title: Functional total anomalous pulmonary venous connection via levoatriocardinal vein.
abstract: We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the "dead-end" left atrium, the pulmonary venous flow had a to-and-fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left-side heart obstruction.
authors.affiliation: Division of Cardiology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Taiyu
authors.initials: T
authors.lastname: Hayashi
authors.affiliation: Division of Fetal Medicine, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Katsusuke
authors.initials: K
authors.lastname: Ozawa
authors.affiliation: Division of Fetal Medicine, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Rika
authors.initials: R
authors.lastname: Sugibayashi
authors.affiliation: Division of Fetal Medicine, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Seiji
authors.initials: S
authors.lastname: Wada
authors.affiliation: Division of Cardiology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ono
mesh_terms: D000328:Adult; D004452:Echocardiography; D005260:Female; D006325:Heart Atria / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D021621:Imaging, Three-Dimensional; D007231:Infant, Newborn; D011247:Pregnancy; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011668:Pulmonary Veno-Occlusive Disease / Q000151:congenital / Q000175:diagnosis / Q000209:etiology*; D014057:Tomography, X-Ray Computed
keywords: atrial septum; fetal echocardiography; levoatriocardinal vein; pulmonary veins; total anomalous pulmonary venous connection