Page 7 - Journal of Structural Heart Disease Volume 2, Issue 5

Original Research Article
Journal of Structural Heart Disease, October 2016, Volume 2, Issue 5:208-212
DOI: http://dx.doi.org/10.12945/j.jshd.2016.013.15
Received: December 20, 2015 Accepted: January 23, 2016 Published online: October 2016
Partial Anomalous Pulmonary Venous Return into the Inferior Vena Cava in a 28-Year-Old Female: A Variant of Scimitar Syndrome Amenable to Interventional Treatment
Scimitar Syndrome Variant
Jana-K. Dieks, MD*, Michael Steinmetz, MD, Thomas Paul, MD, Heike E. Schneider, MD
Department of Pediatric Cardiology and Intensive Care Medicine, University Hospital, Georg-August-University Göttingen, Germany
Abstract
Scimitar syndrome is a rare congenital heart defect associated with right-sided partial anomalous pulmo- nary venous return (PAPVR) into the inferior vena cava (IVC). We describe the case of a 28-year-old female diagnosed with PAPVR with a typical curvilinear pat- tern of the right lower pulmonary vein—the so-called “scimitar sign”—on chest x-ray. Anatomical abnormali- ties on magnetic resonance imaging included a lower right pulmonary vein draining into the IVC and an additional vein connecting all right-sided pulmonary veins toward the left atrium. Because her anatomy was suitable, the patient underwent cardiac catheteriza- tion with occlusion of the anomalous right lower pulmonary vein with an Amplatzer Vascular Plug with excellent postinterventional results.
Copyright © 2016 Science International Corp.
Key Words
PAPVR • Amplatzer vascular plug • Catheter intervention • Congenital heart disease
Introduction
The term “scimitar syndrome” was rst employed by Neill et al. in 1960 to describe a rare type of partial anomalous pulmonary venous return (PAPVR) in
combination with a hypoplastic right lung that receives its blood supply from systemic arteries [1]. Associated features include dextroposition of the heart, bronchopulmonary sequestration, and various extracardiac and additional cardiac anomalies. The classic radiographic nding is the “scimitar sign” on chest x-ray resulting from the curved anomalous right pulmonary vein draining into the inferior vena cava (IVC). Two forms of scimitar syndrome can be di erentiated. In the infantile variant, patients usually present with tachypnea, heart failure, and pulmonary hypertension, and signi cant morbidity and mortality [2, 3]. In the childhood/adult form, patients are less severely a ected and may be asymptomatic until a diagnosis is established. The adult variant of scimitar syndrome is commonly associated with right lung hypoplasia, abnormalities of vascular supply, dextrocardia, and abnormalities of bronchial segmentation [4]. Treatment generally implies a surgical approach, and procedural details vary depending on individual anatomic and pathologic features, as well the surgeon’s preference. Procedures frequently include intra-atrial ba e repair and reim- plantation of the scimitar vein into the left atrium and, if necessary, simultaneous ligation of collateral arteries supplying the right lung, right lower lobe
* Corresponding Author:
Jana-K. Dieks, MD
University Hospital, Georg-August University Göttingen
Department of Pediatric Cardiology and Intensive Care Medicine
Robert-Koch Str. 40, 37075 Göttingen, Germany
Tel.: +49 551 39 66203; Fax: +49 551 39 22561; E-Mail: jana.dieks@med.uni-goettingen.de
Fax +1 203 785 3346
E-Mail: jshd@scienceinternational.org http://structuralheartdisease.org/
© 2016 Journal of Structural Heart Disease Published by Science International Corp. ISSN 2326-4004
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