Percutaneous treatment with the ‘valve-in-valve’ technique in congenitally corrected transposition of the great arteries and systemic atrioventricular valve with severe double lesion: case report

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease characterized by atrioventricular (AV) and ventriculoarterial discordance, allowing near-normal circulation without early surgical correction. However, long-term complications such as valvular dysfunction, systemic right ventricular (RV) failure, and pulmonary hypertension are common. Management becomes particularly challenging in patients with prior interventions, where anatomical complexity and elevated surgical risk limit therapeutic options.

We present the case of a 47-year-old Chinese female diagnosed with CCTGA who had previously undergone surgical implantation of a bioprosthetic valve in the systemic AV position in her home country. She presented with significant deterioration in functional capacity, and imaging revealed severe dysfunction of the surgical prosthesis, characterized by severe mixed valvular disease, aneurysmal dilation of the left atrium, and severe systemic RV dysfunction. A multidisciplinary team assessment determined that the patient was at high surgical risk, leading to the decision to perform a percutaneous valve-in-valve implantation. An Edwards SAPIEN® 26 mm bioprosthetic valve was successfully implanted, yielding excellent haemodynamic results without complications. The patient was discharged 24 h post-procedure with significant clinical improvement.

Although percutaneous interventions for systemic tricuspid valve disease have been described, they typically involve native valves and remain anecdotal. To date, no published reports have documented valve-in-valve implantation in a systemic AV prosthesis in a patient with CCTGA. This case illustrates the feasibility and safety of such an approach in a high-risk patient, offering a valuable alternative to surgical replacement and potentially delaying the need for heart transplantation.