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Pediatric pulmonary hypertension: diagnosis and management

Dadlani, Gul H.a,b; Sosa, Philb; Cobb, Harrisonb; Akshatha, Akshathab

Current Opinion in Cardiology: January 2016 - Volume 31 - Issue 1 - p 78–87
doi: 10.1097/HCO.0000000000000243
PEDIATRICS: Edited by James C. Huhta

Purpose of review The article is intended to provide an overview of the most current modalities for the diagnosis and management of pulmonary hypertension in the pediatric population.

Recent findings The WHO has recently updated the classification for pulmonary hypertension to include an expanded range of pediatric disease cohorts. We continue to gain knowledge of traditional pulmonary hypertension therapies (phosphodiesterase inhibitors, endothelin-receptor antagonists, and prostanoids) and remain optimistic that new therapeutic modalities [cyclic guanosine monophosphate (cGMP) stimulators, oral prostacyclins, and gene therapy] will lead to improved outcomes in pediatric patients.

Summary Pediatric pulmonary hypertension is a heterogeneous disorder that has the potential to improve in some cases as children grow throughout childhood. Utilization of dedicated multidisciplinary teams of medical providers is necessary to deliver the highest level of medical care to this complex patient population. Ongoing development of enhanced screening protocols, novel disease-specific therapeutic targets, and comprehensive registries will hopefully lead to improved morbidity and mortality in the future.

aJohns Hopkins All Children's Heart Institute, St Petersburg, Florida

bDepartment of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, Florida, USA

Correspondence to Gul H. Dadlani, MD, Johns Hopkins All Children's Heart Institute, 2nd Floor Outpatient Care Center #206, 601 5th Street South, St Petersburg, FL 33701, USA. Tel: +1 727 767 3333; fax: +1 727 767 8990; e-mail:

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