Department of Pediatric Cardiac Surgery, Necker Sick Children Hospital-M3C, University Paris Descartes, Paris, France.
Department of Pediatric Cardiology, Necker Sick Children Hospital-M3C, University Paris Descartes, Paris, France.
Eur J Cardiothorac Surg. 2019 Sep 1;56(3):541-548. doi: 10.1093/ejcts/ezz085.
A double orifice of the left atrioventricular valve (LAVV) associated with atrioventricular septal defects (AVSD) can significantly complicate surgical repair. This study reports our experience of AVSD repair over 3 decades, with special attention to the zone of apposition (ZoA) of the main orifice, and presents a technique of hemivalve pericardial extension in specific situations.
We performed a retrospective study from 1987 to 2016 on 1067 patients with AVSD of whom 43 (4%) had a double orifice, plus 2 additional patients who required LAVV pericardial enlargement. Median age at repair was 1.3 years. Mean follow-up was 8.2 years (1 month-32 years).
Associated abnormalities of the LAVV subvalvular apparatus were found in 7 patients (5 parachute LAVV and 2 absence of LAVV subvalvular apparatus). ZoA was noted in 4 patients (9%): partially closed in 15 (35%) and completely closed in 24 (56%). Four patients required, either at first repair or secondarily, a hemivalve enlargement using a pericardial patch without closure of the ZoA. The early mortality rate was 7% (n = 3), all before 2000. Two patients had unbalanced ventricles and the third had a single papillary muscle. There were no late deaths. Six patients (14%) required 7 reoperations (3 early and 4 late reoperations) for LAVV regurgitation and/or dysfunction, of whom 4 (9%) required mechanical LAVV replacement (all before 2000). Freedom from late LAVV reoperation was 97% at 1 year, 94% at 5 years and 87% at 10, 20 and 30 years. Unbalanced ventricles (P = 0.045), subvalvular abnormalities (P = 0.0037) and grade >2 LAVV postoperative regurgitation (P = 0.017) were identified as risk factors for LAVV reoperations. Freedom from LAVV mechanical valve replacement was 95% at 1 year, 90% at 5 years and 85% at 10, 20 and 30 years. An anomalous LAVV subvalvular apparatus was identified as a risk factor for mechanical valve replacement (P = 0.010). None of the patients who underwent LAVV pericardial extension had significant LAVV regurgitation at the last follow-up examination.
Repair of AVSD and double orifice can be tricky. Preoperative LAVV regurgitation was not identified as an independent predictor of surgical outcome. LAVV hemivalve extension appears to be a useful and effective alternate surgical strategy when the ZoA cannot be closed.
左房室瓣（LAVV）双孔与房室间隔缺损（AVSD）可显著增加手术修复的复杂性。本研究报告了我们在 30 多年间对 AVSD 修复的经验，特别关注主孔的贴附区（ZoA），并介绍了在特定情况下使用半瓣心包扩张的技术。
我们对 1987 年至 2016 年间 1067 例 AVSD 患者进行了回顾性研究，其中 43 例（4%）有双孔，另有 2 例需要 LAVV 心包扩大。修复时的中位年龄为 1.3 岁。平均随访时间为 8.2 年（1 个月至 32 年）。
发现 7 例（5 例降落伞型 LAVV 和 2 例 LAVV 瓣下结构缺失）存在 LAVV 瓣下结构异常。4 例（9%）存在 ZoA：15 例（35%）部分关闭，24 例（56%）完全关闭。4 例患者（均在 2000 年前）需要使用心包补片进行半瓣扩张，以扩大 LAVV，而不关闭 ZoA。早期死亡率为 7%（n=3），均在 2000 年前。2 例患者存在心室不平衡，第 3 例患者存在单一乳头肌。无晚期死亡。6 例（14%）因 LAVV 反流和/或功能障碍需要 7 次手术（3 次早期，4 次晚期），其中 4 例（9%）需要机械 LAVV 置换（均在 2000 年前）。1 年、5 年、10 年、20 年和 30 年时，无晚期 LAVV 再次手术的生存率分别为 97%、94%、87%、87%、87%。心室不平衡（P=0.045）、瓣下结构异常（P=0.0037）和术后 LAVV 反流程度>2 级（P=0.017）被确定为 LAVV 再次手术的危险因素。1 年、5 年、10 年、20 年和 30 年时，无机械 LAVV 置换的生存率分别为 95%、90%、85%、85%、85%。异常的 LAVV 瓣下结构被确定为机械瓣膜置换的危险因素（P=0.010）。最后一次随访时，接受 LAVV 心包扩张术的患者均无明显 LAVV 反流。
AVSD 和双孔修复可能比较棘手。术前 LAVV 反流未被确定为手术结果的独立预测因素。当 ZoA 无法关闭时，LAVV 半瓣扩张术似乎是一种有用且有效的替代手术策略。
