Current challenges in pediatric pulmonary hypertension.
Current challenges in pediatric pulmonary hypertension.
复制标题
小儿肺动脉高压的当前挑战。
DOI：
10.1055/s-0033-1356461
复制
发表时间：
2013
影响因子：
3.2
通讯作者：
Ivy,DavidDunbar
中科院分区：
文献类型：
--
作者：
Takatsuki,Shinichi;Ivy,DavidDunbar
儿科人群中的肺动脉高压（PAH）与多种潜在疾病和病因、显着的发病率和死亡率相关。在大多数患者中，儿童 PAH 是特发性的或与先天性心脏病 (CHD) 相关，肺动脉高压 (PH) 与结缔组织病相关，这是儿童的罕见原因。儿科 PH 的分类通常遵循 WHO 分类，但对胎儿起源的 PH 和发育异常的重要性的认识导致了新的儿科特异性分类的形成。荷兰的发病率数据显示，每百万儿童特发性 PAH 的年发病率和点患病率分别为 0.7 和 4.4，相关肺动脉高压-CHD 病例的年发病率和点患病率分别为 2.2 和 15.6。尽管新型选择性肺血管扩张剂的治疗可以改善儿科人群的血流动力学和功能，但儿童的治疗很大程度上取决于成人循证研究的结果和临床医生治疗儿童的经验。最近一项关于西地那非及其长期扩展的随机临床试验在美国和欧洲提出了不同的建议。
Pulmonary arterial hypertension (PAH) in the pediatric population is associated with a variety of underlying diseases and causes, significantly morbidity and mortality. In the majority of patients, PAH in children is idiopathic or associated with congenital heart disease (CHD), with pulmonary hypertension (PH) associated with connective tissue disease, a rare cause in children. Classification of pediatric PH has generally followed the WHO classification, but recognition of the importance of fetal origins of PH and developmental abnormalities have led to the formation of a new pediatric-specific classification. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic PAH and 2.2 and 15.6 for associated pulmonary arterial hypertension-CHD cases per million children. Although the treatment with new selective pulmonary vasodilators offers hemodynamic and functional improvement in pediatric populations, the treatments in children largely depend on results from evidence-based adult studies and experience of clinicians treating children. A recent randomized clinical trial of sildenafil and its long-term extension has led to disparate recommendations in the United States and Europe.