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Childhood idiopathic pulmonary arterial hypertension: a national cohort study

Moledina, S ; Hislop, A A ; Foster, H ; Schulze-Neick, I ; Haworth, S G

Heart (British Cardiac Society), 2010-09, Vol.96 (17), p.1401-1406 [Periódico revisado por pares]

London: BMJ Publishing Group Ltd and British Cardiovascular Society

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Título:
Childhood idiopathic pulmonary arterial hypertension: a national cohort study
Autor: Moledina, S ; Hislop, A A ; Foster, H ; Schulze-Neick, I ; Haworth, S G
Assuntos: Adolescent ; Age Factors ; Antihypertensive Agents - therapeutic use ; Biological and medical sciences ; Cardiology. Vascular system ; Child ; Child, Preschool ; Cohort analysis ; Drug Therapy, Combination ; Epidemiologic Methods ; epidemiology ; Exercise Test - methods ; Female ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Pulmonary - diagnosis ; Hypertension, Pulmonary - drug therapy ; Hypertension, Pulmonary - epidemiology ; Hypertension, Pulmonary - physiopathology ; Male ; Medical prognosis ; Medical sciences ; paediatric cardiology ; paediatrics ; Pneumology ; pulmonary ; pulmonary arterial hypertension (PAH) ; Pulmonary hypertension ; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases ; Sex Factors ; Standard scores ; Statistical analysis ; survival ; Treatment Outcome ; United Kingdom - epidemiology
É parte de: Heart (British Cardiac Society), 2010-09, Vol.96 (17), p.1401-1406
Notas: 195248.
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Descrição: ObjectiveTo clarify the clinical characteristics and epidemiology of idiopathic pulmonary arterial hypertension (IPAH) in childhood, a rare condition with a bad prognosis, poorly documented in children. Also, to describe the long-term outcome.DesignA retrospective study of 7 years' experience.SettingUK Service for Pulmonary Hypertension in Children based at a tertiary referral centre.Patients64 children.InterventionsPatients were initially treated with prostanoids (n=15), bosentan (n=23), sildenafil (n=9), combination therapy (n=11) or calcium channel antagonists (n=6).Main outcome measuresWHO functional class, distance walked in 6 minutes, escalation of therapy, survival, transplant-free survival.ResultsIncidence of IPAH was 0.48 cases per million children per year and the prevalence was 2.1 cases per million. 31% presented with syncope. Oedema was rare. During the first year of follow-up WHO functional class and 6-minute walk distance improved significantly. Survival at 1, 3 and 5 years was 89%, 84% and 75%, respectively; while transplant-free survival was 89% 76% and 57%, respectively. Factors predicting worse survival were WHO functional class (HR 2.4, p=0.04) and poor height and weight z-score (p<0.05 for both) at presentation.ConclusionsWe showed, for the first time, that the incidence of IPAH is lower in children than 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. This 7-year experience confirms the significant improvement in survival over historical controls.
Editor: London: BMJ Publishing Group Ltd and British Cardiovascular Society
Idioma: Inglês

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Childhood idiopathic pulmonary arterial hypertension: a national cohort study

Moledina, S ; Hislop, A A ; Foster, H ; Schulze-Neick, I ; Haworth, S G

London: BMJ Publishing Group Ltd and British Cardiovascular Society

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