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Published on: May 11, 2015
Pulmonary arterial hypertension in children: a medical update
Erika B Rosenzweig1, Robyn J Barst
1Department of Pediatrics, Columbia University College of Physician & Surgeons, New York, NY 10032, USA. esb14@columbia.edu
Insights
Pediatric pulmonary arterial hypertension (PAH) management is evolving with novel therapies offering improved survival. This review navigates recent advancements and target populations, including sickle cell disease patients, for better pediatric PAH care.
Area of Science:
- Cardiology
- Pediatric Pulmonology
- Vascular Biology
Background:
- Pulmonary arterial hypertension (PAH) is a severe condition leading to right heart failure.
- Historically, idiopathic pulmonary arterial hypertension (IPAH) had a poor prognosis in children, with median survival of 10 months.
- Continuous intravenous epoprostenol, approved in 1995, significantly improved outcomes for severe PAH.
Purpose of the Study:
- To review recent literature (past year) on novel therapies for pediatric PAH.
- To provide insights into managing pediatric PAH, including emerging treatments.
- To identify and discuss target pediatric populations, such as those with sickle cell disease.
Main Methods:
- Comprehensive literature review of studies published within the last year.
- Analysis of available and emerging novel therapeutic strategies for pediatric PAH.
- Examination of current approaches to managing pediatric PAH patients.
Main Results:
- Significant advances in PAH treatment have occurred, though a cure remains elusive.
- Pediatric patients have benefited from novel PAH therapies, expanding treatment options.
- Pulmonary hypertension is increasingly recognized as a risk factor in pediatric sickle cell disease.
Conclusions:
- Navigating the rapidly advancing field of pediatric PAH requires updated knowledge of novel therapies.
- Effective management strategies are crucial for improving outcomes in children with PAH.
- Further research and clinical application of new treatments are essential for pediatric PAH patients.
Abstract:
Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by a progressive pulmonary vasculopathy with ensuing right heart failure if left untreated. In the 1980's, prior to the current treatment era, idiopathic pulmonary arterial hypertension (IPAH) carried a poor prognosis with a 10 month median survival for children after diagnosis. However, in 1995 continuous intravenous epoprostenol was approved for the treatment of severe PAH, improving hemodynamics, quality of life, exercise capacity, functional class and survival. In the past decade there have been further advances in the treatment of PAH; however, there is still no cure. While much of the groundbreaking clinical research has been performed in adults, children have also seen the benefits of PAH novel therapies. The target population among pediatric patients is expanding with the recent recognition of pulmonary hypertension as a risk factor for sickle cell disease patients. With rapid advances, navigating the 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 provides insights into the management of pediatric PAH patients.
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