Ivy D D, Feinstein J A, Humpl T, Rosenzweig E B
University of Colorado Denver School of Medicine and The Children's Hospital, United States.
Prog Pediatr Cardiol. 2009 Dec 1;27(1-2):13-23. doi: 10.1016/j.ppedcard.2009.09.004.
Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, hemoglobinopathies, hepatopulmonary syndrome, portopulmonary hypertension and HIV. Three classes of drugs have been extensively studied for the treatment of IPAH in adults: prostanoids (epoprostenol, treprostinil, iloprost, beraprost), endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan), and phosphodiesterase inhibitors (Sildenafil, tadalafil). These medications have been used in treatment of children with pulmonary arterial hypertension, although randomized clinical trial data is lacking. As pulmonary vasodilator therapy in certain diseases may be associated with adverse outcomes, further study of these medications is needed before widespread use is encouraged.
儿童中，对除先天性心脏病以外的肺动脉高压病因的认识正在增加。诊断和治疗肺动脉高压的任何潜在病因对于肺动脉高压的最佳管理至关重要。本文讨论了有关儿童中与肺动脉高压相关的几种疾病的现有知识：特发性肺动脉高压（IPAH）、肺毛细血管瘤病、肺静脉闭塞病、血红蛋白病、肝肺综合征、门肺高压和HIV。三类药物已被广泛研究用于治疗成人IPAH：前列环素类药物（依前列醇、曲前列尼尔、伊洛前列素、贝拉前列素）、内皮素受体拮抗剂（波生坦、西他生坦、安立生坦）和磷酸二酯酶抑制剂（西地那非、他达拉非）。这些药物已用于治疗儿童肺动脉高压，尽管缺乏随机临床试验数据。由于某些疾病中的肺血管扩张剂治疗可能与不良后果相关，因此在鼓励广泛使用之前，需要对这些药物进行进一步研究。
