Osakwe Onyekachukwue, B Das Bibhuti
Department of Pediatrics, Division of Cardiology, University of Mississippi Medical Center, Jackson, MS 39216, USA.
Children (Basel). 2022 Sep 22;9(10):1444. doi: 10.3390/children9101444.
Patients with nephrotic syndrome (NS) are at an increased risk for thromboembolic events, such as deep venous and arterial thrombosis and pulmonary embolism (PE). In general, PE in children differs from adults in incidence, predisposition, pathophysiology, presenting symptoms, and management strategies. There is a lack of treatment guidelines for PE in children, and the management strategies are mostly extrapolated from adult data. This case report highlights the presentation of acute cor pulmonale due to massive PE associated with NS and a successful pulmonary endarterectomy that reversed the child's pulmonary hypertension and normalized right ventricular function.
肾病综合征（NS）患者发生血栓栓塞事件的风险增加，如深静脉和动脉血栓形成以及肺栓塞（PE）。一般来说，儿童PE在发病率、易患因素、病理生理学、临床表现和治疗策略方面与成人不同。目前缺乏针对儿童PE的治疗指南，其治疗策略大多是从成人数据推断而来。本病例报告重点介绍了与NS相关的大面积PE导致的急性肺心病表现以及成功的肺动脉内膜剥脱术，该手术逆转了患儿的肺动脉高压并使右心室功能恢复正常。
