Krokovay Anna, Knirsch Walter, Kelly-Geyer Janet, Cesnjevar Robert, Dave Hitendu
Division of Congenital Cardiovascular Surgery, University Children's Hospital Zurich, Switzerland.
Children's Research Centre, University of Zurich, Switzerland.
Ann Pediatr Cardiol. 2022 Jul-Aug;15(4):425-428. doi: 10.4103/apc.apc_232_21. Epub 2023 Jan 6.
Right ventricle-dependent coronary circulation coexisting with left main coronary atresia in the setting of pulmonary atresia-intact ventricular septum is rare. In the case described, the left coronary artery (LCA) origin from the aorta could not be found on conventional angiography or cardiac magnetic resonance imaging. During surgery, multiple LCA branches originating from the finger-like continuum of the primitive right ventricular sinusoidal network were observed. A Damus-Kaye-Stansel anastomosis and an aortopulmonary shunt operation were performed. Shunt takedown and a bidirectional Glenn anastomosis followed at 3 months of age. At 18 months follow-up, the child is thriving with stable hemodynamics and a saturation of 85%. Awareness about this rare coronary artery anomaly is necessary to prevent catastrophic consequences. The challenges, complications, and lessons learned while treating this rare variant are discussed.
在肺动脉闭锁-室间隔完整的情况下，右心室依赖性冠状动脉循环与左冠状动脉主干闭锁并存的情况较为罕见。在所描述的病例中，在传统血管造影或心脏磁共振成像上均未发现发自主动脉的左冠状动脉（LCA）。手术过程中，观察到多个LCA分支起源于原始右心室窦状网络的指状连续结构。进行了Damus-Kaye-Stansel吻合术和体肺分流手术。3个月大时进行了分流拆除和双向Glenn吻合术。在18个月的随访中，患儿生长良好，血流动力学稳定，血氧饱和度为85%。认识到这种罕见的冠状动脉异常对于预防灾难性后果很有必要。本文讨论了治疗这种罕见变异时面临的挑战、并发症及经验教训。
