A case of multiple small pulmonary arteriovenous fistulas with chronic hepatitis and abdominal vessel abnormalities

Abstract

A 63-year-old female was admitted with dyspnea on exertion. She had previously suffered from a large amount of epistaxis. Chest X-ray showed the bilateral diffuse granular infiltrates. Arterial blood analysis revealed severe hypoxemia and pulmonary function test showed reduced function of diffusing capacity. Dynamic radionuclide perfusion scintigraphy performed with 99mTc-labeled MAA demonstrated passage of microspheres through the pulmonary vasculature into the systemic circulation, indicating a right-to-left shunt. Although selective pulmonary wedge angiography did not directly demonstrate pulmonary arteriovenous fistulae, the histopathological findings of TBLB specimens revealed multiple dilated alveolar vessels that microspheres could pass through. In addition, chronic hepatitis, dilatation of splenic vein and spleno-renal anastomosis had been present. This type of pulmonary arteriovenous fistula is said to be a rare dispersed telangiectasic type, associated with a part of Rendu-Osler-Weber syndrome and a cause of hypoxemia in liver cirrhosis. We consider that in this case, the chronic hepatitis may have played an important role of developing multiple small pulmonary arteriovenous fistulas.