Munaf Misbah, Farooqui Sofia, Kazmi Syeda K, Ul-Haque Ibtehaj
Internal Medicine, Dow University of Health Sciences, Karachi, PAK.
Cureus. 2020 Jun 30;12(6):e8937. doi: 10.7759/cureus.8937.
Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital cardiac anomaly defined by atrio-ventricular and ventriculo-arterial discordance. This malformation makes up less than 1% of congenital heart defects. We report here a case of a 15-year-old female who presented to our hospital with dyspnea as seen in the New York Heart Association (NYHA) Functional Classification class III and hemoptysis. She was clinically found to have marfanoid habitus, and subsequent echocardiographic study disclosed CCTGA-associated with Ebstein's anomaly, ventricular septal defect, left ventricular outflow obstruction, right ventricular outflow obstruction, co-existing dextrocardia, atrial septal defect, patent ductus arteriosus, non-confluent pulmonary arteries, and pulmonary atresia. This case highlights the association between such rare cardiac conditions. To the best of our knowledge, this is the first case of CCTGA at a young age, with the aforementioned abnormalities documented in the literature reported from Pakistan.
先天性矫正型大动脉转位(CCTGA)是一种罕见的先天性心脏异常,其定义为房室和心室动脉不一致。这种畸形在先天性心脏缺陷中占比不到1%。我们在此报告一例15岁女性患者,她因纽约心脏协会(NYHA)心功能分级III级的呼吸困难和咯血前来我院就诊。临床检查发现她有马方综合征体型,随后的超声心动图研究显示CCTGA合并埃布斯坦畸形、室间隔缺损、左心室流出道梗阻、右心室流出道梗阻、并存右位心、房间隔缺损、动脉导管未闭、肺动静脉不连接和肺动脉闭锁。该病例突出了这些罕见心脏疾病之间的关联。据我们所知,这是巴基斯坦文献报道中首例年轻患者患有CCTGA并伴有上述异常情况。