Pediatric Pulmonary Hypertension in the Netherlands

Abstract

Background-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. Methods and Results-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 (nϭ160; 5%), lung disease/hypoxemia (nϭ253; 8%), thromboembolic disease (nϭ5; Ͻ1%), and transient (nϭ2691; 82%) and progressive (nϭ154; 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 (nϭ36; iPAH) and PAH associated with CHD (nϭ111; PAH-CHD). Pulmonary arterial hypertension associated with CHD represented highly heterogeneous subgroups. Syndromes were frequently present, especially in progressive PAH (nϭ60; 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. Conclusions-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. (Circulation. 2011;124:1755-1764.)

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

“…Early evidence for the epidemiology of PH originated from adult registries; however, several newer pediatric international registries have contributed to our understanding of the disease in children as well. [1][2][3][4][5] Pediatric PH can be associated with a variety of underlying conditions, including congenital heart disease (CHD), connective-tissue disorders, pulmonary disease, and endocrine, metabolic, and infectious diseases such as HIV. PH is usually progressive from infancy to adulthood and associated with poor prognosis.…”

Registries for paediatric pulmonary hypertension

“…In the UK, the incidence and prevalence of idiopathic PAH (IPAH) was 0.48 and 2.1 per million children, respectively [8]. In the Netherlands, the incidence and prevalence of IPAH was 0.7 and 4.4 per million children, whereas PAH associated with congenital heart disease (PAH-CHD) had an incidence of 2.2 and prevalence of 15.6 per million [9]. Of note, the PAH disease spectrum may somewhat differ in the individual tertiary centre and in multicentre registries [13].…”

“…The estimated prevalence of PAH is 15-50 cases per million adults [4-6] and 2-16 cases per million children [7][8][9]. In certain at-risk groups, however, the occurrence of PAH is substantially higher.…”

Right Ventricular Remodeling in Idiopathic and Scleroderma‐Associated Pulmonary Arterial Hypertension: Two Distinct Phenotypes

“…22 Earlier cohort studies have demonstrated improved outcomes in adult patients with PAH related to Eisenmenger syndrome (ES) compared with patients with IPAH and have attributed this improved survival in part to better adaptation of the RV, evidenced by hypertrophy and increased RV mass. [23][24][25][26] Similarly, Rich and colleagues 27 have noted differences in RV size and morphology when contrasting the postmortem RVs of a long-term survivor and a short-term survivor with IPAH, highlighting the importance of RV hypertrophic response to increased afterload. These observations suggest a potential relationship between RV hypertrophy and outcomes in PAH.…”