Shakibi J G, Rastan H, Nazarian I, Paydar M, Aryanpour I, Siassi B
Jpn Heart J. 1978 May;19(3):439-51. doi: 10.1536/ihj.19.439.
A 10-month-old boy is presented who had isolated unilateral absence of the right pulmonary artery. He suffered from hemoptysis and severe congestive heart failure. The patient underwent prosthetic anastomosis of the right to the main pulmonary artery. Although the hemodynamic response was favorable, his oxygenation did not improve due to diffuse pulmonary arteriovenous fistulae of the affected lung. The patient succumbed 3 months after operation due to massive uncontrollable hemoptysis from the right lung. Isolated unilateral absence of the pulmonary artery is a rare lesion. In our review of the world literature as of November 1976, 47 cases (including this report) of the unilateral absence of the pulmonary artery have been reported. Of these 25.5% had pulmonary hypertension and only 4 cases underwent successful repair of the lesion. Though repair of this defect can be carried out, the result may not be always gratifying. Our experience with this case has led us to consider a lung biopsy before proceeding to the surgical repair of the lesion. If the affected lung shows arteriovenous abnormalities the operation should not be recommended.
现报告一名10个月大的男孩，其右侧肺动脉孤立性缺如。他患有咯血和严重的充血性心力衰竭。该患者接受了右侧肺动脉与主肺动脉的人工吻合术。尽管血流动力学反应良好，但由于患侧肺弥漫性肺动静脉瘘，其氧合情况并未改善。患者术后3个月因右肺大量无法控制的咯血而死亡。孤立性单侧肺动脉缺如是一种罕见的病变。在我们截至1976年11月对世界文献的回顾中，已报道了47例（包括本报告）单侧肺动脉缺如的病例。其中25.5%患有肺动脉高压，只有4例病变修复成功。尽管可以对该缺陷进行修复，但结果可能并不总是令人满意。我们对该病例的经验使我们考虑在对病变进行手术修复之前进行肺活检。如果患侧肺显示动静脉异常，则不建议进行手术。
