Journal of Cardiology and Heart Failure

ABSTRACT

Background

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital cardiac anomaly. Adult survival is uncommon due to progressive systemic right ventricular dysfunction, conduction abnormalities, and associated structural defects. Among these, aortic stenosis is an exceedingly rare association and is the focus of this case report.

Case Presentation

We report a 45-year-old woman with previously undiagnosed CCTGA who presented with syncope due to complete heart block. Evaluation revealed severe valvular aortic stenosis and a moderate ventricular septal defect with elevated pulmonary vascular resistance. Given the high surgical risk associated with systemic right ventricular dysfunction, a tailored transcatheter approach was planned.

Outcome

The patient underwent dual-chamber permanent pacemaker implantation followed by balloon aortic valvuloplasty. The procedure was successfully completed without complications, resulting in symptomatic improvement and stabilization. Surgical intervention was deferred due to prohibitive risk.

Conclusion

This case highlights the rare coexistence of severe aortic stenosis and complete heart block in adult CCTGA and emphasizes the importance of individualized, multidisciplinary decision-making. In high-risk patients, a combined transcatheter strategy may offer a safe and effective palliative alternative to surgery.

Keywords: Adult Congenital Heart Disease; CCTGA; Aortic Stenosis; Dextrocardia; Heart Block