Anuwatworn Amornpol, Gedela Maheedhar, Bendaly Edgard, Prescott-Focht Julia A, Yee Jimmy, Clark Richard, Jonsson Orvar
Sanford Cardiovascular Institute, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA.
Department of Internal Medicine, University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA.
Case Rep Cardiol. 2016;2016:8164923. doi: 10.1155/2016/8164923. Epub 2016 Nov 14.
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.
静脉窦型房间隔缺损是一种罕见的先天性房间隔交通缺损，位于右心房与腔静脉交界处。它占所有房间隔缺损病例的5% - 10%。由于其发病率低且解剖结构复杂，静脉窦型房间隔缺损的诊断给临床带来了挑战，可能会延误诊断和治疗。先进的心脏影像学检查是诊断这一临床病症以及描绘其解剖结构和任何相关交通情况的有用工具。手术矫正该异常是主要治疗方法。我们讨论一位43岁的西班牙裔女性患者，她在腹腔镜子宫肌瘤切除术后出现呼吸困难和低氧血症。她九年前在另一家医院被诊断为围产期心肌病。经食管超声心动图和胸部计算机断层血管造影证实诊断为静脉窦型房间隔缺损。还发现她患有肺动脉高压和艾森曼格综合征。在一项血流动力学研究中，她对血管扩张剂有反应，随后接受了安立生坦和他达拉非治疗。六个月后，她的症状改善，肺动脉高压减轻。我们还观察到右向左分流逐渐逆转。该病例说明了血管扩张剂治疗在逆转艾森曼格生理状态方面的潜在益处，这可能会促使将房间隔缺损的手术修复作为主要治疗方法。
