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Idiopathic Pulmonary Arterial Hypertension in Children: A Review

机译：儿童特发性肺动脉高压的研究进展

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Pediatric pulmonary hypertension (PH) is a heterogeneous disorder that leads to significant morbidity and mortality if left untreated. The Nice WHO classification from 2013 classifies PH into five groups: broadly, Group I pulmonary arterial hypertension (PAH), which includes idiopathic PAH, PAH associated with congenital heart disease and others; Group II comprises post-capillary PH (left heart disease), Group III comprises lung disease, Group IV thromboembolic disease and Group V miscellaneous causes of PH. The majority of pediatric PH comprise either Group I or Group III PH, but often children manifest features of several groups and are treated with targeted therapy. PAH in children requires a multifaceted approach. The pathogenesis is complex and it involves pulmonary vasoconstriction, endothelial dysfunction, inflammation and cell proliferation. Large multicenter and international registries have been formed with the aim of furthering understanding of this disease and developing appropriate therapeutic guidelines for managing pediatric PH. In this review, we describe the epidemiology, pathophysiology, clinical presentation, diagnosis and management of pediatric pulmonary hypertension.
机译：小儿肺动脉高压（PH）是一种异质性疾病，如果不及时治疗会导致明显的发病率和死亡率。从2013年开始的尼斯WHO分类将PH分为五类：广泛地，第一类肺动脉高压（PAH），包括特发性PAH，与先天性心脏病相关的PAH等。 II组包括毛细血管后PH（左心病），III组包括肺部疾病，IV组血栓栓塞性疾病和V组PH的其他原因。多数儿科PH包括I组或III组PH，但是儿童经常表现出几类特征，并接受靶向治疗。儿童的PAH需要采取多方面的方法。发病机理很复杂，涉及肺血管收缩，内皮功能障碍，炎症和细胞增殖。已经形成了大型的多中心和国际注册机构，其目的是加深对这种疾病的了解，并制定适当的治疗指南来管理小儿PH。在这篇综述中，我们描述了小儿肺动脉高压的流行病学，病理生理学，临床表现，诊断和管理。

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