Alomrani Ahmed, Alshammari Mohammed, Bindakhil Fahad, Shabi Hadi, Alkhodair Abdullah
Department of Cardiac Sciences, Ministry of National Guard Health Affairs, PO Box 22490, Riyadh 11426, Saudi Arabia.
College of Medicine, King Saud bin Abdulaziz University for Health Sciences, PO Box 3660, Riyadh 11481, Saudi Arabia.
Eur Heart J Case Rep. 2025 May 7;9(5):ytaf161. doi: 10.1093/ehjcr/ytaf161. eCollection 2025 May.
Congenitally corrected transposition of the great arteries (CCTGA), or L-loop TGA, is a rare congenital heart defect, comprising <1% of congenital heart diseases, with an incidence of ∼1 in 33 000 births. It is characterized by atrioventricular and ventriculo-arterial discordance, where the left ventricle connects to a right atrium and pumps deoxygenated blood into the pulmonary artery, while the right ventricle (RV) connects to a left atrium and pumps oxygenated blood into the aorta. Congenitally corrected transposition of the great arteries often coexists with other cardiac anomalies, although ∼10% of cases are isolated. A case report highlights a unique instance of coronary artery disease (CAD) in CCTGA.
An 83-year-old male with CCTGA presented with persistent nausea, fatigue, poor oral intake, and epigastric pain. He had a history of hypertension, Type II diabetes, dyslipidaemia, ischaemic heart disease, and chronic atrial fibrillation. On examination, he was stable but showed signs of a urinary tract infection. A cardiac workup revealed no ischaemic changes on electrocardiography, but a cardiac computed tomography identified significant CAD involving multiple vessels. A decision was made to perform percutaneous coronary intervention on the right coronary artery, successfully placing two stents.
In patients with CCTGA, major factors contributing to morbidity and mortality include progressive decline in systemic RV function and systemic tricuspid valve regurgitation. A retrospective study showed that 25% of uncomplicated CCTGA patients develop heart failure by age 45, while approximately two-thirds of complicated cases do. Survival beyond age 70 is rare. Prompt management of CAD through angioplasty is critical to prevent further deterioration of RV function and worsening tricuspid regurgitation. However, the atypical positioning of the aorta and coronary arteries complicates selective coronary angiography, making a challenging diagnosis and treatment.
先天性矫正型大动脉转位（CCTGA），即L型环大动脉转位，是一种罕见的先天性心脏缺陷，在先天性心脏病中占比不到1%，发病率约为每33000例出生中有1例。其特征是房室和心室 - 动脉不一致，即左心室连接到右心房并将脱氧血液泵入肺动脉，而右心室（RV）连接到左心房并将含氧血液泵入主动脉。先天性矫正型大动脉转位常与其他心脏异常并存，不过约10%的病例是孤立存在的。一份病例报告突出了CCTGA中冠状动脉疾病（CAD）的一个独特实例。
一名患有CCTGA的83岁男性出现持续恶心、疲劳、食欲不振和上腹部疼痛。他有高血压、II型糖尿病、血脂异常、缺血性心脏病和慢性心房颤动病史。检查时，他情况稳定，但有尿路感染迹象。心脏检查显示心电图无缺血性改变，但心脏计算机断层扫描发现多支血管存在严重CAD。决定对右冠状动脉进行经皮冠状动脉介入治疗，成功置入了两枚支架。
在CCTGA患者中，导致发病和死亡的主要因素包括系统性右心室功能的逐渐下降和系统性三尖瓣反流。一项回顾性研究表明，25%的无并发症CCTGA患者在45岁时会发生心力衰竭，而约三分之二的复杂病例会出现这种情况。70岁以后存活的情况很少见。通过血管成形术及时处理CAD对于防止右心室功能进一步恶化和三尖瓣反流加重至关重要。然而，主动脉和冠状动脉的非典型定位使选择性冠状动脉造影变得复杂，导致诊断和治疗具有挑战性。
