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Congenital pulmonary arteriovenous malformation: A rare cause of cyanosis in childhood

Authors:

H Mottaghi,

Division of Cardiology, Department of Pediatrics, Imam Reza Hospital, IR
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M Kahrom ,

Department of Cardiac Surgery, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, IR
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M Hassan Nezafati,

Department of Cardiac Surgery, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, IR
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H Kahrom

Department of Cardiac Surgery, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, IR
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Abstract

Pulmonary arteriovenous malformations (PAVMs) are caused by abnormal communications between pulmonary arteries and pulmonary veins, which are most commonly congenital in nature. Although these lesions are uncommon, they are an important part of the differential diagnosis of common pulmonary problems such as hypoxaemia, pulmonary nodules and cyanosis.

PAVM is a rare disorder with an incidence of 2-3 per 1,000,000 population. It occurs twice as often in women as in men, but there is a male predominance in newborns. Around 10% cases of PAVM are identified in infancy or childhood, followed by a gradual increase in the incidence through the fifth and sixth decades. Approximately 70% of the cases of PAVM are associated with hereditary haemorrhagic telangiectasia (HHT). Conversely, about 15 to 35% of patients with HHT have PAVM. PAVMs may cause hypoxaemia and dyspnoea due to right to left shunting, but frequently remain undiagnosed. This intrapulmonary malformation is described in two patients who presented with severe cyanosis.

Index words: pulmonary arteriovenous malformation; cyanosis; right to left shunting

DOI: 10.4038/cmj.v55i1.1707

Ceylon Medical Journal Vol.55, No.1 March 2010 pp.23-25

DOI: http://doi.org/10.4038/cmj.v55i1.1707
How to Cite: Mottaghi, H. et al., (2010). Congenital pulmonary arteriovenous malformation: A rare cause of cyanosis in childhood. Ceylon Medical Journal. 55(1), pp.23–25. DOI: http://doi.org/10.4038/cmj.v55i1.1707
Published on 30 Mar 2010.
Peer Reviewed

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