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Partial anomalous pulmonary venous connection (PAPVC) is a relatively uncommon congenital heart defect that is usually associated with either sinus venosus or secundum atrial septal defect (ASD). Patients with an isolated single PAPVC are usually asymptomatic; whereas, co-existence of sinus venosus or a secundum ASD results in right ventricular dilatation and is associated with higher morbidity and mortality. Majority of patients with a secundum ASD and single PAPVC, undergo surgical repair. Outcomes with trans-catheter closure of a secundum atrial septal defect and unrepaired single PAPVC have not been reported.
Between Jan-2005 to Dec-2015, we have treated 7 patients with trans-catheter closure of a secundum ASD in presence of an unrepaired PAPVC at the Toronto Congenital Cardiac Center for Adults.
Four were female, and the mean age was 43.9±17.0 years (26 - 79 years). Three patients had anomalus left upper lobe pulmonary vein, 2 had right upper lobe pulmonary vein and 2 had right upper and mid lobe pulmonary veins in addition to a secundum ASD. Pre-intervention pulmonary to aortic flow ratio (Qp:Qs) was 2.2±1.2 (1.3-4.3) (n=6). All patients were successfully treated with an Amplatzer septal occlude (median size - 22mm, 10-28 mm). At the follow-up of 13±16 months (2-48 months) 6 patients were asymptomatic, whereas one patient reported persistence of exertional dyspnea, however better than pre-intervention. None of the patients required repeat intervention. Post-intervention there was a significant reduction in right ventricular size, measured as basal-diameter in the apical 4-chamber view (Pre-intervention: 5.4±1.0 cm (4.4-6.8 cm), post-intervention: 4.6±1.0 cm (3.6-6.5 cm)), along with reduction in tricuspid regurgitation severity grade (2.4±1.4 vs. 2.0±1.2).
Trans-catheter closure of a secundum atrial septal defect in presence of an anomalous pulmonary venous return is a feasible and safe treatment option with good intermediate term results. These patients should be followed regularly to assess subsequent right ventricular remodeling and long-term outcomes.