法洛四联症全修复的长期结果。

The long-term result of total repair for tetralogy of Fallot.

机构信息

Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Bucheon, South Korea.

出版信息

Eur J Cardiothorac Surg. 2010 Sep;38(3):311-7. doi: 10.1016/j.ejcts.2010.02.030. Epub 2010 Mar 25.

DOI:10.1016/j.ejcts.2010.02.030

PMID:20346688

Abstract

OBJECTIVE

The objective of this study was to evaluate the long-term outcome of total repair for tetralogy of Fallot.

METHODS

Between April 1986 and December 2007, a total of 734 patients underwent total repair for tetralogy of Fallot. There were 444 males and 290 females. The median age and weight were 17.2 months (0.4-329.6 months) and 9.5kg (2.6-53.5kg). The median follow-up duration was 150.2 months (1.9-356.2 months).

RESULTS

There were 27 early deaths (3.7%) and 13 late deaths. A longer cardiopulmonary bypass time and the use of total circulatory arrest were risk factors for early death. The overall survival rate was 94.8%, 92.8% and 92.8% at 10, 20 and 25 years, respectively. The presence of pulmonary atresia was a risk factor for long-term survival. Re-operation or re-intervention was required in 224 patients (31.7%). The most common causes of re-operation or re-intervention were pulmonary regurgitation in 109 patients and branch pulmonary artery stenosis in 127 patients. Freedom from re-operation or re-intervention rate was 81.5%, 68.9% and 46.6% at 5, 10 and 20 years, respectively. Reconstruction of the right ventricular outflow tract with other than non-trans-annular repair and branch pulmonary arterioplasty at the time of total repair were the risk factors for late re-operation or re-intervention. The use of a monocusp patch was not associated with early mortality or re-operation. At the latest follow-up, most patients were in the New York Heart Association functional class 1 or 2.

CONCLUSIONS

The long-term outcome of total repair for tetralogy of Fallot was satisfactory. A longer cardiopulmonary bypass time and the use of deep hypothermic circulatory arrest were associated with early mortality. The patients with pulmonary atresia have poorer late survival. Preservation of the pulmonary annulus can reduce the re-operation rate. A small pulmonary artery that requires augmentation may increase the risk of re-operation. The use of a monocusp in patients who underwent trans-annular repair has no benefit for early survival, the postoperative recovery and avoidance of re-operation. Age was not a risk factor for early mortality and re-operation.

摘要

目的

本研究旨在评估法洛四联症全修复的长期结果。

方法

1986 年 4 月至 2007 年 12 月，共有 734 例法洛四联症患者接受全修复治疗。其中男性 444 例，女性 290 例。中位年龄和体重分别为 17.2 个月（0.4-329.6 个月）和 9.5kg（2.6-53.5kg）。中位随访时间为 150.2 个月（1.9-356.2 个月）。

结果

早期死亡 27 例（3.7%），晚期死亡 13 例。体外循环时间较长和使用全循环阻断是早期死亡的危险因素。总体生存率分别为 10、20 和 25 年时的 94.8%、92.8%和 92.8%。肺动脉闭锁是长期生存的危险因素。224 例患者需要再次手术或再次介入治疗（31.7%）。最常见的再次手术或再次介入治疗原因是 109 例患者的肺动脉瓣反流和 127 例患者的分支肺动脉狭窄。5、10 和 20 年时无再次手术或再次介入治疗的比例分别为 81.5%、68.9%和 46.6%。全修复时采用非跨瓣环修复和分支肺动脉成形术进行右心室流出道重建是晚期再次手术或再次介入治疗的危险因素。使用单瓣叶补片与早期死亡率或再次手术无关。在最近的随访中，大多数患者处于纽约心脏协会功能分级 1 或 2 级。

结论

法洛四联症全修复的长期结果令人满意。体外循环时间较长和使用深低温循环阻断与早期死亡率相关。肺动脉闭锁的患者晚期生存率较差。保留肺动脉瓣环可降低再次手术的发生率。需要增强的较小肺动脉可能增加再次手术的风险。在接受跨瓣环修复的患者中使用单瓣叶补片对早期生存、术后恢复和避免再次手术没有益处。年龄不是早期死亡率和再次手术的危险因素。