1 熊本市立熊本市民病院小児循環器内科Department of Pediatric Cardiology, Kumamoto City Hospital ◇ Kumamoto, Japan
2 熊本市立熊本市民病院小児心臓外科Pediatric Cardiac Surgery, Kumamoto City Hospital ◇ Kumamoto, Japan
低酸素血症と家族歴より遺伝性出血性毛細血管拡張症（HHT）に合併した多発性の肺動静脈瘻（PAVM）と診断された．流入動脈径3 mm未満であったが，低酸素血症を認める多発性病変のため症状改善を目的とし右左短絡量の多い順にコイル塞栓術を施行した症例を経験した．通常流入動脈径3 mm以上の場合は重篤な中枢神経合併症のリスクが高いことから，積極的治療介入を要す．最近ではデバイスの進歩もあり，3 mm未満の病変に対する治療適応の幅も広がってきていることから症例毎の検討を要す．HHTは常優染色体遺伝する疾患であり，家族歴を有する症例においてはスクリーニング検査を施行することでPAVMの早期診断に至りうる可能性がある．特にHHT type 1は多発性PAVMの合併率が高く，慎重なフォロー，適切な治療介入による症状改善，合併症予防が大切である．
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.
Key words: pulmonary arteriovenous malformation; hereditary hemorrhagic telangiectasia; coil embolization
© 2017 特定非営利活動法人日本小児循環器学会© 2017 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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