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To review the diagnosis, management and outcomes of patients with antenatal and postnatal diagnosis of total anomalous pulmonary venous connexion (TAPVC) associated with unbalanced ventricles who underwent surgical intervention.This was an observational descriptive study involving retrospective review over 13 years from cardiac and fetal cardiac database at a single tertiary cardiac centre.We reviewed 30 cases of TAPVC with associated complex congenital cardiac lesion between 2003 and October 2016. Of these, 19/30 cases were associated with unbalanced ventricles.Amongst the live born antenatal patients who elected to proceed with surgery (8/13), only 2/8 are alive (currently aged 22 months and 7 years). The remaining patients who had undergone surgery (6/8) – had only survived between 6 days to 44 weeks (median days of survival 22 days). The total mortality was 84%, and survival was 30% for those undergoing surgery. We also reviewed the isomeric status and types of TAPVC (tables 2 and 3)With our data, we have found that the surgical outcome of TAPVC with unbalanced ventricles is poor, even with patients who are non-isomeric. The antenatal diagnosis of TAPVC with complex anatomy remains challenging, however should be suspected and evaluated further in the context of complex cardiac diagnosis.