1 独立行政法人地域医療連携推進機構 九州病院小児科Department of Pediatrics, Japan Community Health Care Organization Kyushu Hospital ◇ Fukuoka, Japan
2 独立行政法人地域医療連携推進機構 九州病院心臓血管外科Department of Cardiovascular Surgery, Japan Community Health Care Organization Kyushu Hospital ◇ Fukuoka, Japan
孤立性片側肺静脈閉塞は非常に稀な先天性心疾患で，反復する喀血や肺炎などから診断に至る症例が多い．自然予後も様々であるため治療介入の是非や時期に苦慮する症例も存在する．今回自験3例を報告し，診断及び治療の留意点を検討する．
症例1は4歳女児で，1歳6か月より喀血と肺炎を繰り返し，造影CTで右肺静脈欠損と，肺血流シンチグラフィーで右肺血流減少を認め右肺静脈閉鎖が疑われた．右肺動脈楔入造影により閉鎖肺静脈腔を同定し，Sutureless法による肺静脈再建を行った．
症例2は4歳女児で，喀血で入院し，肺血流シンチグラフィーで左肺血流減少を認め，左肺動脈楔入造影により左肺静脈閉鎖と診断した．肺静脈近位端と左房との距離が離れていたので肺静脈再建適応外であった．
症例3は36歳女性で，生後4か月より肺炎と喀血を繰り返していた．肺血流シンチグラフィーで右肺動脈血流低下があり肺動脈楔入造影で右肺静脈閉鎖と診断した．年齢から肺静脈再建は断念し，右肺切除は本人も希望しなかった．
幼少期より反復する喀血や肺炎を呈する場合は，非常に稀な疾患であるが本症を鑑別にあげ，肺血流シンチグラフィーで片側肺の著しい血流低下があれば積極的に肺動脈楔入造影で確定診断を進めることが肝要である．幼児期までに診断し，かつ閉鎖肺静脈腔が解剖学的に修復可能であれば肺静脈再建は治療選択肢の一つとなり，右側肺静脈閉鎖においてはSutureless法は術後肺静脈狭窄を予防する有効な手術方法であると考えられる．
Isolated unilateral pulmonary venous atresia (PVA) is an extremely rare anomaly that typically presents with hemoptysis and pneumonia; however, the most appropriate management strategy remains controversial due to the high variability in clinical course between cases. Herein, we present three cases and discuss the diagnostic and therapeutic considerations.
Case 1: A 4-year-old girl presented with a history of recurrent hemoptysis and pneumonia since 18 months of age. Lung perfusion scintigraphy demonstrated decrease in the right pulmonary blood flow. Pulmonary arterial wedge angiography (PAWA) identified a right PVA. The patient underwent pulmonary vein reconstruction using a sutureless technique.
Case 2: A 4-year-old girl was referred due to hemoptysis. Lung perfusion scintigraphy demonstrated a decrease in left pulmonary blood flow, and the diagnosis was confirmed by PAWA. We abandoned the repair in this case due the presence of a long gap between the left pulmonary vein and left atrium.
Case 3: A 36-year-old female presented with a history of recurrent hemoptysis and pneumonia from infancy. PVA was suspected according to the results of lung perfusion scintigraphy, and the diagnosis was confirmed by PAWA. We abandoned the repair in this case as the development of pulmonary arterial obstructive disease was expected.
If lung perfusion scintigraphy demonstrates a remarkable reduction in unilateral pulmonary blood flow, PVA should be suspected and PAWA should be performed to confirm the diagnosis. Pulmonary vein reconstruction represents a treatment option in cases where the diagnosis is made in early childhood and anatomical repair is feasible. For right PVA, the sutureless technique is considered to be effective in preventing postoperative pulmonary venous stenosis.
Key words: unilateral pulmonary venous atresia; sutureless technique; hemoptysis; recurrent pneumonia; pulmonary arterial wedge angiography
© 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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