日本新生児成育医学会雑誌 34(2)：159-164；2022
| 日本新生児成育医学会雑誌 第34巻 第2号 38 ～ 43頁（2022年） |
| 受付日：2021．03．01 |
| 受理日：2022．02．10 |
| 酸素化不良が遷延し新生児期に発見された先天性孤立性一側肺動脈欠損症の1 例 |
| A Case of Congenital Isolated Unilateral Absence of the Pulmonary Artery Diagnosed in the Neonatal Period after Prolonged Oxygen Desaturation |
| ＊ 1 福岡市立こども病院 新生児科，＊ 2 福岡市立こども病院 胎児循環器科 |
| ＊ 1 Department of Neonatology, Fukuoka Children’s Hospital，＊ 2 Department of Fetal Cardiology, Fukuoka Children’s Hospital |
| 古園美和＊ 1・楠田 剛＊ 1・芹田陽一郎＊ 1・野口雄史＊ 1・市山正子＊ 1・金城唯宗＊ 1・漢 伸彦＊ 2 |
| Miwa FURUZONO ＊ 1，Takeshi KUSUDA ＊ 1，Yoichiro SERITA ＊ 1，Yushi NOGUCHI ＊ 1，Masako ICHIYAMA ＊ 1，Tadamune KINJO ＊ 1，Nobuhiko KAN ＊ 2 |
| Key Words：isolated absence of pulmonary artery，cyanosis，neonate |
| 一側肺動脈欠損は稀な疾患であり多くは心内形態異常を伴うが，伴わない場合は孤立性一側肺動脈欠損症と呼ば
れる。今回，酸素化不良が遷延し新生児期に発見された症例を経験した。
症例は日齢1 の女児。在胎39 週3 日，3,512g で仮死なく出生。出生後SpO2 低値であり酸素投与を開始された。 超音波検査で出生後の肺血管抵抗低下の遅れによる低酸素血症と診断して，酸素投与を継続したが酸素化不良は遷 延した。日齢9 に造影CT 検査を施行，右肺動脈の起始部が欠損しており孤立性一側肺動脈欠損症と診断した。日 齢21，心臓カテーテル検査のpulmonary vein wedge angiography で右肺動脈の末梢側が造影された。大動脈か らの側副血行路は同定できなかった。右肺血流を確保する目的で日齢45 に上行大動脈から右肺動脈へcentral shunt を造設した。術中所見では胎児期に右肺を栄養していた右動脈管の閉鎖を認めた。 今回，高肺血流を認めない孤立性一側肺動脈欠損症を経験した。先天性心疾患の鑑別に心臓超音波検査をする際 は，左右肺動脈の分枝の確認が重要である。 |
| Unilateral absence of the pulmonary artery is extremely rare and usually accompanied by intracardiac malformation.
Without any intracardiac malformations, it is known as isolated unilateral absence of the pulmonary artery. We encountered
such a case in a neonate with prolonged oxygen desaturation.
The patient was a one-day-old female neonate. Her gestational age was 39 weeks and 3 days, birth weight was 3,512 g, and there was no asphyxia. After birth, her oxygen saturation was low and supplemental oxygen was provided. Ultrasound indicated hypoxemia due to delayed decrease in postnatal pulmonary vascular resistance, so we continued administration of supplemental oxygen, but there was no improvement. A contrast-enhanced CT was taken on day 9 after birth, which revealed a deficit in the origin part of the right pulmonary artery. We therefore diagnosed this case as an isolated unilateral absence of the pulmonary artery. On day 21 after birth, her right pulmonary artery was enhanced by pulmonary vein wedge angiography. Collateral flow from aorta to the right lung was not identified. On day 45 after birth, a central shunt procedure was conducted to maintain her right pulmonary blood flow. Intraoperative findings concluded that the right ductus arteriosus that was supposedly feeding into the right lung during the fetal period had occluded after birth. We experienced a case of unilateral absence of the pulmonary artery without increased pulmonary blood flow. When performing echocardiography to distinguish congenital heart disease, it is important to confirm the bifurcation of both pulmonary arteries. |