Multimodality Imaging Diagnostic Approach of Systemic-to-Pulmonary Vein Fistulae

A 26-year-old man with a history of bilateral lung transplantation for pulmonary cystic fibrosis 6 months before was admitted in our institution for acute heart failure. Cardiac magnetic resonance imaging (CMR) showed an increased aortic output, as aortic flow assessed by velocity mapping was twofold the pulmonary flow, an occluded superior vena cava (SVC), and enlarged azygos vein. A systemic-to-pulmonary vein fistula (SAPVF) was suspected. The selective angiography showed numerous fistulae between intercostals, thyro-cervical, internal mammary arteries and pulmonary veins. The thoracic CT performed before the CMR, which was initially considered as normal, showed well these arteriovenous fistulae after 3D MIP reconstruction. This particular observation highlights the great value of multimodality imaging for the diagnosis of this rare pathology. The MR velocity mapping is a noninvasive imaging technique of great interest to guide the diagnosis of arteriovenous fistulae, and further indicating more invasive complementary imaging modalities like selective arterial angiography.