Abstract:
:Pulmonary arterial hypertension (PAH) in the pediatric population is associated with a variety of underlying diseases and causes, significantly morbidity and mortality. In the majority of patients, PAH in children is idiopathic or associated with congenital heart disease (CHD), with pulmonary hypertension (PH) associated with connective tissue disease, a rare cause in children. Classification of pediatric PH has generally followed the WHO classification, but recognition of the importance of fetal origins of PH and developmental abnormalities have led to the formation of a new pediatric-specific classification. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic PAH and 2.2 and 15.6 for associated pulmonary arterial hypertension-CHD cases per million children. Although the treatment with new selective pulmonary vasodilators offers hemodynamic and functional improvement in pediatric populations, the treatments in children largely depend on results from evidence-based adult studies and experience of clinicians treating children. A recent randomized clinical trial of sildenafil and its long-term extension has led to disparate recommendations in the United States and Europe.
journal_name
Semin Respir Crit Care Medjournal_title
Seminars in respiratory and critical care medicineauthors
Takatsuki S,Ivy DDdoi
10.1055/s-0033-1356461subject
Has Abstractpub_date
2013-10-01 00:00:00pages
627-44issue
5eissn
1069-3424issn
1098-9048journal_volume
34pub_type
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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pub_type: 杂志文章,评审
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更新日期:2005-04-01 00:00:00
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pub_type: 杂志文章
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journal_title:Seminars in respiratory and critical care medicine
pub_type: 杂志文章,评审
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更新日期:2016-04-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2019-02-01 00:00:00
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pub_type: 杂志文章,评审
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pub_type: 杂志文章,评审
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更新日期:2018-02-01 00:00:00
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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pub_type: 杂志文章
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
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journal_title:Seminars in respiratory and critical care medicine
pub_type: 杂志文章
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pub_type: 杂志文章,评审
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更新日期:2012-08-01 00:00:00
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journal_title:Seminars in respiratory and critical care medicine
pub_type: 杂志文章,评审
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更新日期:2014-04-01 00:00:00
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journal_title:Seminars in respiratory and critical care medicine
pub_type: 杂志文章,评审
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更新日期:2015-02-01 00:00:00
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journal_title:Seminars in respiratory and critical care medicine
pub_type: 杂志文章,评审
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