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Case Reports in Cardiology
Volume 2016, Article ID 8164923, 5 pages
http://dx.doi.org/10.1155/2016/8164923
Case Report

Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

1Sanford Cardiovascular Institute, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA
2Department of Internal Medicine, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA
3Department of Pediatrics, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA
4Department of Radiology, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA

Received 30 July 2016; Revised 20 September 2016; Accepted 16 October 2016

Academic Editor: Christopher S. Snyder

Copyright © 2016 Amornpol Anuwatworn et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5–10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.