Kuniyoshi Y, Koja K, Iha K, Akasaki M, Miyagi K, Kusaba A
Second Department of Surgery, Faculty of Medicine, University of Ryukyus, Okinawa, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1994 Apr;42(4):598-602.
Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation, which is usually fatal without early surgical correction. The number of reports of the radical operation has been recently increasing, but reports of its long-term postoperative results are rare, especially those of the reoperative cases. The 14-year-old patient, who had been operated radically with a 8 mm diameter graft at 7 months of age, weighing 4550 g at the time, was reoperated, because he developed pulmonary hypertension due to the narrowing of the graft. He had no clinical symptoms and no abnormal signs on chest X-P nor ECG, eventhough severe stenosis of the graft was present. The graft was replaced with a larger 12 mm diameter graft under ECC, and PFO closure and TAP were done at the same time. The patient's pulmonary artery pressure reduced substantially following surgery. We conclude that cautious postoperative follow-up including angiographical examination is important, and that early reoperation before occlusion of the graft should be done.
右肺动脉起源于升主动脉是一种罕见的先天性畸形,若不早期手术矫正通常会致命。根治性手术的报告数量最近有所增加,但关于其术后长期结果的报告很少,尤其是再次手术病例的报告。该14岁患者在7个月大时接受了直径8毫米移植物的根治性手术,当时体重4550克,因移植物狭窄导致肺动脉高压而接受再次手术。尽管存在移植物严重狭窄,但他没有临床症状,胸部X线和心电图也没有异常体征。在体外循环下用直径更大的12毫米移植物替换了移植物,同时进行了卵圆孔未闭封堵术和三房心手术。术后患者的肺动脉压力大幅降低。我们得出结论,包括血管造影检查在内的谨慎术后随访很重要,并且应在移植物闭塞前尽早进行再次手术。