3D reconstruction is important when evaluating MAPCAs for unifocalisation.

To cite: Ranchordás S, Abecasis M, Anjos R. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014208827 DESCRIPTION Pulmonary atresia with ventricular septal defect, hypoplastic pulmonary arteries and major aortopulmonary collateral arteries is a complex and heterogeneous cardiac disease. Currently, there are two different major surgical approaches: one involves major aortopulmonary collateral arteries unifocalisation, connecting them to the native pulmonary arteries circuit, connection of the right ventricle to the ‘neo-pulmonary arteries’ and concomitant/delayed ventricular septal defect closure. The other option is based on simple rehabilitation of the native pulmonary arteries by increasing their blood supply. We present the case of a 3-year-old girl referred to our hospital for heart surgery. An echocardiogram revealed pulmonary atresia with ventricular septal defect, right aortic arch, confluent pulmonary arteries and multiple major aortopulmonary collateral arteries with significant flow. She was submitted to catheterisation, which confirmed the latter. A CT angiography demonstrated single supply to different pulmonary segments originating either from the pulmonary arteries or from major aortopulmonary collateral arteries from the descending thoracic aorta, with three-dimensional (3D) reconstruction providing excellent anatomical information with a view to surgery (figure 1A, B). She was submitted to a modified Blalock-Taussig shunt with a 4 mm Goretex graft between the brachiocephalic artery and the left pulmonary artery, and unifocalisation of major aortopulmonary collateral arteries. A repeat catheterisation was performed some months after surgery, revealing a good surgical result (figure 2). Additionally, major aortopulmonary collateral arteries to lung areas with double supply were embolised. Use of multiple imaging techniques, particularly 3D reconstruction, is extremely important to plan and monitor this type of intervention.