RESUMEN

Complete assessment of the source of pulmonary blood supply and delineation of the anatomy of pulmonary arteries are essential for the management and prognostic evaluation of pulmonary atresia (PA) patients. Invasive cardiac catheterization is considered the gold standard imaging modality to achieve this. We investigated the role of contrast enhanced magnetic resonance angiography (MRA) to evaluate the pulmonary blood supply and the anatomy of the pulmonary arteries and compared this with cardiac catheterization in children with PA. We studied 20 children with PA. Median age was 2.5 years (range 6 months-13 years). All patients were examined with cardiac catheterization and contrast enhanced MRA, and the results of both modalities were compared. There was a complete agreement between both modalities in the detection of the main pulmonary artery morphology and determination of the confluence state of the central pulmonary arteries. There was an 88% agreement for patency of the ductus arteriosus and 66% for patency of the surgically placed shunt. There was a complete agreement between both techniques on determining the presence of collaterals more than 2.5 mm. Twenty-eight collaterals of less than 2.5 mm were detected only by contrast enhanced MRA. There was a strong correlation between both modalities in measuring the pulmonary arteries and collaterals diameter (P<0.001). Contrast enhanced MRA is a safe and accurate non-invasive technique to evaluate the pulmonary artery morphology and the sources of pulmonary blood supply in children with PA.