Pediatric Cardiology and Cardiac Surgery
Online ISSN : 2187-2988
Print ISSN : 0911-1794
ISSN-L : 0911-1794
Case Report
Coil Embolization for Multiple Pulmonary Arteriovenous Malformations in a Patient with Hereditary Hemorrhagic Telangiectasia
Osamu MatsuoKei HondaKoichi YatsunamiKoji Fukae
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JOURNAL OPEN ACCESS

2017 Volume 33 Issue 3 Pages 241-246

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Abstract

We report a case of a 13-year-old girl with multiple pulmonary arteriovenous malformations (PAVM) associated with hereditary hemorrhagic telangiectasia (HHT) type 1. The feeding artery diameter of all PAVMs was less than 3 mm, but the patient presented with hypoxemia. Therefore, we performed coil embolization for the large lesion until oxygenation was improved. A feeding artery of PAVM that exceeds 3 mm in diameter should be treated because it has an increased risk of serious cerebral complications. With progress of tools such as detachable microcoils and vascular plugs, we need to examine adaptation of treatment of PAVM with feeders smaller than 3 mm in every case. HHT is an autosomal-dominant disease caused by mutations in five to six genes. HHT may lead to early diagnosis of PAVM in cases with a family history by conducting a screening test. In particular, HHT type 1 has a high incidence of the complication of multiple PAVMs. Careful follow-up and prevention of complications by appropriate treatment intervention are important.

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© 2017 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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