林 彩世¹, 小澤 克典¹, 室本 仁¹, 杉林 里佳¹, 小杉 洋平², 柴田 優花², 池ノ上 学³, 和田 誠司¹, 伊藤 裕司², 左合 治彦¹

Sayaka HAYASHI¹, Katsusuke OZAWA¹, Jin MUROMOTO¹, Rika SUGIBAYASI¹, Youhei KOSUGI², Yuka SHIBATA², Satoru IKENOUE³, Seiji WADA¹, Yusi ITO², Haruhiko SAGO¹

¹国立成育医療研究センター周産期・母性診療センター胎児診療科, ²国立成育医療研究センター周産期・母性診療センター新生児科, ³慶応義塾大学病院産婦人科

¹Division of Fetal Medicine, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, ²Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, ³Department of Obstetrics and Gynecology, Keio University School of Medicine

キーワード : fetal hepatic hemangioma, high-output cardiac failure, combined cardiac output, umbilical venous flow volume, fetal cardiovascular function

胎児巨大肝血管腫は動静脈シャントによって高拍出性心不全を呈し，胎盤循環も悪化する．そのため胎盤循環を含めた胎児循環の評価を行い適切な娩出時期を決定する必要があるが，いまだ確立した評価方法がない．臍静脈血流量（UVFV）の総心拍出量（CCO）における割合（UVFV/CCO）は胎盤循環の指標となることが報告されており，この指標を含めた心循環機能評価を行うことで早期に病状の変化をとらえた胎児巨大肝血管腫の症例を報告する．妊娠27週に心胸郭断面積比（CTAR）48.5％の心拡大と軽度の僧帽弁・三尖弁逆流を認めた．CCO 1,214 ml/min，UVFV 350 ml/minと増加を認めたが，UVFV/CCOは28.9％と保たれ，羊水量は正常で胎児水腫を認めなかった．しかし，妊娠30週1日にCTAR 62.9％と増悪，CCO 1,620 ml/min，UVFV 236 ml/minとなり，UVFV/CCOは14.6％に低下した．胎児水腫は認めなかったが羊水過少となり，Biophysical profiling score 2/10点と低値であった．妊娠30週4日に胎児心拍数陣痛図において基線細変動の減少が出現し，胎児機能不全と診断，緊急帝王切開術を施行した．児は2,340 g，Apgar score1点（1分値），5点（5分値），臍帯動脈血pH 7.254で出生した．日齢1.5に肝血管腫に対してコイル塞栓術を行ったが原病のコントロールがつかず，グラム陽性球菌による敗血症で日齢70に永眠した．UVFV/CCOの減少が巨大肝血管腫による胎児胎盤循環の悪化を反映すると思われた．

Fetal huge hepatic hemangioma causes high-output cardiac failure due to arteriovenous shunting that requires assessment of fetal cardiovascular function. The ratio of umbilical venous flow volume (UVFV) to combined cardiac output (CCO) is reported to be an indicator of placental circulation. We report a case of fetal huge hepatic hemangioma with assessment of fetal cardiovascular function using the ratio of UVFV to CCO (UVFV/CCO). At 27 weeks of gestation, cardiomegaly (cardiothoracic area ratio (CTAR) 48.5%) and mild mitral and tricuspid valve regurgitation were detected on fetal ultrasonography. Increased CCO (1,214 ml/min) and UVFV (350 ml/min) but normal UVFV/CCO (28.9%) were observed. The amniotic fluid volume was normal with no sign of hydrops. At 30 weeks +1 day of gestation, the CTAR (62.9%) and CCO (1,620 ml/min) were increased, and UVFV (236 ml/min) was decreased, resulting in low UVFV/CCO (14.6%). Although there was no sign of hydrops, oligohydramnios was seen, and the biophysical profiling score (BPS) was 2/10 points. At 30 weeks +4 days of gestation, emergency cesarean section was performed because of decreased baseline variability on cardiotocography (CTG). The birth weight of the male newborn was 2,340 g, the pH of the umbilical arterial blood was 7.254, and the Apgar scores at 1 and 5 minutes were 1 and 5 points, respectively. Although coil embolization of the right hepatic arteries was performed at 1 and 5 days of age, the size of the hemangioma did not decrease. He died at 70 days of age due to sepsis caused by Gram-positive cocci. The UVFV/CCO decreased along with deterioration of fetoplacental circulation in a case of fetal huge hepatic hemangioma.