High life time risk for pulmonary valve replacement in adults with congenital heart defects with obstruction of the right ventricular outflow tract

Background: Valve replacement is a common procedure in patients with congenital heart disease (CHD) particularly in those with right ventricular outflow tract obstruction (RVOTO). In this study, we assessed the burden of first and subsequent pulmonary valve replacement (PVR) in longterm survivors of CHD.

Methods: 175 adults with CHD (ACHD) and RVOTO from the GoEMAH registry (Göttinger ACHD registry) were assigned to 2 groups: primarily acyanotic defects (n=52) and primarily cyanotic defects with postnatal surgical correction (n=123: tetralogy of Fallot (TOF, n=99), pulmonary atresia with ventricular septal defect (PA-VSD, n=21), double outlet right ventricle (DORV, n=3)). Median age was 36 years (IQR: 27-46; range 18 to 62) with TOF patients being the oldest group (median age 42 years (IQR: 30-50)). There were no differences in sex or NYHA classes between both groups.

Results: 154 PVRs were performed in 96 patients (55% of 175 patients) from birth until assessment in 2024. There were significant differences between the groups regarding the time of first PVR and re-PVR. 90% of patients with PA-VSD had their first PVR within the first decade of life, while in TOF patients, first PVR was most commonly between the third and fifth decade (75% of TOF patients with PVR), and was more or less evenly distributed across lifespan in patients with primarily acyanotic defect. Re-PVR occurred at least once in 39 patients (41% of 96 patients with PVR) during their lifespan. The most common approach for PVR was surgical replacement (n=123 (80%) surgical PVR vs. n=31 (20%) interventional PVR) with the use of a homograft in 86% of surgical valve replacements. In 67 cases, a homograft was implanted at the age of ≥14 years and required replacement in 16 cases (24%) at a median time of 9 years (IQR: 4-15) after implantation.

Conclusion: PVR rate was particular high in patients with primarily cyanotic congenital heart defect with RVOTO. Patients who required their first PVR within the first decade of life had an especially high rate of Re-PVR, while in TOF patients, this rate remained stable at less than 10% per decade up to the 5th decade. Homografts were the most common PVR with a median time-to replacement of 9 years in nearly ¼ of cases.