El-Kersh Karim, Homsy Elie, Daniels Curt J, Smith J Shaun
Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Disorders Medicine, University of Louisville, Louisville, KY, USA.
Department of Internal Medicine, Division of Pulmonary, Critical Care & Sleep Medicine,The Ohio State University Wexner Medical Center, USA.
Respir Med Case Rep. 2019 Apr 3;27:100833. doi: 10.1016/j.rmcr.2019.100833. eCollection 2019.
Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly that results in a left-to-right shunt. Based on the shunt fraction, PAPVR has a wide spectrum of presentations. If a significant left-to-right shunt is left unrepaired, pulmonary vascular remodeling can occur resulting in the development of pulmonary arterial hypertension (PAH). Furthermore, if the condition is associated with an atrial septal defect (ASD), the patient can develop shunt reversal and Eisenmenger's syndrome in setting of severe PAH. Management plans include close observation, surgical repair, and treatment with pulmonary artery vasodilator therapies. Here, we present multiple cases of PAPVR to highlight the wide spectrum of presentations and the individualized treatment for each case.
部分性肺静脉异位引流(PAPVR)是一种罕见的先天性异常,可导致左向右分流。根据分流分数,PAPVR有广泛的表现形式。如果显著的左向右分流未得到修复,可发生肺血管重塑,导致肺动脉高压(PAH)的发展。此外,如果该疾病与房间隔缺损(ASD)相关,在严重PAH的情况下,患者可出现分流逆转和艾森曼格综合征。治疗方案包括密切观察、手术修复以及肺动脉血管扩张剂治疗。在此,我们展示多例PAPVR病例,以突出其广泛的表现形式以及针对每个病例的个体化治疗。