Ikai Akio
Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, 860 Urushiyama Aoi-ku, Shizuoka, 420-8660, Japan.
Gen Thorac Cardiovasc Surg. 2018 Jul;66(7):390-397. doi: 10.1007/s11748-018-0948-4. Epub 2018 May 25.
Treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is challenging and controversial. Basically, the collateral arteries are treated surgically by unifocalization to integrate them and unify the blood flow in the pulmonary circulation. These major collateral arteries are anatomically similar to the bronchial arteries; however, they develop into different vessels because of different environmental exposures. Currently, treatment plans involving surgical intervention in early infancy to address the multiple variations of major aortopulmonary collateral arteries are being established to achieve definitive intracardiac repair. Historically, several surgical approaches have been proposed, including multi-stage unifocalization performed by lateral thoracotomy, rehabilitation of the central pulmonary artery followed by intracardiac repair, and single-stage unifocalization by a midline incision. Recently, single-stage unifocalization performed during infancy has become the preferred method for achieving low right ventricular pressure after closing the ventricular septal defect. Furthermore, for the maintenance of the lowest possible right ventricular pressure after definitive repair, combination therapy or hybrid therapy with catheter and surgical intervention is essential. The surgical treatment strategy for a single ventricle with major aortopulmonary collateral arteries is more difficult than that involving two ventricles.
治疗伴有室间隔缺损及主要体肺侧支动脉的肺动脉闭锁具有挑战性且存在争议。基本上，通过单源化手术治疗这些侧支动脉，将它们整合起来并统一肺循环中的血流。这些主要侧支动脉在解剖学上与支气管动脉相似；然而，由于不同的环境暴露，它们发育成了不同的血管。目前，正在制定涉及婴儿早期手术干预以处理主要体肺侧支动脉多种变异的治疗方案，以实现确定性的心内修复。从历史上看，已经提出了几种手术方法，包括通过侧胸壁切开术进行的多阶段单源化、中央肺动脉修复后进行的心内修复，以及通过中线切口进行的单阶段单源化。最近，婴儿期进行的单阶段单源化已成为在关闭室间隔缺损后实现低右心室压力的首选方法。此外，为了在确定性修复后维持尽可能低的右心室压力，导管和手术干预的联合治疗或杂交治疗至关重要。伴有主要体肺侧支动脉的单心室的外科治疗策略比涉及两个心室的情况更困难。
