Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France

Fraisse, Alain; Jaïs, Xavier; Schleich, Jean-Marc; Filippo, Sylvie Di; Maragnès, Pascale; Beghetti, Maurice; Gressin, Virginie; Voisin, M.; Dauphin, Claire; Clerson, Pierre; Godart, François; Bonnet, Damien

doi:10.1016/j.acvd.2009.12.001

Cited by 128 publications

(129 citation statements)

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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%

“…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].…”

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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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Registries for paediatric pulmonary hypertension

Hansmann

Hoeper

2013

Eur Respir J

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“…In children, PAH may be even less common, with an estimated prevalence of PAH (excluding Persistent Pulmonary Hypertension of the Newborn [PPHN] and PAH caused by congenital heart disease [PAH-CHD]) of 3.7 per million children based on a registry in France [8]. However, because PAH-CHD represents a large proportion of cases (up to 40%) [9], this figure is likely to be underestimated.…”

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confidence: 99%

Cumulative Distribution Functions of Sildenafil Citrate on Exercise Capacity and Hemodynamic Function in Children with Pulmonary Arterial Hypertension

Hwang¹

2013

J Pulm Respir Med

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“…1 The prevalence of pulmonary arterial hypertension (PAH) is estimated to be <1 per 100,000 children with an incidence of 1 to 2 new cases/million/year. 2 Prior to the current era, the median survival after diagnosis of IPAH was reported in the National Institutes of Health Primary Pulmonary Hypertension Registry as 2.8 years in adults and only 10 months in children. 3 In the modern era, with early diagnosis and initiation of therapy, survival at 1, 2, and 3 years has been reported to be 99%, 96%, and 84% in children and 88%, 76%, and 63% in adults.…”

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confidence: 99%

“…Advances in treatment have not only increased the life expectancy for these patients, but also improved quality of life. [1][2][3][4][5][6][7][8][9] Although there is yet no cure for PAH, nor a single therapeutic approach that is uniformly successful, therapy has dramatically improved over the past several decades, resulting in sustained clinical and hemodynamic improvement as well as increased survival in children with various types of PAH. [10][11] The recently published American Heart Association/American Thoracic Society guidelines outline the current approach and summarize recommendations for treatment.…”

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confidence: 99%