Diffuse pulmonary arteriovenous fistulas: report of 2 cases and literature review

Abstract

Objective To describe the clinical characteristics of diffuse pulmonary arteriovenous fistulas (PAVFs) and to evaluate the methods of diagnosis and treatment.Methods Retrospective analysis of two patients with diffuse PAVFs who were diagnosed in Peking University First Hospital and the literatures on the subject were reviewed.Results Both patients presented with the triad of dyspnea on exertion,cyanosis,and clubbing.Case 1 was a woman with hereditary telangiectasia disease (HHT),who also presented with repeated epistaxis and facial telangiectasia.Case 2 was a man with hepatic cirrhosis,who presented with hepatopulmonary syndrome.Echocardiographies of 2 patients were both normal,peripheral vein contrast echocardiography showed abnormal filling of the left atrium with echo contrast material.Chest CT showed bilateral intrapulmonary artery dilatation,subpleural small nodular,reticular and patchy opacities.Pulmonary angiography showed that pulmonary circulation time was both shortened.Pulmonary angiography also showed that lower left dorsal segment artery and pulmonary vein were directly connected in case 2,and then tanscatheter embolization was carried out.However,oxygenation of this patientwasn't improved.Case 1 was alive and case 2 died of liver cirrhosis after 5-year follow up,neither of them had brain complications.Conclusions Diffuse PAVFs are clinically rare and present with the triad of dyspnea on exertion,cyanosis,and clubbing.Pulmonary angiography and contrast echocardiography are helpful for diagnosis. Key words: Diffuse pulmonary arteriovenous fistula; Hereditary haemorrhagic telangiectasia; Hepatopulmonary syndrome; Contrast echocardiography