北村 絵里, 山本 祐華, 板倉 敦夫

Eri KITAMURA, Yuka YAMAMOTO, Atsuo ITAKURA

順天堂大学産婦人科

Department of Obstetrics and Gynecology, Juntendo University Faculty of Medicine

キーワード : ductus venosus agenesis, sinus of valsalva aneurysm, fetal echocardiography, prenatal diagnosis, venous malformation

先天性静脈管欠損では心臓の前負荷調節が行われないことから胎児心不全を来す可能性があるため，臍帯静脈の流入部の評価とともに心機能の評価を行い，胎児心不全の徴候を見逃さないことが重要である．また，非常に稀な疾患である先天性バルサルバ洞瘤は，破裂，血栓や冠動脈圧迫などのリスクを有するため，生後継続した慎重な管理が求められる．今回先天性静脈管欠損とバルサルバ洞瘤の2つの稀な疾患を合併した症例を経験したため報告する．症例は29歳，1妊0産の初産婦．妊娠26週の胎児超音波検査で著明に拡張した臍静脈が直接右心房へ流入していることから先天性静脈管欠損と診断し，また大動脈の走行異常と弁異常も疑われた．妊娠後期までに臍静脈の拡張は改善傾向であり，心不全徴候は認めなかった．妊娠39週で胎児機能不全のために緊急帝王切開術で分娩となり，1,972gの男児を出生した．生後の心臓超音波検査ではバルサルバ洞瘤，両大血管右室起始症，心房中隔欠損症，三尖弁異形成と診断された．さらに，食道閉鎖症および鎖肛も認め，VACTERL連合と診断された．本症例の先天性静脈管欠損は臍静脈が直接右心房へ流入するが，肝内で狭小化を伴うことから右心房への負荷を軽減し，心拡大を来たさずに経過したと考える．

Agenesis of ductus venosus (ADV) is a rare condition with a variable prognosis depending on the venous connection and accompanying complications. Congenital sinus of valsalva aneurysm (SVA) is an extremely rare condition that may induce rupture, thrombosis, or compression of the coronary artery. This report describes the case of a 26-year-old primipara woman who presented a dilated umbilical vein going directly into the right atrium, which was diagnosed as extrahepatic drainage of ADV and aortic valve abnormality at 26 weeks of gestation. There was no sign of cardiomegaly or cardiac dysfunction during gestation. The dilation of the umbilical vein got better at 36 weeks of gestation. A male weighing 1,972 g was delivered by cesarean section due to non-reassuring fetal status at 39 weeks of gestation. After birth, SVA, double outlet right ventricle, atrial septal defect, and tricuspid dysplasia were confirmed. With esophageal atresia and imperforate anus, the baby was diagnosed as VACTERL association. In this case, although the umbilical vein ran directly into the right atrium, there was no cardiomegaly due to the kink in the umbilical vein right after the insertion to the abdomen, which would reduce the direct load to the heart. Since ultrasound findings of SVA during the fetal stage are very rare, the images presented here may be of interest to readers.