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We report the case of a 14-year-old girl with di use pulmonary arteriovenous malformations (PAVM) associated with mutations of the endoglin (ENG) gene (p. Ala160del, c.479_481 delCTG; ex4). She was diagnosed at 9 years of age and had undergone percutaneous transcatheter embolization (TCE) ve times since the diagnosis. However, the e ects were limited. Initial oxygen saturation a er coil embolization was over 90%, gradually falling to 80% a er every treatment. With di use PAVM, embolization of the stulas or feeding vessels still leaves a chance for enlargement of another feeding vessel. Enlarged feeding vessels increase stula in ow and consequently induce PAVM development and hypoxia. We considered this as the cause of the repeated hypoxemia. Because embolization of the stulas alone did not seem su cient to interrupt the blood supply, we decided to perform coil embolizations and sacri ce normal pulmonary artery trees. Occluding normal pulmonary arteries is known to reduce pulmonary capillary beds and can induce pulmonary arterial hypertension. However, to improve hypoxemia, we considered that TCE with sacri ce of normal pulmonary arteries would be necessary. Repeated TCE brought about a good clinical course without pulmonary artery pressure elevation. TCE with sacri ce of normal pulmonary arteries may o er a promising and e ective choice of treatment in some cases of PAVM. It is necessary to pay close attention to the development of pulmonary arterial hypertension in long-term follow-up.

nihon shoni junkanki gakkai zasshi/nihon shōni junkanki gakkai zasshi

A Case of Diffuse Pulmonary Arteriovenous Malformationss Successfully Treated by Percutaneous Transcatheter Embolization with Sacrifice of Normal Pulmonary Arteries