Transcatheter Edge-to-Edge Repair of the Systemic AV Valve in Atrial Switch TGA

Adults with dextro-transposition of the great arteries (d-TGA) repaired with an atrial switch operation frequently develop systemic atrioventricular valve regurgitation (SAVVR), which is strongly associated with progressive systemic right ventricular (RV) failure and adverse outcomes. Surgical reintervention carries substantial risk in this population, while experience with transcatheter edge-to-edge repair (TEER) remains limited, particularly in atrial-switch anatomy requiring transbaffle access. We report a technically novel strategy using right internal jugular delivery and electrosurgery-assisted transbaffle puncture to facilitate systemic AV valve TEER after Mustard repair when transfemoral access was unsuitable. We report a 32-year-old man with atrial-switch d-TGA, prior systemic atrioventricular valve annuloplasty, transvenous implantable cardioverter-defibrillator leads, and severe SAVVR causing advanced heart failure. At baseline, he was in NYHA class III, with 6-min walk distance of 290 m and NT-proBNP of 7100 pg/mL. Preprocedural imaging demonstrated hostile iliofemoral venous anatomy, precluding transfemoral access. A multimodality imaging-guided strategy using right internal jugular venous access and electrosurgery-assisted transbaffle traversal was employed to reach the pulmonary venous atrium. TEER of the systemic AV valve was successfully performed with implantation of two PASCAL ACE devices, resulting in SAVVR reduction from severe to mild without procedural complications or need for inotropic or intravenous vasodilator support. At 6-month follow-up, the patient remained in NYHA class II, 6-min walk distance improved to 365 m, NT-proBNP decreased to 1670 pg/mL, and SAVVR reduction remained sustained. This case demonstrates that SAVV TEER in atrial-switch d-TGA can be performed via a jugular approach using electrosurgery-assisted transbaffle access when transfemoral routes are unsuitable. An anatomy-driven, Heart Team-based approach integrating advanced imaging may expand transcatheter treatment options for highly selected adults with complex congenital heart disease and limited surgical alternatives.