成人左冠状动脉起源于肺动脉外科治疗

Surgical treatment of adult patients with anomalous left coronary artery from the pulmonary artery

目的探索成人左冠状动脉起源于肺动脉的外科治疗效果。方法 1991年11月至2017年11月,手术治疗36例年龄>18岁左冠状动脉起源于肺动脉患者,其中男9例、女27例,年龄(36.6±13.3)岁,体重(60.0±9.4)kg。术前超声心动图显示左心室射血分数(LVEF)为57%±6%,左心室舒张期末内径(LVEDD)为(52.3±6.3)mm;二尖瓣反流(MR)重度1例,中度5例。其中行冠状动脉再植17例,肺动脉内隧道修补术(Takeuchi术)16例,异常起源左冠状动脉结扎+冠状动脉旁路移植术3例,同期行二尖瓣成形术6例。结果体外循环时间为(152.5±72.9)min,主动脉阻断时间(101.9±43.6)min,无院内死亡,呼吸机辅助时间(17.3±16.3)h,ICU滞留时间(43.1±30.7)h,术后LVEF为59%±6%,较术前无明显改善(P=0.10),术后LVEDD为(46.9±5.9)mm较术前显著缩小(P=0.02),6例二尖瓣成形患者中,1例重度变为轻度,5例中度变为微量。所有患者顺利出院。随访35例,平均5.5年,最长26年,随访期间无死亡,2例行肺动脉内隧道修补术患者发生肺动脉内隧道瘘,分别成功行介入封堵术和外科修补术;所有患者心功能分级(NYHA)Ⅰ或Ⅱ级;最后一次随访LVEF为69%±7%,较术前显著改善,LVEDD为(48.7±5.9)mm;MR中度2例,轻度10例。结论成人左冠状动脉起源于肺动脉外科治疗近期和远期效果满意,肺动脉内隧道修补术远期可能出现内隧道瘘需要再次干预。

Objective To review the experience of the surgical treatment of adult patients with anomalous left coronary artery from the pulmonary artery(ALCAPA). Methods A retrospective, single institution review was conducted on thirty-six adult patients with ALCAPA surgical treatment from November 1991 to November 2017 in Fuwai Hospital. Of these patients, nine were males and twenty-seven were females. The mean age was 36.6±13.3 years. The mean weight was 60.0±9.4 kg. The preoperative echocardiography showed the mean left ventricular ejection fraction(LVEF) was57%±6% and the mean left ventricular end-diastolic dimension(LVEDD) was 52.3±6.3 mm. Severe mitral regurgitation(MR) was seen in one patient, moderate in five patients. The operative procedures included coronary artery reimplantation in seventeen patients, Takeuchi operation in sixteen patients, ligation of left coronary artery plus coronary artery bypass graft in three patients. In addition, six patients underwent mitral valve repair. Results There was no inhospital mortality. The mean cardiopulmonary bypass time was 152.5±72.9 min and aortic cross clamp time was101.9±43.6 min, respectively. The mean mechanical ventilation time and ICU time was 17.3±16.3 h and 43.1±30.7 h,respectively. The mean postoperative LVEF was 59%±6%, which did not significantly improve compared with preoperative LVEF. However, the mean postoperative LVEDD of 46.9±5.9 mm had significant reduction compared with the preoperative LVEDD. Of the six patients with mitral valve repair, one was mild and the other five were trivial. Thirtyfive patients(97.2%) completed the follow-up with a mean time of 5.5 years. All the patients survived with New York Heart Association class Ⅰor Ⅱ. Two patients needed interventional occlusion or re-operation due to the fistula of internal tunnel within the pulmonary artery. At the latest echocardiography, the mean LVEF of 69%±7% improved significantly compared with the preoperative LVEF. Mild MR was detected in ten patients, moderate in two patients during the followup period. Conclusion The surgical treatment of adult patients with ALCAPA has satisfactory short-and long-term results. The patients who underwent Takeuchi procedure may need re-operation due to fistula of internal tunnel within the pulmonary artery during the long-term follow-up.