Practice Guidelines

AHA/ATS Release Guidelines on the Diagnosis and Treatment of Pediatric Pulmonary Hypertension

 


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Am Fam Physician. 2016 Apr 1;93(7):605-614.

Key Points for Practice

• A comprehensive history and physical examination, combined with diagnostic testing and formal assessment of cardiac function, should be done at the time of initial pulmonary hypertension diagnosis.

• Echocardiography is the preferred tool for screening and serial testing, and should be performed to assess clinical status and changes in therapy.

• Conventional therapies used for heart failure are also used to treat right ventricular failure.

• Patients with severe pulmonary hypertension should be advised not to participate in competitive sports.

From the AFP Editors

Pulmonary hypertension in children is distinct from adult hypertension. It is linked to issues of lung growth and development, and is often related to impaired functional and structural adaptation of pulmonary circulation during transition from fetal to postnatal life. Pulmonary hypertension in children is defined as a resting mean pulmonary artery pressure of more than 25 mm Hg beyond the first few months of life. These guidelines from the American Heart Association (AHA) and the American Thoracic Society (ATS) address evaluation and treatment of pediatric pulmonary hypertension, including diagnosis, pharmacotherapy, and outpatient treatment recommendations.

Diagnosis

A comprehensive history and physical examination, combined with diagnostic testing and formal assessment of cardiac function, should be done at the time of initial pulmonary hypertension diagnosis. Critical diagnostic testing includes chest radiography, electrocardiography, echocardiography, chest computed tomography (CT) with or without contrast media, six-minute walk test, laboratory studies including brain natriuretic peptide levels, and cardiac catheterization. Targeted evaluation with pulmonary function testing, magnetic resonance imaging, lung perfusion scanning, cardiopulmonary exercise testing, and a sleep study may be appropriate in some patients. Follow-up visits should be performed every three to six months, with more frequent visits for patients with advanced disease or after initiation of or change in therapy.

ECHOCARDIOGRAPHY

Echocardiography is noninvasive and useful in identifying potential causes of pulmonary hypertension, right ventricular function, and assessing related comorbidities. It is the preferred tool for screening and should typically be performed every four to six months to monitor disease progression, or if there is a change in therapy.

CARDIAC CATHETERIZATION

Cardiac catheterization should be performed before starting therapy

Coverage of guidelines from other organizations does not imply endorsement by AFP or the AAFP.

This series is coordinated by Sumi Sexton, MD, Associate Deputy Editor.

A collection of Practice Guidelines published in AFP is available at http://www.aafp.org/afp/practguide.



 

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