Percutaneous treatment with the ‘valve-in-valve’ technique in congenitally corrected transposition of the great arteries and systemic AV valve with severe double lesión. Case report

Background Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease characterized by atrioventricular 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. Case summary 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 atrioventricular (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 right ventricular 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 26mm® bioprosthetic valve was successfully implanted, yielding excellent hemodynamic results without complications. The patient was discharged 24 hours post-procedure with significant clinical improvement. Discussion 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.