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The presented case is a 13-year-old boy with Rendu–Osler–Weber syndrome. He first presented with asymptomatic peripheral cyanosis at the age of 5 years 10 months, with echocardiography revealing no intracardiac anomaly. Screening computed tomography showed bilateral multiple arteriovenous malformations. The first coil embolisation of the largest malformation in the inferior portion of the left upper lung was performed at 7 years 2 months, with improvement in O2 saturation from 81% to 93% achieved. The second coil embolisation for the residual shunts was performed 2 months later. After 4 years of uneventful life, he suffered four episodes of syncope and haemoptysis from 12 to 13 years of age. Further embolisation using more and more coils was performed during each episode until stable clinical condition was finally achieved. Rendu–Osler–Weber syndrome is an autosomal dominant hereditary disease. It is frequently associated with pulmonary arteriovenous malformation. Due to the high incidence of neurological complications in untreated patients, therapeutic embolisation is beneficial.
This article has been adapted from Chen S-J, Wang J-K. Coil embolisation for diffuse pulmonary arteriovenous malformations Heart 2008;94:596
Competing interests: None.
▸ Supplementary animation of 3D CT pulmonary angiograms rotated at 360° along the long axis of the body is published online only at http://heart.bmj.com/content/vol94/issue5
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