Abstract
BACKGROUND: Arteriovenous (AV) fistulae is an extremely rare disease of vascular malformation that involves fistulae formation between the systemic and pulmonary AV systems. CASE REPRESENTATION: This case report describes a rare systemic-pulmonary AV fistulae of congenital origin, accompanied by pulmonary hypertension, as determined by aortic angiography and echocardiography. CONCLUSION: Characteristics, diagnosis, and therapeutic approaches of this rare abnormality are explored.