期刊
CURRENT OPINION IN CARDIOLOGY
卷 27, 期 2, 页码 70-81出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/HCO.0b013e32835018cd
关键词
children; endothelin receptor antagonists; phosphodiesterase-5; prostacyclin; pulmonary hypertension
资金
- National Institutes of Health (NIH) Specialized Centers of Clinically Oriented Research [HL-HL684923]
- NIH/NCRR Colorado CTSI [UL1 RR025780]
- Jayden DeLuca Foundation
- Leah Bult Pulmonary Hypertension Fund
Purpose of review Pulmonary arterial hypertension (PAH) is an important cause of morbidity and mortality in children. Approved medications for the treatment of adult PAH have been used to treat children, but evidence-based treatment algorithms for children are lacking. Recent findings Pediatric PAH registries have begun to define the incidence and prevalence of idiopathic PAH and PAH associated with congenital heart disease. A pediatric-specific classification of pulmonary hypertensive vascular disease has been proposed. Furthermore, the first randomized placebo-controlled trial of type-5 phosphodiesterase therapy in treatment-naive children with PAH has been completed and reported. This trial highlights the importance of the difficulties of performing clinical trials in children with targeted PAH therapy as well as the importance of long-term follow-up of adverse events. Summary Classification, clinical trials, and therapy for children with PAH must take into account the unique aspects of PAH in children.
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