Childhood idiopathic pulmonary arterial hypertension: a national cohort study

Moledina, Shahin; Hislop, Alison A.; Foster, H.; Schulze‐Neick, Ingram; Haworth, Sheila G.

doi:10.1136/hrt.2009.182378

Cited by 204 publications

(218 citation statements)

References 25 publications

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“…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.…”

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confidence: 99%

“…In France, for example, the prevalence of paediatric PAH was estimated to be 2.2 cases per million children [7]. 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].…”

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confidence: 99%

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Registries for paediatric pulmonary hypertension

Hansmann

Hoeper

2013

Eur Respir J

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@ERSpublications Paediatric pulmonary arterial hypertension should be treated specifically and differently from adult pulmonary arterial hypertension http://ow.ly/mzPDT Pulmonary arterial hypertension (PAH) is a progressive, angio-obliterative disease leading to increased pulmonary vascular resistance, right heart failure, and death in ,25-60% of PAH patients 5 years after diagnosis [1][2][3]. 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. For example, the prevalence is 0.5% in HIV-infected patients [10] and 4-6% in schistosomiasis [11]. These diseases are far more common in developing countries with limited health care and, thus, the real burden of PAH worldwide is probably underestimated.Historically, extrapolation from adult pulmonary hypertension studies has been used to make assumptions on the disease in children; however, this approach is neither validated nor appropriate [12]. The recent comprehensive analysis of paediatric registries allowed predictions on the epidemiology, clinical practice and outcome of PAH in childhood. In France, for example, the prevalence of paediatric PAH was estimated to be 2.2 cases per million children [7]. 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]. In a British cohort study, the 5-year survival for children with IPAH was only 75% with a freedom from death or transplantation of only 57% (1986 and 2000 with follow-up to 2007) [8].Patients, family members and healthcare providers should be aware of the unique features of paediatric pulmonary hypertensive vascular disease (PPHVD) [14,15]. These include the fetal origins, developmental and adaptive aspects of pulmonary vascular disease (PVD) and right ventricular dysfunction (RVD) in both childhood-and adult-onset disease. The proposal for a new classification of PPHVD (Panama, 2011 [14]) aims to incorporate such specific paediatric aspects that are not adequately addressed in the current classification of pulmonary hypertension (Dana Point, CA, USA, 2008) [16]. The Panama proposal of PPHVD is still very new, not broadly (internationally) accepted yet, and probably will undergo further modifications, according to international meetings (e.g., the PH World Symposium, Nice, France, 2013) and feedback from healthcare providers.

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confidence: 99%

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confidence: 99%

Registries for paediatric pulmonary hypertension

Hansmann

Hoeper

2013

Eur Respir J

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“…Finally, untreated paediatric PAH has been reported to be associated with a median survival of 10 months, which is worse compared with adults (median untreated survival of 2.8 yrs) [12]. However, these unfavourable survival times for paediatric PAH have been challenged by recent data [5,13].…”

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confidence: 95%

“…Haemodynamic alterations, including increased pulmonary arterial pressure and resistance, as per definition of the disease, are present in both children and adults, and dyspnoea at exertion is the most common clinical symptom in both populations [3,4]. Heart failure with oedema seems to be less frequent in children, whereas syncope occurs more frequently in children with idiopathic PAH (IPAH) [3][4][5].…”

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confidence: 99%

Treatment options for paediatric pulmonary arterial hypertension

Berger¹,

Bonnet²

2010

European Respiratory Review

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Pulmonary arterial hypertension (PAH) is a serious, progressive condition, which can present idiopathically or secondary to conditions such as systemic sclerosis or congenital heart disease. The condition exists in both adult and paediatric forms, which possess several similar characteristics. Adult and paediatric PAH can, however, be distinguished based on underlying pathology and the presence of age-specific conditions, some of which are related to poor lung development in children. Improved knowledge of vascular biology has led to the development of several PAH-specific therapies, which have demonstrated clinical benefits in adults, including improved exercise capacity and prolonged survival. Treatment data in paediatric PAH are scarce. Although limited, the existing data indicate that current treatments for paediatric PAH are well tolerated and effective, at least in the short-and medium-term. Nevertheless, the current guidelines for clinicians, which recommend use of the adult treatment algorithm in paediatric patients, appear justified when judged according to the available evidence. However, further randomised, controlled trials are necessary to increase the evidence base for treatment of paediatric PAH, especially in relation to age-specific conditions. At present, early initiation of treatment and combination pharmacological therapy may offer the most promising courses of action to improve outcomes in paediatric PAH.

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“…46 A unique national patient cohort of childhood pulmonary hypertension was reported from the UK. 47 The authors showed, for the first time, that the incidence of pulmonary hypertension is lower in children than in adults and that the clinical features can be different. Most children present with clinical evidence of advanced disease, and clinical status at presentation is predictive of outcome.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%