Nineteen-year experience with total anomalous pulmonary venous return at a tertiary care hospital in a low- and middle-income country: a single-centre retrospective study

Total Anomalous Pulmonary Venous Return (TAPVR) is a rare congenital defect requiring surgical correction. Outcomes are influenced by associated anomalies and preoperative pulmonary venous obstruction, which increase morbidity, mortality, and postoperative pulmonary hypertension risk. This study reviews our institutional experience with pediatric TAPVR repair, emphasizing postoperative outcomes and prognostic determinants. This single-centre retrospective cohort (2006–2024) at Aga Khan University Hospital included pediatric TAPVR patients (< 18 years). Preoperative, intraoperative, and postoperative data were analyzed. Continuous variables summarized as mean/SD or median/IQR and categorical as frequencies, with appropriate statistical tests applied. Our cohort included 80 patients (65% male, mean weight 7.6 ± 6.3 kg). Subtypes were supracardiac 43.8%, cardiac 35%, infracardiac 6.3%, and mixed 15%. Mean age at presentation was 20.1 ± 38.5 months, with 55.6 ± 80.5 days to surgery. ASD (86.3%) was most common. Median CPB and cross-clamp times were 90 and 45 min, respectively. Open-chest (23.8%) and extracorporeal support (70%) showed no subtype significance. Median hospital stay was 8 (9) days with CICU stay as 4 (6). Postoperative outcomes included PAH (22.5%), sepsis (18.8%), PVO (6.3%), tracheostomy (7.5%), reoperation (5%) and mortality reported in 17.5% (higher in cardiac TAPVR, p = 0.044). Univariate analysis linked younger age, low weight, TAPVD subtype, postop PVO, and reduced RV function to outcomes; multivariate confirmed only reduced RV function. While preop PVO was significantly associated with postoperative PAH. TAPVR repair yielded favourable outcomes; however vigilant monitoring of RV function and preoperative PVO is essential to improve survival and reduce complications.