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法洛四联症合并肺动脉闭锁的外科治疗

Surgical treatment of tetralogy of Fallot with pulmonary atresia.

作者信息

Alfieri O, Blackstone E H, Kirklin J W, Pacifico A D, Bargeron L M

出版信息

J Thorac Cardiovasc Surg. 1978 Sep;76(3):321-35.

PMID:682664
Abstract

Thirteen (16 percent) of 80 patients with tetralogy of Fallot and pulmonary atresia undergoing corrective operations between Jan. 1, 1967, and Jan. 1, 1978, died in the hospital. The hospital mortality rate was 13 percent (10 deaths) among the 77 patients with confluent right and left pulmonary arteries. The risk of operation was not significantly affected by age at operation or by use of a valved external conduit versus a transannular outflow patch. It was affected (p = 0.008) by the ratio of peak right ventricular to left ventricular pressure (PRV/LV) immediately after repair. This (PRV/LV) was determined primarily by size of left and right pulmonary arteries. An equation was developed relating postrepair PRV/LV to diameter of right and left pulmonary artery (normalized by dividing by size of descending thoracic aorta), body surface area, and possible arborization abnormalities and stenoses of the right and left pulmonary arteries. Cardiac performance after repair was better in those in whom a transannular patch was used rather than a valved external conduit. Important pulmonary dysfunction postoperatively occurred more often in patients with large "bronchial" arteries than in those without them, but was less when these were not ligated. Four (8 percent) of 48 traced hospital survivors died late postoperatively. Reoperations late postoperatively were required only in patients receiving valved external conduits. Forty (91 percent) of 44 living traced patients are asymptomatic.

摘要

1967年1月1日至1978年1月1日期间接受矫治手术的80例法洛四联症合并肺动脉闭锁患者中,有13例(16%)死于医院。在77例左右肺动脉汇合的患者中,医院死亡率为13%(10例死亡)。手术风险不受手术时年龄或使用带瓣外管道与经环流出补片的显著影响。它受修复后即刻右心室与左心室压力峰值之比(PRV/LV)的影响(p = 0.008)。该比值(PRV/LV)主要由左右肺动脉的大小决定。建立了一个方程,将修复后的PRV/LV与左右肺动脉直径(通过除以降主动脉大小进行标准化)、体表面积以及左右肺动脉可能的分支异常和狭窄相关联。使用经环补片而非带瓣外管道的患者修复后的心脏功能更好。术后重要的肺功能障碍在有粗大“支气管”动脉的患者中比没有的患者更常见,但在未结扎这些动脉时发生率较低。48例追踪到的医院幸存者中有4例(8%)术后晚期死亡。术后晚期再次手术仅发生在接受带瓣外管道的患者中。44例存活且被追踪的患者中有40例(91%)无症状。

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引用本文的文献

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Definition and Management of Segmental Pulmonary Hypertension.节段性肺动脉高压的定义与管理
J Am Heart Assoc. 2018 Jul 4;7(14):e008587. doi: 10.1161/JAHA.118.008587.
3
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Effect of systemic pulmonary anastomosis.
室间隔缺损并主-肺动脉侧支动脉的肺动脉闭锁。体肺吻合术的效果。
Br Heart J. 1981 Feb;45(2):133-41. doi: 10.1136/hrt.45.2.133.
4
Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.室间隔缺损合并主-肺动脉侧支动脉的肺动脉闭锁患者肺循环的生长与发育
Br Heart J. 1980 Jul;44(1):14-24. doi: 10.1136/hrt.44.1.14.
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Duplicate sources of pulmonary blood supply in pulmonary atresia with ventricular septal defect.室间隔缺损合并肺动脉闭锁时肺动脉供血的重复来源
Br Heart J. 1981 Sep;46(3):263-8. doi: 10.1136/hrt.46.3.263.
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Hypoplasia of the intrapulmonary arteries in children with right ventricular outflow tract obstruction, ventricular septal defect, and major aortopulmonary collateral arteries.
Pediatr Cardiol. 1985;6(3):137-43. doi: 10.1007/BF02336552.
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