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RCH>Division of Surgery>Cardiology>Pulmonary Atresia with VSD and MAPCAs

Pulmonary Atresia with VSD and MAPCAs

  • This defect is a form of Tetralogy of Fallot in which there is complete obstruction of the Pulmonary Artery resulting in total diversion of blood from the right ventricle into the aorta.¬† Survival depends on the presence of a number of naturally occurring connecting blood vessels between the Aorta and the Pulmonary Arteries in the lungs, which are called 'Collaterals' - or sometimes¬†'MAPCAs'¬† (Major Aorto-Pulmonary Collateral Arteries).


    The 'Collaterals' are usually several in number and carry blood to different parts of the lung circulation. Surgery is often a matter of connecting up the multiple Collaterals at different operations before performing a Complete Repair in suitable cases. The final 'Repair' usually involves insertion of a graft valve ('Conduit') to replace the absent Pulmonary Valve.


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