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 38(1): 48-53 (2022)
doi:10.9794/jspccs.38.48

症例報告Case Report

異なる生命予後を示したscimitar症候群の同胞例とリスク因子の検討Familial Scimitar Syndrome with Different Outcomes and Risk Factors

1福岡市立こども病院小児循環器科Department of Pediatric Cardiology, Fukuoka Children’s Hospital ◇ Fukuoka, Japan

2榊原記念病院小児循環器科Department of Pediatric Cardiology, Sakakibara Heart Institute ◇ Tokyo, Japan

3金沢医科大学病院心臓血管外科Department of cardiovascular Surgery, Kanazawa Medical University Hospital ◇ Ishikawa, Japan

4東邦大学医療センター大森病院新生児科Department of Neonatology, Toho University Omori Medical Center ◇ Tokyo, Japan

受付日:2021年6月19日Received: June 19, 2021
受理日:2021年12月6日Accepted: December 6, 2021
発行日:2022年2月1日Published: February 1, 2022
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Scimitar症候群とは,先天性心疾患,肺形成異常,主に右肺からの下大静脈への肺静脈還流異常が見られる稀な症候群である.その重症度の幅は大きく,表現型に多様性を認める.今回,異なる生命予後を示したscimitar症候群の同胞例を経験したので,ほかのscimitar症候群を合わせた全8症例の予後因子について検討した.成人例は無症状に対し,右肺低形成,肺動脈径の左右差,先天性心疾患の合併は小児例に有意に認め生後1か月以内に全例,発症した.小児例の6例中4例に手術を施行し,1例は手術適応外だった.3例が死亡し,そのうち2例に複雑心疾患を合併し,死亡した3例すべてに気道病変を認めた.肺高血圧を確認したのは2例で,すべて小児例で気道病変合併例のみ死亡した.また死亡例に側副血管の灌流領域の多い傾向も認めた.予後因子について発症時期,複雑心疾患,肺高血圧,側副血管の灌流領域によるうっ血性心不全に加え気道病変の有無が考えられた.

Scimitar syndrome is an uncommon congenital heart and lung abnormality, characterized by an aberrant pulmonary venous return to the inferior vena cava, often from the right lung. The clinical manifestation varies greatly in terms of severity and phenotype. We recently observed familial scimitar syndrome, which had both positive and negative outcomes. Besides six additional cases of scimitar syndrome, we investigated risk factors for death. Because of hypoplasia of the right pulmonary artery or lung itself, all infantile cases had heart symptoms before the age of 1 month and concomitant congenital heart disorder was more common. Intracardiac repair or Norwood operation were performed in four of six infants, whereas Norwood operation was deemed contraindicated in one of the remaining two infants. Three of these operative cases died, of whom complex congenital heart diseases were seen in two cases. Airway malformations were seen in all of these fatal cases. Two of six infantile cases had the pulmonary vascular obstructive disease, and one died from an airway deformity. Furthermore, in situations of mortality, the region of the lung supplied by aortopulmonary collateral blood vessels are larger. Our study revealed that infantile-onset of the symptoms of heart failure, the presence of complex congenital heart disease, pulmonary vascular obstructive disease, the presence of congestive heart failure due to aortopulmonary collateral blood vessels, and airway malformations were risk factors for mortality in patients with scimitar syndrome.

Key words: infantile congenital heart disease infantile; pulmonary airway malformation; pulmonary hypertension; scimitar syndrome

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This page was last modified on 2022-05-25T19:50:00.000+09:00


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