Sónia Melo Gomes1, Mónica Batista2, Ana Teixeira3, Graça Nogueira3, Ana Almeida4, Isabel Mendes Gaspar4, Rui Anjos3, Fernando Maymone Martins3
1 S. Pediatria H. Caldas da Rainha, CH Oeste Norte
2 S. Pediatria, H. Espírito Santo, Évora
3 S. Cardiologia Pediátrica, H. Santa Cruz, C H Lisboa Ocidental
4 S. Genética Médica, H. S. Maria, CH Lisboa Norte
RESUMO
]]> Apresenta-se o caso de um rapaz de 11 anos, referenciado por episódios recorrentes de dispneia de esforço e dor torácica associados a habitus marfanoide.Apresentava tórax com pectus excavatum exuberante e circulação venosa colateral superficial, cianose ungueal permanente, unhas em vidro de relógio e hipoxemia.
A avaliação cardiológica revelou prolapso mitral e raiz da aorta não dilatada. A ressonância magnética pulmonar evidenciou uma fístula arteriovenosa (FAV) pulmonar aneurismática à direita, de grandes dimensões.
O doente foi tratado por via percutânea através de embolização, tendo sido identificadas outras FAV bilateralmente e embolizadas três com maiores dimensões. As saturações transcutâneas de oxigénio melhoraram de 80 para 95%.
A apresentação pouco habitual com múltiplas FAV pulmonares bilaterais, confere a este caso um mau prognóstico a médio prazo. A embolização percutânea sendo um método menos invasivo e possível de repetição, é actualmente o tratamento de eleição.
Palavras-chave: fístula arterio-venosa pulmonar, cianose, tratamento percutâneo.
Pulmonary arteriovenous fistula: a rare cause of cyanosis
ABSTRACT
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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Sónia Melo Gomes,
Rua Dr Bastos Gonçalves, 3-3B,
1600-898 Lisboa
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