Case Report Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy Amornpol Anuwatworn, 1 Maheedhar Gedela, 2 Edgard Bendaly, 3 Julia A. Prescott-Focht, 4 Jimmy Yee, 1 Richard Clark, 1 and Orvar Jonsson 1 1 Sanford Cardiovascular Institute, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA 2 Department of Internal Medicine, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA 3 Department of Pediatrics, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA 4 Department of Radiology, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA Correspondence should be addressed to Maheedhar Gedela; maheedhargedela@gmail.com Received 30 July 2016; Revised 20 September 2016; Accepted 16 October 2016 Academic Editor: Christopher S. Snyder Copyright © 2016 Amornpol Anuwatworn et al. his 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. 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 conirmed 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 Tadalail. Ater six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-let shunt. his case illustrates the potential beneit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment. 1. Introduction Sinus venosus atrial septal defect (SVASD) is one of the major categories of atrial septal defect (ASD). SVASD was irst reported in 1858 and accounts for 5–10% of cases of ASD [1, 2]. It usually coexists with partial anomalous pulmonary venous return (PAPVR), which leads to additional let-to-right shun- ting. SVASD is generally unrecognized until the ith decade of life when it presents as right heart failure and/or pul- monary hypertension [3]. SVASDs are usually located near the superior or inferior vena cava entry into the right atrium. his interatrial communication leads to volume overload, right-sided chamber dilation, and ultimately pulmonary hypertension [1, 3]. In this paper, we present a case of pulmonary artery hypertension and Eisenmenger syndrome due to a superior form of SVASD. We will also discuss the beneicial role of vasodilator therapy in reversing the right- to-let shunt, which may stabilize a patient for future surgery. 2. Case Report A 43-year-old Hispanic woman was transferred to our faci- lity with hypoxia following laparoscopic hysteroscopy and myomectomy. She reported experiencing exertional dysp- nea for four years. She also disclosed a history of car- diomegaly that was discovered on both a chest X-ray and through transthoracic echocardiography (TTE) nine years ago at another hospital, and she had been treated for postpartum cardiomyopathy. Physical examination revealed the following: heart rate, 90 beats/minute; blood pressure, 126/85 mmHg; respiratory rate, 24 breaths/minute; and oxy- gen saturation, 88% on 15 liters of oxygen by nasal cannula. Hindawi Publishing Corporation Case Reports in Cardiology Volume 2016, Article ID 8164923, 5 pages http://dx.doi.org/10.1155/2016/8164923