Velasco Alvarez P D, González Cerna J L
Bol Med Hosp Infant Mex. 1976 Sep-Oct;33(5):1085-98.
Three patients with interruption and seven with hypoplasia of the aortic arch were treated surgically. The subclavian artery and the aortic isthmus were employed for reconstructing the aortic arch in five, and a Dacron prosthesis was used to restore the aortic continuity in five. A ductus arteriosus coexisted in all patients and a ventricular or atrial septal defect in nine. Congestive heart failure and pulmonary hypertension were prominent clinical features, and the role of the ductus and other intracardiac anomalies on their pathogenesis in discussed. Six patients, one with an interrupted and five with a hypoplastic arch survived but three have evidence of either pulmonary vascular disease or significant pulmonary hypertension. Only one patient with hypoplasia of the arch is now considered cured after his ventricular septal defect was closed in a second operation. The analysis of this and other series in the literature indicate a high mortality rate of the conditions; however, early surgical treatment, which is the only effective means to control heart failure and avoid the effects of prolonged pulmonary hypertension, has brought upon a decline in mortality in recent years.
三名主动脉弓中断和七名主动脉弓发育不全的患者接受了手术治疗。五名患者采用锁骨下动脉和主动脉峡部重建主动脉弓，五名患者使用涤纶人工血管恢复主动脉连续性。所有患者均并存动脉导管未闭，九名患者并存心室或房间隔缺损。充血性心力衰竭和肺动脉高压是突出的临床特征，并讨论了动脉导管及其他心内异常在其发病机制中的作用。六名患者存活，其中一名主动脉弓中断，五名主动脉弓发育不全，但三名有肺血管疾病或重度肺动脉高压的证据。只有一名主动脉弓发育不全的患者在第二次手术中关闭室间隔缺损后目前被认为已治愈。对本系列及文献中其他系列的分析表明这些病症的死亡率很高；然而，早期手术治疗作为控制心力衰竭和避免长期肺动脉高压影响的唯一有效手段，近年来已使死亡率有所下降。
