Agarwal Abhinav, Al Amer Suad R, Al Tarif Habib, Ismael Aieshah Ahmed, Alshaiji Abdulla Faisal, Arulselvam Vimalarani, Kalis Neale Nicola
Mohammed Bin Khalifa Bin Salman Al-Khalifa Cardiac Center, Bahrain Defense Forces Hospital, Awali, Kingdom of Bahrain.
Department Pediatric Cardiology, Royal College of Surgeons of Ireland - Medical University of Bahrain, Muharraq, Kingdom of Bahrain.
Heart Views. 2022 Apr-Jun;23(2):78-85. doi: 10.4103/heartviews.heartviews_77_21. Epub 2022 Jul 23.
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Surgical correction has improved survival but re-intervention is often required.
The objective is to assess outcomes after surgical repair of TOF, long-term follow-up, and factors that influence these results.
This is a retrospective study conducted in a tertiary care center. Records of patients diagnosed with TOF from 1992 to 2019 (37 years) were retrieved from a detailed database. Patients who underwent complete correction were grouped according to diagnosis, the technique utilized in surgical repair, need for staged repair, and syndromic association. Univariate actuarial and event-free survival analysis was performed. The endpoint for an event was death or re-intervention.
A total of 230 patients were diagnosed with TOF and 174 patients underwent complete surgical repair. At 40 years postoperatively, survival was 96%. Actuarial survival was independent of syndromic associations, anatomical diagnosis, type of surgery, or previous shunt. Event-free survival (EFS) survival was 8.12%. EFS was significantly worse for patients with pulmonary atresia (PA) (Hazard ratio, 4.1125; 95% confidence interval [CI], 1.2654-13.3657; < 0.0001) and for those that required homograft/conduit. The median duration for EFS was 22.73 years, 19.58 years, and 9.12 years for transannular patch (TAP), pulmonary valve-sparing (PVS), and homograft group, respectively. The survival curve for the PVS group merged with that of TAP 20 years postoperatively. Similarly, it merged at 22 years for staged versus primary repair and at 22.73 years for syndromic versus nonsyndromic patients. A weak correlation was found between age at surgery and event-free duration (cc, 0.309; < 0.0001). The need for TAP was not influenced by the previous palliation, χ(1, = 154) = 3.36, = 0.0667, or with interval to complete correction after the shunt procedure ( = 0.9672).
Total correction of TOF has low perioperative mortality and good long-term survival, but the need for re-interventions is high. This study demonstrated that patients requiring homograft/conduit and those with a diagnosis of PA had worse outcomes. Comparison between different surgical groups showed merging of survival curves in follow-up that signifies gradual loss of survival advantage over time.
法洛四联症（TOF）是最常见的青紫型先天性心脏病。手术矫正提高了生存率，但通常仍需要再次干预。
目的是评估TOF手术修复后的结果、长期随访情况以及影响这些结果的因素。
这是一项在三级医疗中心进行的回顾性研究。从详细数据库中检索了1992年至2019年（37年）诊断为TOF的患者记录。接受完全矫正的患者根据诊断、手术修复所采用的技术、分期修复的需求以及综合征关联进行分组。进行了单变量精算和无事件生存分析。事件的终点是死亡或再次干预。
共有230例患者被诊断为TOF，174例患者接受了完全手术修复。术后40年时，生存率为96%。精算生存率与综合征关联、解剖诊断、手术类型或既往分流无关。无事件生存率（EFS）为8.12%。肺动脉闭锁（PA）患者的EFS明显更差（风险比，4.1125；95%置信区间[CI]，1.2654 - 13.3657；P < 0.0001），以及那些需要同种异体移植物/导管的患者。经环补片（TAP）、保留肺动脉瓣（PVS）和同种异体移植物组的EFS中位持续时间分别为22.73年、19.58年和9.12年。PVS组的生存曲线在术后20年与TAP组的曲线合并。同样，分期修复与一期修复在22年时合并，综合征患者与非综合征患者在22.73年时合并。手术年龄与无事件持续时间之间存在弱相关性（cc，0.309；P < 0.0001）。TAP的需求不受既往姑息治疗的影响（χ(1, n = 154) = 3.36，P = 0.0667），也与分流手术后至完全矫正的间隔时间无关（P = 0.9672）。
TOF的完全矫正围手术期死亡率低且长期生存率良好，但再次干预的需求较高。本研究表明，需要同种异体移植物/导管的患者以及诊断为PA的患者预后较差。不同手术组之间的比较显示随访中生存曲线合并，这表明随着时间推移生存优势逐渐丧失。
