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.
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