Congenitally Corrected Transposition of Great Arteries with Dextrocardia, Patent Ductus Arteriosus, Atrial Septal Defects and Ventricular Septal Defects in a 15-Year-Old Marfanoid Habitus Patient: A Case Study

Abstract

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.

Percutaneous closure of an atrial septal defect in adult patients with congenitally corrected transposition of the great arteries

“…Another likely anomaly is secundum-type atrial septal defect that can be isolated, but it is rare. 2,8,9 Few studies have mentioned other rare associations like Criss cross heart 10 and aortic coarctation. 11…”

“…Atrial septal defect is rare in congenitally corrected transposition of the great arteries, 2 and information around it is scarce. Repairment of a large atrial septal defect, isolated or simultaneously with another defect, may be necessary.…”

“…Association of corrected transposition of the great arteries and isolated atrial septal defect is a very rare condition. 2 In this condition, a left-to-right shunt through the inter-atrial septal defect is present, so it is expected that in the long run, it will increase the right heart volume overload and enlargement, and of course, in these special patients, subpulmonic ventricle (morphological left ventricle) enlarges and right-sided atrioventricular valve (mitral valve) regurgitation would be evident. In these cases, as the patient grows up, left to right shunt through atrial septal defect increases, and patient becomes symptomatic.…”