Congenital disconnection of the pulmonary arteries

J. Karila Cohen, D. Bonnet
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Abstract

Introduction

Disconnected pulmonary artery is a rare anomaly that can be isolated or associated with complex intracardiac malformations. Early re-implantation of the disconnected pulmonary artery is recommended to allow growth and satisfactory pulmonary perfusion while preventing collateral arteries development.

Objective

To describe the characteristics of patients with disconnected pulmonary artery and, for those who had surgical re-implantation, to determine the incidence, delay and predictive factors of re-intervention for reconnected pulmonary artery stenosis.

Methods

We include patient with diagnosis of congenitally disconnected PA and surgical repair at our institution.

Results/Expected results

Retrospective observational study of 55 patients with a disconnected pulmonary artery. Fifty-one underwent surgical correction and were followed up at our institution between 2000 and 2022.

Disconnected pulmonary arteries were observed in isolation in 31% of the cases. The most frequent form was left pulmonary artery originating from the arterial duct (58%). The re-implantation was done at median age of 12 days. Anastomotic stenosis was observed during follow-up in 71% of the patients with 75% of them requiring re-intervention (55% of the population). The median delay to re-intervention was 3.2 years after re-implantation, and more than 25% of re-interventions on the re-implanted pulmonary artery occurred within the first postoperative year. We found more re-intervention if associated cardiac defect, without significant statistic difference. Weight at re-confluence, presence of ductal tissue at the origin of the pulmonary artery, and prior shunt placement on the disconnected pulmonary artery were not found to be risk factors for re-intervention (Figure 1).

Conclusion/Perspectives

After surgical re-implantation of pulmonary artery, more than 50% of patients required re-intervention for pulmonary artery stenosis. Technical improvements should be sought to reduce incidence of this complication.

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先天性肺动脉断裂
引言肺动脉断开是一种罕见的异常,可以孤立或与复杂的心内畸形相关。建议尽早重新植入断开的肺动脉,以允许生长和令人满意的肺灌注,同时防止侧支动脉发育。目的描述肺动脉断开患者的特点,并针对那些进行手术再植入的患者,确定再介入治疗肺动脉狭窄的发生率、延迟和预测因素。方法我们包括诊断为先天性PA断开并在我们机构进行手术修复的患者。结果/预期结果55例肺动脉断开患者的回顾性观察研究。2001年至2022年间,我们机构对51例患者进行了手术矫正和随访。31%的患者孤立地观察到肺动脉断开。最常见的形式是源自动脉导管的左肺动脉(58%)。在中位年龄12天时进行再次植入。71%的患者在随访期间观察到吻合口狭窄,其中75%的患者需要再次干预(占总人数的55%)。再植入后再干预的中位延迟时间为3.2年,超过25%的再植入肺动脉再干预发生在术后第一年内。我们发现,如果相关心脏缺陷,则会有更多的再次干预,没有显著的统计学差异。再次汇合时的体重、肺动脉起点处导管组织的存在以及之前在断开的肺动脉上进行分流均未被发现是再次干预的风险因素(图1)。结论/透视在肺动脉手术再次植入后,超过50%的患者因肺动脉狭窄需要再次干预。应寻求技术改进以减少这种并发症的发生率。
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来源期刊
Archives of Cardiovascular Diseases Supplements
Archives of Cardiovascular Diseases Supplements CARDIAC & CARDIOVASCULAR SYSTEMS-
自引率
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发文量
508
期刊介绍: Archives of Cardiovascular Diseases Supplements is the official journal of the French Society of Cardiology. The journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles, editorials, and Images in cardiovascular medicine. The topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Additionally, Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.
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