Congenital Pulmonary Vascular Anomalies and Disease

Alexander Phan MD, Larry A. Latson Jr MD, MS, Daniel Vargas MD, Joanna G. Escalon MD

Abstract

• Congenital pulmonary vascular anomalies and diseases encompass a wide range of entities involving the pulmonic valve, pulmonary arteries, and/or pulmonary veins, many of which occur in the setting of additional congenital anomalies.
• Given the advancements in treatments, these patients have longer life expectancies, and therefore familiarity with these entities, including pre-operative and post-operative imaging and possible post-repair complications are imperative.
• Some entities are asymptomatic or variable in their presentation/onset and therefore radiologists may be the first to discover.
• Contrast-enhanced cross-sectional imaging, whether with computed tomography angiography or magnetic resonance angiography, should be considered the first-line modality in these adult patients and is generally sufficient to make the diagnosis, problem-solve, and perform follow-up evaluation.