 2017 年间,我们根据 TOF-肺动脉瓣狭窄、TOF-肺动脉闭锁和 TOF-右心室双出口的诊断将 306 例患者分为解剖亚组。患者接受了瓣环成形环(TAP)、保留瓣膜修复(VSR)或右心室到肺动脉的管道(RVPA 管道)治疗。
共有 21 例死亡(6.9%),其中 14 例为初次矫正后的院内死亡(4.6%),4 例为 PVR 后死亡(1.3%)。1 例患者在非心脏手术后死亡(0.3%)。过去 12 年中没有观察到早期死亡。91 例患者(30.4%)在中位时间 12.1±7.0 年后接受了 PVR,早期死亡率为 4.4%( = 4),晚期无死亡。TAP、VSR 和 RVPA 管道的患者在免于再次手术方面有显著差异。多变量分析显示,跨瓣环修复( = 0.016)、初次姑息治疗( <0.001)、存在主-肺动脉侧支循环(MAPCA; = 0.023)和肺动脉瓣评分 < -4.0( = 0.040)是 PVR 的显著危险因素。
TOF 修复的长期预后良好,发病率和死亡率较低。肺动脉瓣评分 < -4.0、跨瓣环修复和存在 MAPCA 与更早的 PVR 相关。非 VSR 和 TOF-肺动脉闭锁导致更早的再次手术,但对生存没有负面影响。