房室间隔缺损合并法洛四联症或双出口右心室的手术策略。
Surgical strategy for atrioventricular septal defect and tetralogy of Fallot or double-outlet right ventricle.
机构信息
Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, Minnesota, USA.
出版信息
Ann Thorac Surg. 2013 Jun;95(6):2079-84; discussion 2084-5. doi: 10.1016/j.athoracsur.2013.02.016. Epub 2013 Apr 18.
BACKGROUND
Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period.
METHODS
From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt.
RESULTS
Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups.
CONCLUSIONS
Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.
背景
法洛四联症，或右心室双出口伴房室（AV）间隔缺损（TOF/DORV-AVSD）较为罕见，目前仅有有限的长期数据。我们报告了我们机构在 50 年期间的经验和结果。
方法
从 1961 年 1 月至 2011 年 1 月，73 例（50 名男性[68%]）患者，平均年龄为 6.8 ± 4.4 岁（范围，1 个月至 35 岁），接受了 TOF/DORV-AVSD 的手术修复。症状包括 50 例（69%）发绀和 12 例（17%）心力衰竭。25 例（34%）患者合并唐氏综合征。Rastelli 分型 A、B 和 C 分别见于 12%、7%和 81%的患者。40%的患者存在中度或更常见的房室瓣（AVV）反流。49 例（67%）患者有过既往姑息治疗，包括 36 例接受了体肺分流术。
结果
手术治疗包括 35 例（48%）进行两心室完全修复（CR）和 38 例（52%）进行单心室（SV）姑息治疗。总体而言，CR 的早期死亡率为 31%，SV 为 34%；1990 年后，CR 的死亡率为 6%，SV 为 14%。1990 年前的修复（p = 0.008）和存在明显的共同 AVV 反流（p = 0.016）是两组患者早期死亡的单因素危险因素。中位随访时间为 9.8 年（最长 32 年）。CR 的晚期死亡率为 6%（n = 6），SV 为 18%（n = 9）（p = 0.95）。存在明显的右 AVV 反流与晚期死亡相关（p = 0.02）。CR 的总体生存率在 1 年、5 年和 15 年分别为 92%、77%和 77%，SV 为 83%、79%和 70%（p = 0.9）。CR 的 1 年、5 年和 15 年无再手术生存率分别为 95%、85%和 67%，SV 分别为 96%、91%和 82%（p = 0.1）。再手术最常见的原因是右心室流出道病变、Fontan 修正术和 AVV 干预。右 AVV 反流（p = 0.018）和 1990 年前的修复（p = 0.041）是两组患者晚期再手术的危险因素。
结论
TOF/DORV-AVSD 的完全修复是目前的标准治疗方法，在当前时代，其早期死亡率较低，具有合理的长期结果。SV 姑息治疗仍存在显著风险。AVV 反流是死亡和再手术的重要危险因素。
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