42例婴幼儿完全性肺静脉异位引流的治疗体会

Experience of Treatment for Total Anomalous Pulmonary Venous Connection in Forty-Two Infants

目的:总结婴幼儿完全性肺静脉异位引流的早期诊断和治疗经验。方法:1995年3月至2005年3月,我院共完成42例完全性肺静脉异位引流的手术治疗,男33例,女9例,年龄18天～3岁,平均(4.3±3.2)月,体重3.2～11kg,平均(5.4±1.2)kg。心上型27例,心内型10例,心下型3例,混合型2例。所有患者均由超声心动图确诊,11例行心导管检查,3例心下型行磁共振成像检查。均在全麻低温体外循环下行矫治术。6例心上型采用左心房顶部入路。结果:死亡4例,死亡率9.52%(3例心上型围术期死亡,1例心内型术后1年因肺静脉回流梗阻死亡)。3例心下型无死亡。心律失常7例中,6例为心上型双心房横切口,1例为心内型。随访39例,随访时间1月～10年,存活者心功能明显改善。结论:完全性肺静脉异位引流可由超声心动图确诊,必要时辅以心导管检查及磁共振成像检查。新生儿及婴儿期手术可获得良好效果。心上法矫治心上型可减少心律失常的发生率。

Objective：To review the experience of the early diagnosis and treatment of total anomalous pulmonary venous connection （TAPVC） in infants. Methods： Between March 1995 and March 2005,42 infants underwent surgical correction of TAPVC. There were 33 males and 9 females with mean age of（4.3 ± 3.2） months （ 18 days to 3 years） and mean weight of（5.4 ±1.2） kg （3.2-11 kg）. All patients were diagnosed by echocardiography. There were 27 supracardiac, 10 cardiac, 3 infracardiac, and 2 mixed types. Cardiac catheterization was performed in 11 patients,and 3 infracardiac patients received MRI examination. The total correction was performed with cardiopulmonary bypass. Six supracardiac patients were operated on through the top approach of left atrium. Results：There were 4 deaths with a mortality of 9. 52%. Three supracardiac patients died perioperatively. One cardiac patient died of pulmonary venous obstruction 1 year afteroperation. Three inffracardiac patients survived. Arrhythmia occurred in 7 patients,6 had transverse atrial incisions, and one was of cardiac type. Thirty-nine patients were followed up for 1 month to 10 years. All survivors＇ cardiac function were improved. Conclusion： The diagnosis of TAPVC can be confirmed by echocardiography. When in need, cardiac catheterization and MRI may be performed. Surgical repair of TAPVC is conducted as soon as possible. The superior approach for correction of supracardiac type of TAPVC can reduce postoperative morbidity from arrhythmia.