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Published on: May 11, 2015
Pulmonary arterial hypertension in children: a medical update
Erika B Rosenzweig1, Robyn J Barst
1Columbia University College of Physicians & Surgeons, New York, NY 10032, USA. esb14@columbia.edu
Insights
Novel therapies are improving outcomes for pediatric pulmonary arterial hypertension (PAH). However, managing this serious condition remains challenging, with new patient groups emerging for targeted treatments.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Pharmacology
Background:
- Pulmonary arterial hypertension (PAH) in children presents unique management challenges.
- Rapid advancements in PAH understanding and treatment necessitate updated pediatric literature reviews.
Purpose of the Study:
- To review recent literature (past year) on available and emerging novel therapies for pediatric pulmonary arterial hypertension (PAH).
- To outline an approach for targeting specific pediatric populations with PAH.
- To provide insight into current management strategies for pediatric PAH patients.
Main Methods:
- Comprehensive literature review focusing on the past year's publications.
- Analysis of novel therapeutic agents and their application in pediatric PAH.
- Identification of emerging target patient groups for PAH therapies.
Main Results:
- Therapeutic advances have improved prognosis for children with PAH.
- Pediatric PAH remains a complex condition requiring challenging management.
- New patient groups, including those with sickle cell disease, congenital diaphragmatic hernia, and Eisenmenger syndrome, are candidates for novel PAH treatments.
Conclusions:
- Data from pediatric PAH studies are often limited to small, uncontrolled case reports.
- Caution is advised when interpreting findings from small, uncontrolled pediatric studies.
- While novel agents show promise for pediatric PAH, further controlled studies are needed to confirm their impact across diverse subgroups.
Purpose Of Review:
With rapid advances in the understanding and treatment of pulmonary arterial hypertension, navigating the pediatric literature becomes challenging. A comprehensive review of the most recent literature over the past year on available and emerging novel therapies as well as an approach to target pediatric populations will provide insight into the current management of pediatric pulmonary hypertension patients.
Recent Findings:
Recent therapeutic advances have significantly improved the prognosis for children with pulmonary arterial hypertension. Pediatric pulmonary arterial hypertension continues to be a serious condition, however, which is extremely challenging to manage. There are also new target groups, such as those with sickle cell disease, congenital diaphragmatic hernia and Eisenmenger syndrome who may be candidates for treatments previously used for idiopathic pulmonary arterial hypertension patients.
Summary:
The data in children are often limited to case reports as many of those described here. Thus, the reader needs to be cautious about the interpretation of such small uncontrolled studies. While many of these data support the rationale for using novel agents for children with pulmonary arterial hypertension, further controlled and well designed studies are necessary to assess the true impact of these agents on various subgroups of children with pulmonary arterial hypertension.
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