Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Circulation. 2011 Oct 18;124(16):1755-64. doi: 10.1161/CIRCULATIONAHA.110.969584. Epub 2011 Sep 26.
Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH.
Two registries were used to retrospectively identify children (0-17 years) with PH. Overall, 3263 pediatric patients were identified with PH due to left heart disease (n=160; 5%), lung disease/hypoxemia (n=253; 8%), thromboembolic disease (n=5; <1%), and transient (n=2691; 82%) and progressive (n=154; 5%) PAH. Transient PAH included persistent PH of the newborn and children with congenital heart defects (CHD) and systemic-to-pulmonary shunt, in whom PAH resolved after successful shunt correction. Progressive PAH mainly included idiopathic PAH (n=36; iPAH) and PAH associated with CHD (n=111; PAH-CHD). Pulmonary arterial hypertension associated with CHD represented highly heterogeneous subgroups. Syndromes were frequently present, especially in progressive PAH (n=60; 39%). Survival for PAH-CHD varied depending on the subgroups, some showing better and others showing worse survival than for iPAH. Survival of children with Eisenmenger syndrome appeared worse than reported in adults. For iPAH and PAH-CHD, annual incidence and point prevalence averaged, respectively, 0.7 and 4.4 (iPAH) and 2.2 and 15.6 (PAH-CHD) cases per million children. Compared to studies in adults, iPAH occurred less whereas PAH-CHD occurred more frequently.
Pediatric PH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Incidence of pediatric iPAH is lower whereas incidence of pediatric PAH-CHD is higher than reported in adults. Pediatric PAH-CHD represents a heterogeneous group with highly variable clinical courses.
儿科肺动脉高压（PH）和肺动脉高压（PAH）的发病率和患病率尚不清楚。本研究描述了荷兰在 15 年内儿科 PH 的全国流行病学特征以及儿科 PAH 的临床病程。
使用两个登记处回顾性确定 PH 患儿（0-17 岁）。总体而言，确定了 3263 例因左心疾病（n=160；5%）、肺部疾病/低氧血症（n=253；8%）、血栓栓塞性疾病（n=5；<1%）和一过性（n=2691；82%）和进行性（n=154；5%）PAH 而患有 PH 的儿科患者。一过性 PH 包括新生儿持续性 PH 和患有先天性心脏病（CHD）和体肺分流的儿童，分流矫正后 PAH 可得到解决。进行性 PAH 主要包括特发性 PAH（n=36；iPAH）和与 CHD 相关的 PAH（n=111；PAH-CHD）。与 CHD 相关的 PAH 代表高度异质的亚组。综合征经常存在，尤其是在进行性 PAH 中（n=60；39%）。PAH-CHD 的生存率取决于亚组，有些亚组的生存率好于 iPAH，而有些亚组的生存率差于 iPAH。Eisenmenger 综合征患儿的生存率似乎比成人报道的更差。对于 iPAH 和 PAH-CHD，每年的发病率和点患病率平均分别为每百万儿童 0.7 和 4.4（iPAH）和 2.2 和 15.6（PAH-CHD）例。与成人研究相比，iPAH 发生的频率较低，而 PAH-CHD 发生的频率较高。
儿科 PH 的特点是存在各种特定于年龄的诊断，其中大多数为一过性 PAH 形式。儿科 iPAH 的发病率较低，而儿科 PAH-CHD 的发病率高于成人报道。儿科 PAH-CHD 是一组异质性很强的疾病，临床表现差异很大。
