title: Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005.
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.
  
  
  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 (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.
  
  
  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.
authors.affiliation: Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Rosa Laura E
authors.initials: RL
authors.lastname: van Loon
authors.forename: Marcus T R
authors.initials: MT
authors.lastname: Roofthooft
authors.forename: Hans L
authors.initials: HL
authors.lastname: Hillege
authors.forename: Arend D J
authors.initials: AD
authors.lastname: ten Harkel
authors.forename: Magdalena
authors.initials: M
authors.lastname: van Osch-Gevers
authors.forename: Tammo
authors.initials: T
authors.lastname: Delhaas
authors.forename: Livia
authors.initials: L
authors.lastname: Kapusta
authors.forename: Jan L M
authors.initials: JL
authors.lastname: Strengers
authors.forename: Lukas
authors.initials: L
authors.lastname: Rammeloo
authors.forename: Sally-Ann B
authors.initials: SA
authors.lastname: Clur
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
authors.forename: Rolf M F
authors.initials: RM
authors.lastname: Berger
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension; D006330:Heart Defects, Congenital / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000209:etiology*; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D009426:Netherlands / Q000453:epidemiology; D012042:Registries; D012189:Retrospective Studies