Department of Pediatric Cardiac Surgery, NH SRCC Children's Hospital, Mumbai, Maharashtra, India.
Department of Cardiac Anesthesia, NH SRCC Children's Hospital, Mumbai, Maharashtra, India.
World J Pediatr Congenit Heart Surg. 2024 May;15(3):391-393. doi: 10.1177/21501351231196500. Epub 2023 Sep 28.
Circumflex aorta is described as a retroesophageal aortic arch, with opposite-sided descending aorta forming a true vascular ring with ligamentum arteriosum. We report two cases of right-sided circumflex aorta with varied clinical presentation. Computed tomography diagnosed this vascular ring anomaly. The patients were managed with the aortic uncrossing procedure as a primary surgical strategy. Preoperative diagnosis and thorough planning were essential for the successful outcome. The patients did not have any residual symptoms of tracheal and esophageal compression on follow up.
左冠状动脉异常起源于肺动脉是一种罕见的先天性心脏病,其发病率约占先心病的 0.1%~0.2%,可单独存在,也可合并其他心血管畸形。解剖学上,左冠状动脉通常起源于左冠状窦,左前降支和回旋支分别从左冠状动脉主干发出。如果左冠状动脉起源于肺动脉主干,或起源于肺动脉干与右冠状动脉之间,则会引起左心室和主动脉之间的异常血流,导致心肌缺血和心功能不全。左冠状动脉异常起源于肺动脉的临床表现多样,主要取决于左冠状动脉异常起源的位置和合并的其他心血管畸形。常见的临床表现包括心肌缺血、心力衰竭、心律失常、晕厥、猝死等。心电图、超声心动图、冠状动脉造影、多排螺旋 CT 血管成像等检查有助于明确诊断。治疗方法主要包括手术治疗和介入治疗。手术治疗是根治左冠状动脉异常起源于肺动脉的首选方法,介入治疗主要适用于无法耐受手术或不愿意接受手术的患者。