Abstract:
Transposition of the great arteries (TGA) and interruption of the aortic arch (IAA) are uncommon congenital heart diseases. The association between TGA and IAA is rare. The aim of this study is to present a case with combined TGA and IAA, who underwent the primary repair and review the literature with similar cases. The one-month-old patient was admitted with tachypnea and cyanosis. Delayed diagnosis was caused due to the absence of prenatal examination. Echocardiography and computed tomography angiography confirmed TGA with anterior-posterior-oriented great arteries, wide patent ductus arteriosus, type B IAA, ventricular septal defect (VSD) and pulmonary arterial hypertension. The patient underwent a single-stage primary surgical repair process leading to VSD closure, reconstruction of the aortic arch and arterial switch operation in October 2019. The patient is doing well at a 3-month follow-up post-surgery. The echocardiogram suggests a normal systolic function of the ventricles and trivial regurgitation for both aortic and pulmonary valves. CONCLUSIONS: The single-stage repair with VSD closure, reconstruction of aortic arch and arterial switch operation might be an applicable approach for most of the patients with combined TGA and IAA. Long term follow-up is required as a high re-intervention rate for recurrent coarctation, supravalvular aortic stenosis, neoaortic valve regurgitation, obstruction of the right heart system and coronary stenosis has been reported.
摘要:
大动脉转位(TGA)和主动脉弓中断(IAA)是罕见的先天性心脏病。TGA和IAA之间的关联很少。本研究的目的是提出一个TGA和IAA组合的病例,他们接受了一期修复,并回顾了类似病例的文献。1个月大的患者因呼吸急促和紫癜入院。由于缺乏产前检查,导致诊断延迟。超声心动图和计算机断层扫描血管造影证实TGA伴有前-后定向大动脉,宽动脉导管未闭,B型IAA,室间隔缺损(VSD)和肺动脉高压。患者接受了导致VSD闭合的单阶段初级手术修复过程,2019年10月主动脉弓和动脉转换手术的重建。患者在术后3个月的随访中表现良好。超声心动图提示心室的收缩功能正常,主动脉瓣和肺动脉瓣的反流轻微。结论:采用VSD闭合的单阶段修复,主动脉弓重建和动脉转换手术可能是大多数TGA和IAA合并患者的适用方法。需要长期随访,因为复发性缩窄的再干预率高,主动脉瓣上狭窄,新主动脉瓣反流,据报道,右心系统阻塞和冠状动脉狭窄。