Document Detail


Assessing Pulmonary Hypertensive Vascular Disease in Childhood: Data from the Spanish Registry.
MedLine Citation:
PMID:  25379685     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Rationale: There is a lack of knowledge regarding the epidemiology, clinical characterization, and survival in pediatric pulmonary hypertension. Methods: We analyzed data from the Spanish Registry for Pediatric Pulmonary Hypertension. From January 2009 to June 2012, 225 patients diagnosed of pulmonary hypertension in 1998 or after were collected from 21 referral and non-referral centers. We included all Nice etiologies, estimated incidence and prevalence of pulmonary hypertension in the Spanish pediatric population and analyzed risk factors for mortality (Nice etiologic group,clinical and hemodynamic variables). Patients were classified as: group I (pulmonary arterial hypertension n=142, 61%), group II (left heart disease n= 31, 14%), group III (respiratory disease n=41, 18%), group IV (thromboembolic pulmonary hypertension n=2, 1%), or group V (n=10, 4.5%, mostly inherited metabolic diseases). Of the patients studied, 31% had multifactorial pulmonary hypertension. Results: Median age at diagnosis was 4.3±4.9 years (50% <2 y.o).,Survival rates at 1- and 3-years were 80% and 74% for the whole cohort, and 89%, and 85% for patients with pulmonary arterial hypertension. Independent risk factors for mortality included an etiologic group other than pulmonary arterial hypertension (p<0.001), age at diagnosis younger than 2 years old (p<0.001), advanced functional class at diagnosis (p<0.001) and high right atrial pressure at diagnosis (p=0.002). Conclusions: In moderate/severe pediatric pulmonary hypertension, the prognosis is better in pulmonary arterial hypertension than in other Nice categories. In pediatric pulmonary hypertension age at diagnosis younger than 2 years is a risk factor for mortality, in addition to the previously established risk factors.
Authors:
M J Cerro Marín; A Sabaté Rotés; A Rodriguez Ogando; A Mendoza Soto; M Quero Jiménez; J L Gavilán Camacho; I Raposo Sonnenfeld; A Moya Bonora; D C Albert Brotons; A Moreno Galdó;
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-11-7
Journal Detail:
Title:  American journal of respiratory and critical care medicine     Volume:  -     ISSN:  1535-4970     ISO Abbreviation:  Am. J. Respir. Crit. Care Med.     Publication Date:  2014 Nov 
Date Detail:
Created Date:  2014-11-7     Completed Date:  -     Revised Date:  2014-11-8    
Medline Journal Info:
Nlm Unique ID:  9421642     Medline TA:  Am J Respir Crit Care Med     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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