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 31(3): 126-132 (2015)
doi:10.9794/jspccs.31.126

原著Original

肺生検組織所見,臨床経過から考える18トリソミーの管理Management of Trisomy 18 Based on the Clinicopathology of Pulmonary Arteriopathy and Clinical Course

1土谷総合病院小児科Department of Pediatrics, Tsuchiya General Hospital ◇ 〒730-8655 広島市中区中島町3番30号3-30 Nakajima-cho, Naka-ku, Hiroshima-shi, Hiroshima 730-8655, Japan

2土谷総合病院心臓血管外科Department of Cardiovascular Surgery, Tsuchiya General Hospital ◇ 〒730-8655 広島市中区中島町3番30号3-30 Nakajima-cho, Naka-ku, Hiroshima-shi, Hiroshima 730-8655, Japan

受付日:2014年10月23日Received: October 23, 2014
受理日:2015年4月23日Accepted: April 23, 2015
発行日:2015年5月1日Published: May 1, 2015
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背景:18トリソミーの主な死因には心疾患に伴う肺出血や心不全,無呼吸などがある.

方法:高肺血流性心疾患に対して心臓手術を行った18トリソミー19人を対象に,予後を再評価し管理の参考にする目的で肺生検組織所見,経過について後方視的に検討した.

結果:日齢40.8±13.0日に肺動脈絞扼術(PAB)と肺生検を施行.肺小動脈形成不全(MD)4例(21.1%)・低形成(HPA)7例(36.8%),肺胞低形成(AH)12例(63.2%)は過去の18トリソミー以外での報告より多かった.痙攣11例.抜管した17例中閉塞性無呼吸14例.死亡6例(突然死1例,痙攣重積2例,心臓関連死(CRD)3例).PAB施行日齢とCRD, AHと術後挿管期間との間に相関を認めた.

結論:18トリソミーではMDやHPA, AHが多く,心臓関連以外にも生命予後に関わるリスクが多い.心臓手術が長期予後改善に結びつくかは不明だが,積極的治療希望の場合は,長期の人工呼吸管理を避け,生後早期に高肺血流に対するPABを行うべきである.

Background: The major causes of death in patients with trisomy 18 are heart failure, pulmonary hemorrhage because of congenital heart disease (CHD), and sudden death because of apnea.

Methods: Pulmonary artery banding (PAB) and open lung biopsy for pulmonary arterial hypertension were performed in 19 patients with trisomy 18 and CHD. The pathological findings in the small pulmonary arteries and the patients’ clinical course were analyzed.

Results: The mean age of the patients at the time of open lung biopsy was 40.8±13.0 days. Four patients (21.1%) had medial defects of the small pulmonary arteries and seven patients (36.8%) had hypoplasia of the small pulmonary arteries. Twelve patients (63.2%) had alveolar hypoplasia. The prevalence of these defects was higher in patients with trisomy 18 than in previously reported patients without trisomy 18. Eleven patients (57.8%) had associated convulsions. Overall, 14 (82.4%) of 17 patients who had been weaned from ventilatory support had obstructive apnea. Six patients died, one from sudden death, two from status epilepticus, and three from cardiac-related death (CRD). The correlation between the age at the time of PAB and CRD was significant (p<0.05), as was the correlation between the duration of artificial respiration and the grade of alveolar hypoplasia (p<0.01).

Conclusions: Medial defects and hypoplasia of the small pulmonary arteries, and alveolar hypoplasia were identified in relatively large number of patients with trisomy 18. In addition, many of these patients had risk factors affecting their prognosis. Our findings suggest that if parents request surgical intervention for their children with trisomy 18, the duration of artificial respiration should be kept as short as possible and palliative surgery should be performed as early as possible.

Key words: trisomy 18; open lung biopsy; pulmonary artery hypertension; convulsion; apnea

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