Reversible pre-capillary pulmonary hypertension due to long-standing arteriovenous fistula

Chronic kidney disease predisposes patients to the development of pulmonary hypertension (PH) via multiple potential mechanisms. AV fistulas have been found to be superior to AV grafts in terms of maintaining patency, however unlike grafts fistulas can expand significantly over time which predisposes patients to high output heart failure and subsequent PH. This report demonstrates a case of newly diagnosed pre-capillary PH in a young patient presenting with dyspnea. The patient had an AV fistula placed 18 years prior to presentation for CKD and ultimately underwent transplantation. Pulmonary angiography was negative for PE and echocardiography demonstrated an elevated RVSP of 67 mmHg that was confirmed via right heart catheterization. This case was unique in that the patient's pre-capillary PH was reversed after AV fistula ligation and the patient also experienced improvement in renal graft function (Cr 1.43 to 1.2 mg/dL). AV fistulas can have a significant effect on cardiac function and clinicians should survey patients with AV fistulas for PH via screening echocardiography. Fistulas with outputs greater than 1.5-2 L/min should be considered for revision or ligation to decrease the risk of development of PH.