Pulmonary Hypertension: First Guidelines Issued for Children

Marcia Frellick

November 12, 2015

The first guidelines have been issued for diagnosing and treating children with pulmonary hypertension (PH), a sometimes-fatal heart and lung disease that affects 1.9 of every 1000 babies born each year.

The joint American Heart Association/American Thoracic Society guidelines were published online November 3 in Circulation.

"These children suffer with health issues throughout their lives or die prematurely — particularly if they're not properly diagnosed and managed," Stephen L. Archer, MD, the head of medicine at Queens University in Ontario, Canada, and cochair of the guidelines committee, said in an American Heart Association news release. "But with the proper diagnosis and treatment at a specialized center for [PH], the prognosis for many of these children is excellent."

There have been guidelines for diagnosing and treating PH in adults for many years, but they are not directly applicable because causes of the disease in children differ substantially.

The new guidelines include recommendations in the following categories:

Diagnostics, Assessments, and Monitoring

Cardiac catheterization should include acute vasoreactivity testing unless there is a specific contraindication. Also, the 6-minute walk distance test should be used to follow exercise tolerance in patients with pediatric PH who are of appropriate age.

A sleep study should be part of the diagnostic evaluation of patients with PH at risk for sleep-disordered breathing, and a sleep study is indicated for evaluating patients with poor responsiveness to pulmonary arterial hypertension (PAH)-targeted therapies.

Outpatient Care of Children With PH

Children with PH should be evaluated and treated in comprehensive, multidisciplinary clinics at specialized pediatric centers. Outpatient follow-ups at 3- to 6-month intervals are reasonable, more frequently for patients with advanced disease or after initiation of changes.

Because of significant maternal and fetal mortality associated with pregnancy in patients with PH, the guidelines recommend that female adolescents with PH get age-appropriate counseling about pregnancy risks and contraception.

Idiopathic PAH

Referral to lung transplantation centers for evaluation is recommended for patients who are in World Health Organization functional class III or IV on optimized medical therapy or who have rapidly progressive disease.


Genetic testing with counseling can be useful for children with idiopathic PAH or in families with heritable PAH to define the pathogenesis, to determine family members at risk, and for family planning. Members of families with heritable PAH who develop new cardiorespiratory symptoms should be evaluated immediately for PAH.

Lung Diseases

Children with chronic diffuse lung disease should be evaluated for concomitant cardiovascular disease or PH by echocardiogram, especially those with advanced disease. Echocardiography is recommended to assess PH and right ventricular function in patients with severe obstructive sleep apnea.

Congenital Diaphragmatic Hernia

Longitudinal care in an interdisciplinary pediatric PH program is recommended for infants with congenital diaphragmatic hernia who have PH or are at risk of developing late PH. Extracorporeal membrane oxygenation is recommended for patients with congenital diaphragmatic hernia with severe PH who do not respond to medical therapy.

Persistent PH of the Newborn

Inhaled nitric oxide should be used to reduce the need for extracorporeal membrane oxygenation in term and near-term infants with persistent PH of the newborn or hypoxemic respiratory failure who have an oxygenation index that exceeds 25.

Bronchopulmonary Dysplasia

Screening for PH by echocardiogram is recommended in infants with bronchopulmonary dysplasia. Evaluation and treatment of lung disease, including assessments for hypoxemia, aspiration, structural airway disease, and the need for changes in respiratory support, are recommended in infants with bronchopulmonary dysplasia and PH before initiation of PAH-targeted therapy.


Anticoagulation should not be used in young children with PAH because of concerns about harm from hemorrhagic complications.

Hypobaric Hypoxia

Therapy for symptomatic high-altitude pulmonary edema should include oxygen therapy and consideration of immediate descent. Children with high-altitude pulmonary edema should be evaluated to rule out abnormalities of pulmonary arteries or pulmonary veins, lung disease, or abnormal breathing.

The guideline authors acknowledge that gaps remain in the understanding of PH in children and conclude: "This document is intended to provide a foundation for future work directed toward additional discoveries of basic and clinical aspects of disease that will ultimately improve the quality of life and long-term outcomes of children with [pulmonary vascular disease] in diverse settings."

Committee members report association with and/or fees or support from the National Institutes of Health, Canada Institutes of Health Research, CFI, Cardiovascular Medical Research and Education Fund, Canadian Medical Protective Association, Eli Lilly, Actelion Pharmaceuticals, Deloitte, Gilead, Ikaria, Stem Cell Network, Ontario Thoracic Society, Corridor Pharmaceuticals, Arena, Merck, Novartis, Pfizer, BioReference Laboratories, United Therapeutics, and Bayer.

Circulation. Published online November 3, 2015. Full text


Comments on Medscape are moderated and should be professional in tone and on topic. You must declare any conflicts of interest related to your comments and responses. Please see our Commenting Guide for further information. We reserve the right to remove posts at our sole discretion.