Functional total anomalous pulmonary venous connection via levoatriocardinal vein

Pediatrics International : Official Journal of the Japan Pediatric Society
Taiyu HayashiHiroshi Ono

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.

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Related Concepts

2D Echocardiography
Right Atrial Structure
Congenital Heart Defects
Pulmonary Circulation
Pulmonary Veins
Pulmonary Veno-Occlusive Disease (Disorder)
Tomography, X-Ray Computerized Axial
Imaging, Three-Dimensional, Computer Assisted
Birth
Differential Diagnosis

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