Caffarena Calvar J M, Gómez Ullate J M, Caffarena Raggio J M, Sáez Palacios J M, Malo Concepción P
Servicio de Cirugía Cardiovascular, Hospital Infantil La Fe, Valencia.
Rev Esp Cardiol. 1992 Feb;45(2):145-8.
Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation associated with a high mortality during the first year of life. Up to 1990, 75 cases have been reported in the literature. We are reporting 2 cases diagnosed and operated before 3 months of age. Repair was facilitated by the use of deep hypothermic circulatory arrest. The duration of follow up is 5 and 6 years, respectively. Pre and postoperative cardiac catheterization pressure data and angiograms are shown. We review the literature, presenting the physiopathological, embryological and surgical features of this rare congenital anomaly.
右肺动脉起源于升主动脉是一种罕见的先天性畸形，与出生后第一年的高死亡率相关。截至1990年，文献中已报道75例。我们报告2例在3个月龄前诊断并接受手术的病例。采用深低温停循环有助于修复手术。随访时间分别为5年和6年。展示了术前和术后的心导管压力数据及血管造影照片。我们回顾文献，介绍这种罕见先天性异常的生理病理、胚胎学及手术特点。
