P27.05: Prenatal diagnosis of total anomalous pulmonary venous connections (TAPVC): multicentre cohort study and meta‐analysis

Abstract

To describe the sonographic features, associations and outcome of TAPVC in order to facilitate its prenatal diagnosis. Retrospective cohort study and meta-analysis. Assessed variables include: sonographic features including TAPVC subtype, GA at diagnosis, area behind LA, ventricular disproportion, vertical vein, pulmonary venous obstruction, mode of diagnosis, association with cardiac and extra-cardiac conditions, pregnancy and fetoneonatal outcome. Cases associated with right isomerism were excluded from the analysis. A systematic review and meta-analysis were performed, the latter developed in line with PRISMA recommendations. Statistical analysis included both fixed and random effect models. A total of 15 studies and 71 patients (including 13 of the cohort study) were included in the systematic review and meta-analysis. The pooled estimate of the association between TAPVC and CHD was 28.3% (95% CI: 18.1%-41.3%), between TAPVC and extra-cardiac anomalies 18.5% (95% CI: 10.5%-30.6%). An obstructed venous return was observed in 34.1% of the cases (95% CI: 22.7%-47.7%). A favourable outcome was recorded in 43.8% (95% CI: 24.0%-65.8%). Ventricular Disproportion was present in 59.2% (95% CI: 45.1%-72.0%) of the cases, an increased area behind the LA in 58.1% (95% CI: 41.1%-73.5%), a vertical vein in 59.3% (95% CI: 41.1%-75.3%). The diagnosis was established in most cases with colour/power Doppler [(84.9% (95% CI: 67.3%-93.9%)]. 1. Even if we exclude cases associated with right isomerism, TAPVC can be associated with other cardiac and extracardiac anomalies in a significant percentage of cases (28.3% and 18.5%). 2. Leading sonographic signs are: ventricular disproportion, an increased area behind LA and the recognition of a vertical vein (59.2%, 58.1% and 59.3%, respectively). Colour/Power Doppler is the key diagnostic mode for diagnosis. An obstructed venous return can be expected in roughly 1/3 of the cases. Outcome is favourable in < 50% of the cases.