Imaging in Pulmonary Atresia with Ventricular Septal Defect

Pulmonary atresia with ventricular septal defect (PA-VSD) is well recognized as a separate group of diverse congenital malformations resulting from an absence of luminal continuity between the ventricle and the pulmonary arteries in the presence of a ventricular septal defect (VSD). Compared to classic Tetralogy of Fallot (TOF), PA-VSD often has surgical challenges and relatively poorer outcomes due to variable pulmonary blood flow. Advances in cross-sectional imaging techniques like computed tomography (CT) and magnetic resonance imaging (MRI) have revolutionized the management of patients with PA-VSD by presenting a comprehensive evaluation of anatomic structures, including the heart, pulmonary and systemic thoracic vasculature, lungs, and abdomen. The use of multiplanar and 3D CT reconstruction images helps the clinicians and operating surgeons understand the morphology and its impact on management. Multi-detector CT (MDCT) and Magnetic resonance imaging (MRI) complement echocardiography and increasingly substitute for invasive angiography in the management of PA-VSD.