Elami A, Milgalter E, Rein A J, Schwartz D, Yatsiv I
Dept. of Cardiothoracic Surgery, Hebrew University, Hadassah Medical School, Jerusalem.
Harefuah. 1996 Jul;131(1-2):1-3, 72.
Aortopulmonary (AP) window is an uncommon cardiac anomaly which is frequently associated with other cardiac anomalies. Concomitant repair of all intra- and extracardiac anomalies is usually recommended. Between October 1993 and July 1994, 4 patients aged 37 days, 4 weeks, 3.5 and 1.5 years, respectively, underwent repair of AP window and associated malformations. The associated anomalies consisted of interrupted aortic arch (IAA), and atrial and ventricular septal defects in the 1st patient, patent ductus arteriosus in the 2nd, inlet ventricular septal defect in the 3rd and congenital narrowing of the right pulmonary artery and a patent foramen ovale in the 4th. Extreme metabolic acidosis after repair of IAA in the 1st, dictated ligation of the AP window before repair of the intracardiac anomalies at 2 months of age. In the 2nd and 3rd, repair of the window and associated anomalies was performed in a single stage. In the 4th, left pulmonary artery banding preceded complete repair. All patients are doing well at 18, 13, 12 and 11 months, respectively, of follow-up after their last operation. Surgical management of AP window may have to be staged according to the physiological influence of the associated anomalies in each patient, to minimize the cumulative risk of complete repair.
主肺动脉窗是一种少见的心脏畸形，常与其他心脏畸形相关。通常建议同时修复所有心内和心外畸形。1993年10月至1994年7月，分别有4例年龄为37天、4周、3.5岁和1.5岁的患者接受了主肺动脉窗及相关畸形的修复手术。第1例患者的相关畸形包括主动脉弓中断（IAA）、房间隔和室间隔缺损；第2例为动脉导管未闭；第3例为流入道室间隔缺损；第4例为右肺动脉先天性狭窄和卵圆孔未闭。第1例患者在IAA修复后出现严重代谢性酸中毒，因此在2个月大时在心内畸形修复前先行主肺动脉窗结扎术。第2例和第3例患者的主肺动脉窗及相关畸形一期修复。第4例患者先进行左肺动脉束带术，然后再进行完全修复。在最后一次手术后分别随访18个月、13个月、12个月和11个月时，所有患者情况良好。主肺动脉窗的手术治疗可能需要根据每个患者相关畸形的生理影响进行分期，以将完全修复的累积风险降至最低。
