Transcatheter Interventions for Atrioventricular Dysfunction in Patients with Adult Congenital Heart Disease: An International Case Series

Introduction: A substantial proportion of patients with adult congenital heart disease (ACHD) suffer from worsening valvular dysfunction in adulthood. Transcatheter valve interventions can offer a therapeutic alternative to surgery for those at high surgical risk. There is emerging but limited data on transcatheter interventions for atrioventricular (AV) valve dysfunction in patients with ACHD. Methods: We compiled an international collaborative multi-center registry focusing on adult patients with congenital heart disease undergoing transcatheter AV valve interventions (repair or replacement). Included were patients from three international centers who underwent procedures between 2016 and 2022. Demographic, clinical, and procedural data were compiled. Results: Nine patients with ACHD underwent AV valve interventions. The median age was 48 years (IQR (37; 56), 55% women). At baseline, seven patients (78%) were in NYHA functional class III and two (22%) were in NYHA functional class II. The diagnosis of ACHD varied. Three valve interventions were performed on the subpulmonary AV valve and six on the systemic AV valve. The primary valvular pathology was regurgitation (six patients, 78%). Five procedures were valve-in-valve interventions, and four procedures were transcatheter edge-to-edge repair procedures. There were no major complications or peri-procedural complications or peri-procedural mortality. One patient developed a suspected non-obstructive thrombus on the valve that was medically treated. One patient did not improve clinically following the procedure and underwent a heart transplant, one patient died 6 months following the procedure due to a cardiovascular implantable electronic device infection. At one year, six patients were in NYHA functional class I, and one patient was in NYHA functional class III. In conclusion, transcatheter AV heart valve interventions are feasible and safe procedures in carefully selected ACHD patients. These procedures can offer an effective treatment option in these younger patients with high surgical risk.