Vida Vladimiro L, Sanders Stephen P, Bottio Tomaso, Maschietto Nicola, Rubino Maurizio, Milanesi Ornella, Stellin Giovanni
Department of Pediatric Cardiac Surgery, University of Padova Medical School, 35128 Padova, Italy.
Cardiol Young. 2005 Apr;15(2):176-81. doi: 10.1017/S1047951105000363.
We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly.
Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient.
At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation.
Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.
我们描述了3例患有一支肺动脉发自升主动脉、另一支动脉正常发自右心室经肺动脉干的婴儿的手术修复情况。修复包括将异常肺动脉重新植入肺动脉干，并同时修复相关的心内畸形。所有患者均在手术过程中存活，并在临床状况稳定后出院。随后，3例患者中有2例在初次手术后相对较早地出现了手术吻合口狭窄，并接受了再次手术。虽然现在预计这种畸形手术后可以存活，但缺乏远期结果的报告。在得出明确结论之前，需要进行更多的手术。在存在肺动脉瓣和主肺动脉的情况下，一支分支肺动脉发自升主动脉是一种非常罕见的先天性心脏异常。
1995年1月至2003年6月期间，3例女婴表现为一支分支动脉发自升主动脉，而另一支肺动脉发自与右心室流出道相连的肺动脉干。发自右心室的肺动脉在2例患者中位于左侧，在1例患者中位于右侧。
患者分别在13、48和62天时接受了手术修复，包括将异常肺动脉分支重新植入肺动脉干，并同时修复心内畸形。无医院死亡病例。术后并发症包括：2例患者（分别为10天和16天）插管时间延长，1例患者出现低心排综合征，1例患者出现心包填塞，1例患者出现癫痫发作。所有患者均在临床状况良好的情况下出院。无晚期死亡病例。随后，3例患者中有2例在初次手术后相对较早地出现了手术吻合口狭窄，在球囊扩张失败后，接受了手术再次治疗。
虽然现在这种畸形手术可以存活，但缺乏远期结果的报告。3例患者中有2例在手术后相对较早地出现了手术吻合口狭窄。需要进行更多的手术才能得出明确结论。
