Open Access

CASE REPORT

One-Stage Correction with Intra- and Extraatrial Rerouting of Anomalous Systemic and Pulmonary Venous Return and Intraventricular Repair of Double Outlet Right Ventricle in a Patient with Heterotaxy Syndrome

by Helena Staehler¹, Carina Hopfner², Masamichi Ono^1,*, Jürgen Hörer¹

1 Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
2 Department of Pediatric Cardiology and Pediatric Intensive Care, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany

* Corresponding Author: Masamichi Ono. Email:

Congenital Heart Disease 2022, 17(1), 25-30. https://doi.org/10.32604/CHD.2022.018366

Received 15 July 2021; Accepted 16 August 2021; Issue published 26 October 2021

Abstract

We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome. The cardiac malformations included dextrocardia, double outlet right ventricle, pulmonary stenosis, interrupted inferior vena cava, hemiazygos continuation and total anomalous pulmonary venous return. One-stage correction was performed. The atrial procedure consisted of intra- and extraatrial rerouting of the anomalous systemic and pulmonary venous return. The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit. The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta. The right ventricular outflow was ultimately remodeled using a valved conduit. For better perception of the complex morphology, a three-dimensional model was designed, using CT scan images. This proved to be very useful for surgical planning, especially with regard to the intraatrial reconstruction of the systemic and pulmonary venous rerouting.

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