Atrioventricular septal defect and tetralogy of Fallot: a 15-year experience.
Atrioventricular septal defect and tetralogy of Fallot: a 15-year experience.
复制标题
房室间隔缺损和法洛四联症：15 年经验。
DOI：
10.1016/s1010-7940(97)00165-6
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发表时间：
1997
期刊：
影响因子：
--
通讯作者：
M. Leval
中科院分区：
文献类型：
--
作者：
Ralph E. Delius;R. V. Kumar;M. Elliott;J. Stark;M. Leval
目的：房室间隔缺损和法洛四联症是一种相对少见的病变，存在与进、出口瓣膜问题相关的右室功能障碍的风险。出于这个原因，通常选择保守治疗，包括最初的姑息治疗。本报告的目的是回顾我们在这一病变上的经验。患者群：1980年1月至1995年6月，本中心共收治房室间隔缺损和法洛四联症患者35例。其中21例(60%)接受了28次初步姑息性分流手术。14名患者(40%)接受了初步确定的修复。选择一种治疗策略的标准基于许多因素，包括发病时的年龄、病变的解剖结构和症状的严重性。在最初接受分流手术的21名患者中，15名已经接受了最终的手术。在6例未接受明确手术的患者中，3例死亡(2例与分流手术并发症直接相关)，2例正在等待手术，1例失访。结果：所有患者的手术指征均为青紫。术后5年未再手术者占65.1%，再手术多与左房室瓣返流或室间隔缺损区残余漏有关。所有患者最终手术死亡率为10.3%(70%CL 4.5~20%)。所有患者最终修复后7年生存率的精算估计值为77.3%(70%CL 68.7-85.9%)。如果包括姑息性分流手术的死亡率，一期修补术患者的7年生存率精算估计为84.4%(70%CL 73.8-95%)，而初步缓解的患者为65%(70%CL 52.4-77.6%)(P=0.35)。结论：房室间隔缺损合并法洛四联症患者可采用多种手术方法成功治疗。对于精心选择的患者，一期修复可能是一个合理的选择，因为这消除了最初分流手术的发病率和死亡率，以及随后最初缓解和最终修复之间的间隔时间。
AIM: Atrioventricular septal defect and tetralogy of Fallot is a relatively uncommon lesion in which there is a risk of right ventricular dysfunction related to inlet and outlet valve problems. For this reason, conservative management involving an initial palliative procedure is often chosen. The aim of this report is to retrospectively review our experience with this lesion. PATIENT POPULATION: 35 patients with atrioventricular septal defect and tetralogy of Fallot have been surgically managed at this institution between January 1980 and June 1995. Twenty-one (60%) of these patients underwent 28 initial palliative shunt procedures. Fourteen (40%) patients underwent primary definitive repair. The criteria for choosing one management strategy over another was based on a number of factors, including age at presentation, anatomy of the lesion, and severity of symptoms. Of the 21 patients who underwent an initial shunt procedure, 15 have undergone definitive operation. Of the 6 patients who did not undergo definitive operation, three died (two directly related to complications of the shunt procedure), two are awaiting operation, and one was lost to follow-up. RESULTS: The primary indication for operation in all patients was cyanosis. Freedom from reoperation at 5 years after definitive operation was 65.1% for all patients; most reoperations were related to left atrioventricular valve regurgitation or residual leaks across the ventricular septal defect patch. The operative mortality at definitive operation was 10.3% (70% CL 4.5-20%) for all patients. The actuarial estimate of survival 7 years following definitive repair was 77.3% (70% CL 68.7-85.9%) for all patients. The actuarial estimate of survival at 7 years was 84.4% (70% CL 73.8-95%) in the patients undergoing primary repair and 65% (70% CL 52.4-77.6%) in patients initially palliated if the mortality of the palliative shunt procedure is included (P = 0.35). CONCLUSION: Patients with atrioventricular septal defect and tetralogy of Fallot can be successfully managed with a variety of surgical strategies. Primary repair may be a reasonable option in carefully selected patients, as this eliminates the morbidity and mortality of an initial shunt procedure and the subsequent interval between initial palliation and definitive repair.