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Fístula artério-venosa pulmonar: uma causa rara de cianose
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Advisor(s)
Abstract(s)
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 embolizção percutânea
sendo um método menos invasivo e possível
de repetição, é actualmente o tratamento
de eleição.
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 insuffi ciency, and a non dilated aortic root. Magnetic ressonance imaging of the pulmonary vessels showed a large aneurismatic pulmonary arteriovenous fi stula (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.
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 insuffi ciency, and a non dilated aortic root. Magnetic ressonance imaging of the pulmonary vessels showed a large aneurismatic pulmonary arteriovenous fi stula (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.
Description
Keywords
Fístula arterio-venosa pulmonar cianose tratamento percutâneo Pulmonary arteriovenous fistula cyanosis
Citation
Nascer e Crescer 2010; 19(4): 260-264