Multiple Small Pulmonary Arteriovenous Fistulas

Abstract

The condition of multiple small pulmonary arteriovenous fistulas is discussed with regard to diagnosis, complications, and possible etiology. The lesions are believed to be either congenital in origin or secondary to cirrhosis of the liver with portal hypertension. Surgical therapy has not been attempted in this condition because of the diffuse nature of the lesions. A case is discussed wherein the diagnosis of diffuse small pulmonary arteriovenous fistulas was made by cardiac catheterization. The catheter was passed through an atrial septal defect into a pulmonary vein. Difference in oxygen saturation between the pulmonary vein and pulmonary capillaries (pulmonary vein wedge) revealed conclusive evidence of a right-to-left shunt in this area. Etiologic and therapeutic considerations are discussed in view of recent literature on this subject.

Anesthetic management of a pediatric patient with pulmonary arteriovenous fistula undergoing liver transplantation – a case report

“…Patients with HPS have abnormally dilated alveolar capillaries which can cause an intrapulmonary right‐to‐left shunt. In severe cases, disease progression can include the development of a pulmonary AVF . When venous blood from the pulmonary artery is insufficiently oxygenated, patients with HPS or a pathological pulmonary AVF suffer from hypoxemia.…”

“…Nevertheless, these patients must be still at high risk for experiencing complications. Moreover, end‐stage liver disease with HPS typically progresses to development of a pulmonary AVF . A decrease in PVR can adversely affect hypoxemia in patients with HPS without AVF, although increased PVR can exaggerate hypoxemia in patients with AVF.…”

Perfusionsstörungen

“…Solitäre und multiple arteriovenöse Lungenfistein kommen etwa gleich häufig vor (FIEDLER et al 1973). Solitäre Lungenfistein treten etwas öfter in den Unterlappen auf (BoSHER et al 1959); die multiplen Formen können über alle Lungenbezirke verteilt sein (SANDERS et al 1962).…”

Cirrhosis of the Liver – an Unusual Cause of Severe Hypoxemia

“…21, 26]. These anastomoses have also been visualized in vivo in patients with liver disease employing pulmonary angiography [16,26]. In those studies which employed 100% 0 2 breathing to determine absolute right to left shunting through the lungs the persistent unsatura tion of hemoglobin supported pulmonary A-V anastomoses as the prime factor producing the hypoxemia.…”

“…The association of digital clubbing and cirrhosis of the liver has been known since 1884 [11], Arterial unsaturation and cirrhosis in the ab sence of pulmonary disease was first noted in 1935 [30], Abnormal be havior of hemoglobin [17], porto-pulmonary shunts [7,10,28,35], pul monary arterio-venous shunts [6,12,21,25,26], fibrosing alveolitis [14,34] and ventilation perfusion imbalance [19] have all been pro posed as possible mechanisms of the arterial unsaturation. The weight of evidence available at present supports the hypothesis of pulmonary arte rio-venous shunts as the major factor in the arterial unsaturation and hypoxemia associated with cirrhosis of the liver.…”