Abstract

GOMES, Sónia Melo et al. Pulmonary arteriovenous fistula: a rare cause of cyanosis. Nascer e Crescer [online]. 2010, vol.19, n.4, pp.260-264. ISSN 0872-0754.

A 11-year-old boy was referred for recurrent episodes of effort dyspnoea and thoracic pain associated with a marfanoid habitus. Physical examination showed an exuberant pectus excavatum, superficial venous collateral circulation, permanent ungueal cyanosis, digital clubbing and hypoxaemia. Cardiac evaluation revealed mitral valve prolapse without insufficiency, and a non dilated aortic root. Magnetic ressonance imaging of the pulmonary vessels showed a large aneurismatic pulmonary arteriovenous fistula (PAVF) on the right. The patient was treated percutaneously through embolization; other AVFs were found bilaterally and 3 of the largest were also embolized. Transcutaneous oxygen saturation improved from 80 to 95% after catheterization. This unusual presentation with multiple bilateral pulmonary AFVs confers this case an unfavourable medium term prognosis. Embolotherapy, being less invasive and easy to repeat, is nowadays the treatment of choice.

Keywords : pulmonary arteriovenous fistula; cyanosis.

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