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
Pediatric Cardiology and Cardiac Surgery 39(1): 25-30 (2023)

症例報告Case Report

ミルリノン投与後に動脈管の再開大を認めた動脈管依存性先天性心疾患の検討Milrinone Re-Dilates the Ductus Arteriosus in Patients with Ductus Arteriosus-Dependent Congenital Heart Diseases

1京都大学医学部附属病院 小児科Department of Pediatrics, Graduate School of Medicine, Kyoto University ◇ Kyoto, Japan

2あいち小児保健医療総合センター 循環器科Department of Pediatric Cardiology, Aichi Children’s Health and Medical Center ◇ Aichi, Japan

受付日:2022年7月16日Received: July 16, 2022
受理日:2023年2月1日Accepted: February 1, 2023
発行日:2023年2月1日Published: February 1, 2023

動脈管依存性先天性心疾患にとって動脈管開存は生存に必須であるが,経過中に動脈管が狭小化する症例が存在する.今回我々は,PGE1製剤投与中に狭小化した動脈管がミルリノン投与後に再開大した左心低形成症候群(HLHS)と重症肺動脈弁狭窄(cPS)の症例を経験した.HLHS症例は両側肺動脈絞扼術を施行され,Norwood術待機中に動脈管が狭小化した.lipo-PGE1からCD-PGE1に変更,投与量を増量されたが動脈管は開大せず,ミルリノンを開始後0.2 µg/kg/minまで増量された.cPS症例は,経皮的バルーン肺動脈弁形成術(PTPV)待機中に動脈管が狭小化した.lipo-PGE1からCD-PGE1に変更,投与量を増量されたが動脈管は開大せず,ミルリノンを開始後0.4 µg/kg/minまで増量された.いずれもミルリノン開始後,速やかに動脈管は再開大した.動脈管依存性先天性心疾患では動脈管狭小化時に速やかな治療が必要であるが,我々が経験した2症例においてはミルリノン投与が著効した.その作用機序について考察を加え報告する.

Treatment of patent ductus arteriosus is crucial for the survival of patients with ductus arteriosus (DA)-dependent congenital heart diseases. Prostaglandin E1 (PGE1) agents are commonly used to keep the DA open; however, maintaining an appropriately sized DA is difficult. We saw a patient with hypoplastic left heart syndrome (HLHS) and critical pulmonary stenosis (cPS) whose DAs had become narrow and were re-dilated by milrinone. The patient with HLHS had bilateral pulmonary artery banding and was awaiting the Norwood procedure. The diameter of the DA instantly decreased from 6.4 mm to 3.0 mm at 18 days of age during the administration of lipo-PGE1. Although the administration of CD-PGE1 was begun instead of lipo-PGE1, the DA remained narrow. Therefore, we initiated the administration of milrinone at a dosage of up to 0.2 µg/kg/min. The DA quickly re-dilated to a sufficient size and remained open until the Norwood procedure. The patient with cPS was scheduled for percutaneous transluminal pulmonary valvuloplasty (PTPV). The diameter of the DA decreased from 3.7 mm to 1.2 mm at 3 days of age. The agent used was changed from lipo-PGE1 to CD-PGE1, but the DA remained narrow. As a result, we began administering milrinone and gradually increased the dosage to 0.4 µg/kg/min. The DA dilated after the initiation of milrinone treatment and remained open until the scheduled PTPV date. A narrow DA is a critical problem for patients with DA-dependent congenital heart disease, and additional treatments are required to avoid DA closure. Milrinone is another option besides PGE1 agents to re-dilate the DA.

Key words: ductus arteriosus; congenital heart disease; phosphodiesterase 3 inhibitor; prostaglandin E1; milrinone

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