日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 36(1): 65-71 (2020)
doi:10.9794/jspccs.36.65

症例報告Case Report

乳児早期発症し,剖検で確定診断した肺静脈閉塞症の1例An Infantile Case of Pulmonary Veno-Occlusive Disease Confirmed by Autopsy

1あかね会土谷総合病院小児科Department of Pediatrics, Tsuchiya General Hospital ◇ Hiroshima, Japan

2国立研究開発法人国立循環器病研究センター小児循環器科Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center ◇ Osaka, Japan

受付日:2019年5月15日Received: May 15, 2019
受理日:2019年9月13日Accepted: September 13, 2019
発行日:2020年3月1日Published: March 1, 2020
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肺静脈閉塞症(PVOD)は肺静脈の閉塞性病変から肺高血圧を呈する予後不良の疾患である.症例は動脈管開存を合併した1か月男児.肺高血圧クライシスで入院した.肺体血圧比1.3, 平均肺動脈圧43 mmHg, 肺動脈楔入圧6 mmHg, 肺血管抵抗10.3 unit·m2で,エポプロステノール持続静注を含む多剤併用療法を開始した.動脈管は自然閉鎖したが肺高血圧が持続し,特発性肺動脈性肺高血圧と診断した.当初治療への反応は良好であったが,肺血管拡張薬の増量に伴い肺水腫を繰り返した.1歳0か月から急速に右心不全が進行し1歳2か月で永眠した.剖検肺病理で肺小静脈内膜肥厚による肺静脈閉塞および肺胞毛細管の増生を認め,PVODと判明した.一方高度な肺小動脈病変(Heath–Edwards 4度)も認めた.肺血管拡張薬により繰り返す肺水腫をきたす場合はPVODを鑑別に挙げ,肺移植を含めた治療戦略を考えるべきである.

Pulmonary veno-occlusive disease (PVOD) is a rare condition that results in pulmonary hypertension caused by the occlusion of the peripheral pulmonary veins. A 1-month old infant with patent ductus arteriosus presented in critical condition with pulmonary hypertensive crisis. Cardiac catheterization revealed severe pulmonary hypertension (pulmonary to systemic arterial pressure ratio, 1.3; mean pulmonary arterial pressure, 43 mmHg; pulmonary wedge pressure, 6 mmHg; pulmonary vascular resistance, 10.3 units·m2). The patient was started on combination therapy, including continuous intravenous epoprostenol. Although drugs targeting pulmonary arterial hypertension (PAH) were initially able to improve his hemodynamic status, he gradually developed pulmonary edema. Histopathological finding of lung biopsy specimen showed PAH, so he was diagnosed with idiopathic PAH. However, he experienced recurrent pulmonary edema and progressive right heart failure. One year after admission, the patient died from severe heart failure. Histopathological autopsy findings of the lung showed pulmonary venous changes and intimal thickening of the pulmonary veins, and a diagnosis of PVOD was made. Pulmonary arterial changes included cellular and fibrous thickening of the intima, severe medial hypertrophy, and adventitial thickening with plexiform lesions (Heath–Edwards grade 4). A correct diagnosis in this case could not be made by single lung biopsy. Our experience suggests that we should suspect PVOD when patients receiving PAH therapy develop recurrent pulmonary edema, as the only available method of PVOD treatment is early lung transplantation.

Key words: pulmonary veno-occlusive disease; pulmonary arterial hypertension; infantile; PH crisis

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This page was last modified on 2020-04-23T14:17:10.000+09:00


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