Magnetic Resonance Angiography in Paced Complex Heterotaxy Syndrome with Fontan Conduit Obstruction and Venovenous Collateral Decompression

Abstract

Imaging of complex congenital heart diseases (CHDs) in children is challenging. This article reviews the complementary role of high temporal and high spatial resolution magnetic resonance (MR) angiographic imaging techniques in evaluation of a patient with complex congenital cardiovascular disease and related postsurgical complications. A 4-year-old female patient with complex CHD and multiple previous palliative surgical procedures underwent MR angiography to evaluate the cause of refractory hypoxia. High-resolution MR angiography demonstrated the complex postsurgical cardiovascular anatomy and also assisted in the evaluation of cavopulmonary shunt patency and secondary venovenous shunt formation. Time-resolved MR angiography evaluated pulmonary perfusion and demonstrated a significant pulmonary arteriovenous malformation. This information guided physicians in planning further managements, which resulted in a satisfactory clinical outcome.

Society for Cardiovascular Magnetic Resonance/European Society of Cardiovascular Imaging/American Society of Echocardiography/Society for Pediatric Radiology/North American Society for Cardiovascular Imaging Guidelines for the Use of Cardiac Magnetic Resonance in Pediatric Congenital and Acquired Heart Disease: Endorsed by The American Heart Association

“…255,256,[258][259][260] An increased flow volume at the diaphragm indicates significant collateral flow into the aorta, bypassing the narrowing (see Coarctation of the aorta section). 258 Venovenous collaterals are also imaged by CMR [586][587][588] and their presence needs to be taken under consideration prior to intervention.…”