Gupta Umang, Polimenakos Anastasios C, El-Zein Chawki, Ilbawi Michel N
Rush Center for Congenital and Structural Heart Diseases, Rush University Medical Center, Chicago, IL, USA.
Pediatr Cardiol. 2013 Apr;34(4):861-71. doi: 10.1007/s00246-012-0558-3. Epub 2012 Oct 27.
Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7-13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P < 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.
据报道，法洛四联症（TOF）合并完全性房室间隔缺损（CAVSD）的修复手术具有良好的早期和中期效果。然而，发病率仍然很高。迄今为止，采用保留肺动脉瓣技术（PVS）修复CAVSD/TOF以及无需再次进行瓣膜手术的情况尚未明确。为此进行了一项研究以调查其结果。本研究为回顾性调查。在1988年1月至2008年12月期间，连续确定了13例CAVSD/TOF患者，并对他们的记录进行了回顾性分析。在这13例患者中，9例为Rastelli C型CAVSD。9例（69%；7例为C型）存在21三体综合征。5例患者在平均年龄1.7±0.6个月时接受了完全修复前的体肺分流术（SPS）。所有患者均存活至完全修复。在完全修复CAVSD/TOF时，所有患者均采用双补片技术矫正房室间隔缺损。8例（61.5%）患者采用了PVS。其余5例患者采用了跨环补片（TAP）修复。完全修复时的平均年龄为6.3±2.4个月。完全修复时，平均体外循环时间为173.5±30.6分钟，主动脉阻断时间为134.7±28.8分钟。住院1次，无晚期死亡病例。中位随访期为9.2年[四分位间距（IQR），4.7 - 13.3年]。1年、5年和8年的精算生存率分别为90.0±9.5%、90±9.5%和90±9.5%。在12例存活患者中，6例在随访期间进行了一些再次干预。在完全修复后的前11年内，2例患者接受了左房室（AV）瓣修复，1例患者接受了右AV瓣修复。2例患者进行了残余室间隔缺损封堵。4例患者因严重功能不全或狭窄平均在6±21个月时首次接受右心室流出道（RVOT）再次干预。1例患者进行了第二次RVOT再次手术。结果显示，采用PVS的CAVSD/TOF与显著更高的无RVOT再次干预率相关（1年、5年和8年时为100%，而采用TAP的CAVSD/TOF在1年时为80±17.9%，5年时为60±21.9%，8年时为40±21.9%；P<0.05）。接受PVS的患者中没有出现需要再次手术的左心室流出道梗阻。该组患者1年、5年和8年的总体无再次干预率分别为90.9±8.6%、71.6±14.0%和53.7±8.7%。矫正合并CAVSD的TOF可在低风险下进行，中期生存率良好，再次手术率令人满意。几乎三分之二的患者可避免使用TAP，这可能会影响早期RVOT再次干预的发生率。
