Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and children. The disease affects nearly two of every 1,000 babies born each year in the United States, and children can suffer with health issues throughout their lives or die prematurely. However, with a proper diagnosis, rigorous evaluation, and comprehensive treatment, the prognosis for many of these children can be excellent, depending on the underlying cause of the pulmonary hypertension.

The American Heart Association and American Thoracic Society convened a panel of experts to review the current literature and make recommendations on diagnosing, evaluating, and treating pediatric pulmonary hypertension. Published in Circulation and available for free online at http://circ.ahajournals.org, the guidelines are the result of extensive literature reviews of over 600 studies, extensive discussions, and formal scoring of recommendations.

“These first-ever guidelines were rigorously developed in collaboration with a group of diverse experts that cross the traditional silos of several disciplines,” says Steven H. Abman, MD, the guidelines committee co-chair. “To optimize the care of pediatric patients with pulmonary hypertension, increased awareness of at-risk patients and the use of multidisciplinary care teams are critical.”


Key Highlights

According to Dr. Abman, the guidelines provide clinicians with practical advice on classifying the many types of pulmonary hypertension, a key step in determining treatment. The document also discusses proven and emerging medical and surgical therapies as well as treatments that are approved for children with pulmonary hypertension and which drugs and dosages should be used. In addition, guidance is provided for long-term monitoring.


Specialized Care

“It’s important to note that the causes, clinical presentations, and treatment of pulmonary hypertension in children is often different from that of adults,” Dr. Abman says. “These recommendations can guide clinicians on treatment decisions that are specific to this patient group.” They also describe strategies for optimizing comprehensive care by offering advice on supportive care, social aspects of care, and determining when children with pulmonary hypertension can safely engage in exercise or travel on an airplane.

According to the guidelines, directing patients to receive care at pulmonary hypertension centers that specialize in treating the disease is critical. When children are diagnosed with pulmonary hypertension, parents can feel isolated or without strong support in their ability to care for their children. Clinicians should encourage parents to seek physicians and centers that see these children on a regular basis. These institutions are well equipped to offer children with pulmonary hypertension access to new diagnostic methods and emerging drug therapies as well as surgical interventions that have recently been developed.


More to Come

“While the guidelines provide a foundation for taking care of children with pulmonary hypertension, there is still much to be learned about the disease,” Dr. Abman says. “The guidelines can serve as a rallying cry for improving care for the future. We are still lacking more specific data and research to further improve outcomes of children with pulmonary hypertension. We hope the guidelines will not only help clinicians in their care of these patients, but also spur ongoing investigations to address research gaps in the literature.”