日本新生児成育医学会雑誌　第29巻　第1号　103～109頁（2017年）

日本新生児成育医学会雑誌　29(1)：103-109；2017　

受付日：平成28．01．12

受理日：平成28．08．23

経口Prostaglandin E₁製剤により静注用Lipo-Prostaglandin E₁製剤から離脱できた心疾患合併先天異常症の2例

Oral Prostaglandin E1 Derivative Use in Two Cases of Congenital Anomalies Presenting with Congenital Heart Disease

東京大学医学部附属病院　小児科

Department of Pediatrics, The University of Tokyo Hospital

設楽佳彦・高橋尚人・米田康太・井上毅信・青木良則・犬塚　亮・西村　力・土田晋也・岡　明

Yoshihiko SHITARA，Naoto TAKAHASHI，Kota YONEDA，Takanobu INOUE，Yoshinori AOKI，Ryo INUZUKA，Riki NISHIMURA，Shinya TSUCHIDA，Akira OKA

Key Words：patent ductus arteriosus，oral prostaglandin E₁，lipo-prostaglandin E₁，hypoplastic left heart syndrome，interruption of aortic arch

　動脈管依存性心疾患合併先天異常症に対し経口Prostaglandin E₁（以下PGE1）製剤を使用し在宅に移行できた2症例を経験した。症例1は在胎31週4日に出生した左心低形成症候群の男児。日齢183からLipo-prostaglandin E₁（以下Lipo-PGE₁）を漸減し，経口PGE₁を4μg/kg/日で開始した。その4日目から呼吸障害がみられ，7日目に移行を中止した。日齢329から再度経口PGE₁への変更を試みた。4μg/kg/日まで漸増し，日齢346 にLipo-PGE₁を中止でき1歳3か月に退院した。症例2は在胎37週0日に出生した大動脈弓離断症合併の18-トリソミーの男児。日齢100からLipo-PGE₁を漸減したが，動脈管内の血流の加速が出現し経口PGE₁を併用した。4μg/kg/日まで漸増し，Lipo-PGE1を中止でき日齢165に退院した。動脈管開存に経口PGE1が有効な可能性があり，特に在宅移行をめざす場合に有用と考えられた。

　In order to avoid intravenous administration of prostaglandin E1, we tried to use an oral prostaglandin E1 derivative in two cases of congenital anomalies that presented with ductus arteriosus-dependent congenital heart disease. The first patient was a boy delivered at 31 weeks of gestation. The patient had hypoplastic left heart syndrome. The second patient was a boy delivered at 37 weeks of gestation. The patient had trisomy 18 and interruption of the aortic arch. Both patients required intravenous administration of lipo-prostaglandin E1（ lipo-PGE1） immediately after birth. In the first case, the dose of lipo-PGE1 was gradually tapered and an oral prostaglandin E1 derivative（ 4μg/kg/day） was simultaneously started from the 183th day of life. After 4 days, the patient developed respiratory disturbance because of ductus arteriosus closure and administration of the oral prostaglandin E1 derivative was stopped. Oral prostaglandin E1 derivative use was attempted from the 329th day of life and a cessation of the administration of lipo-PGE1 was finally accomplished. In the second patient, the dose of lipo-PGE1 was decreased from the 100th day of life, and the administration of an oral prostaglandin E1 derivative was started. The cessation of administration of lipo-PGE1 was finally accomplished and the patient was discharged from the hospital on the 165th day of life.
　The use of oral prostaglandin E1 derivatives at a dose of 4μg/kg/day was effective for open ductus arteriosus and safe for patients with ductus arteriosus-dependent congenital heart disease. The oral use of a prostaglandin E1 derivative may be useful for patients that will be discharged from the hospital and taken care of at home.