The Pediatric Pulmonary Hypertension Network
Advancing Research • Enhancing Care • Advocacy • Training and Education
PH Guidelines for Return To School: Fall 2020
PPHNet News: Publications
American Heart Association and American Thoracic Society Issue New Pediatric Pulmonary Hypertension Guidelines
Welcome to PPHNet Online
Steve Abman, MD
The Pediatric Heart Lung Center, Department of Pediatrics, University of Colorado School of Medicine
Welcome to PPHNet Online, the web home of the Pediatric Pulmonary Hypertension Network. This web site is part of our organization’s effort to improve outcomes for patients with PPH. Here you will find information about PPH and related disorders, a list of clinical centers with contact information, and reports about current and emerging research topics, as well as other features. We are glad you have come to the site and hope the information you find here is of use.
The web hosting for PPHNet is generously supported by the Jayden DeLuca Foundation.
Welcome to PPHNet, a network of clinical specialists, researchers, and centers bringing a collaborative and multidisciplinary approach to improving care for children with pulmonary vascular disease.
- To improve the health, quality of care and outcomes of children with pulmonary hypertension by enhancing collaborations among centers, in order to develop and apply novel strategies for early recognition and initiation of treatment to reverse, prevent or slow the progression of disease.
- To promote and conduct collaborative activities in research, education and training that leads to the development of effective therapies and their successful application.
This site provides information for PPHNet members, other medical professionals and the general public on PPH. See the For Patients and Families page for more information about Pediatric Pulmonary Hypertension.
About the Organization
PPHNet, the Pediatric Pulmonary Hypertension Network, is an association of medical professionals and centers focused on all aspects of PPH. Providers at these centers offer comprehensive clinical services across a wide range of medical disciplines and initiate and participate in research. For more information about each Center, and contact information, please see the Centers page
About Pediatric Pulmonary Hypertension
Pulmonary Hypertension (PAH) is a rare and serious disease that affects people of all ages, from newborns to the elderly. Its key characteristic is abnormally high blood pressure in arteries that connect the heart to the lungs and take up oxygen. The causes of PAH are variable, depending on the age at onset. Like adult pulmonary hypertension, childhood, or pediatric pulmonary hypertension may have a range of causes, or no cause may be evident, in which case it is called idiopathic.
What happens in PAH? In PAH, the pulmonary arterioles (the tiny branches of the PAs) narrow down. This may be because of the thickening and ultimately the closing down of their walls. It may also occur as a temporary spasm, in response to a stimulus.
Symptoms: Shortness of breath, fainting, fatigue, turning blue, not growing appropriately.
Causes: In infants, PAH can present in the neonatal period with persistent pulmonary hypertension of newborn (PPHN), or in association with congenital diaphragmatic hernia (CDH), congenital heart disease and/or genetic disorders. Infants who are born extremely preterm may develop lung disease (bronchopulmonary dysplasia), and a subset of these may also develop pulmonary hypertension. In older children, causes may include those listed for infants above, as well as idiopathic/heritable PAH, connective tissue diseases, hematologic diseases and clots in the lungs (thromboembolic PAH).
Diagnosis: Diagnosis is usually made via an echocardiogram, and confirmed by cardiac catheterization, lab testing, lung function testing, and other imaging.
Treatment: Several classes of medications are used for treatment of PAH.
Related disorders: Congenital heart disease, scleroderma, lupus, sickle cell and other hematologic diseases, as well as genetic disorders can be related to PAH. For more information, please see the “For Patients and Families” page.
Families and Caregivers: Please see the “Centers” page for information about clinical care in your area. Please note that currently, PPHNet does not provide specialist referrals. Information about clinical trials can be found on the “Registry” page of this website.