AHA releases first-ever pediatric pulmonary hypertension guideline

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A new roadmap to pediatric PH diagnosis and care

The pediatric pulmonary, pediatric cardiology, and neonatal and pediatric intensivists all have greatly anticipated directions for the care of pediatric pulmonary hypertension. The guidelines have excellent care maps for the diagnosis and evaluation of the various etiologies of pulmonary hypertension.

The new guidelines also should help also with insurance authorizations for the expensive medications for pulmonary hypertension! Dr. Robyn J. Barst, a renowned leader in pediatric pulmonary hypertension, who passed away in 2013, would be so proud of this AHA guideline!

Dr. Susan L. Millard is director of research, pediatric pulmonary & sleep medicine at Helen DeVos Children’s Hospital in Grand Rapids, MI.




The American Heart Association and the American Thoracic Society jointly released the first-ever clinical practice guideline for assessing and managing pulmonary hypertension (PH) in the pediatric population, which was published online Nov. 3 in Circulation.

The two organizations developed this guideline because the causes and treatments of PH in neonates, infants, and children are often different from those in adults. The literature for adult PH is “robust,” and there are several treatment guidelines available, whereas pediatric PH has not been well studied, “and little is understood about the natural history, fundamental mechanisms, and treatment of childhood PH,” said Dr. Steven H. Abman, cochair of the guideline committee and a pediatric pulmonologist at the University of Colorado and Children’s Hospital, both in Denver.

“It’s important to note that, although these guidelines provide a foundation for taking care of children with pulmonary hypertension, we still have a huge need for more specific data and research to further improve outcomes,” he said in a statement accompanying the guideline.

This guideline was developed by a working group of 27 clinicians and researchers with expertise in pediatric pulmonology, pediatric and adult cardiology, pediatric intensivism, neonatology, and translational science. They reviewed more than 600 articles in the literature, but given the paucity of high-quality data regarding pediatric PH, the guideline relies heavily on expert opinion and primarily describes “generally acceptable approaches” to diagnosis and management; more specific and detailed recommendations await the findings of future research, Dr. Abman and his associates said (Circulation. 2015 Oct 26. doi:10.1161/CIR.0000000000000329).

In the pediatric population, PH is defined as a resting mean pulmonary artery pressure greater than 25 mm Hg after the first few months of life and is usually related to cardiac, lung, or systemic diseases. Idiopathic PH, a pulmonary vasculopathy, is a diagnosis of exclusion after diseases of the left side of the heart, lung parenchyma, heart valves, thromboembolism, and other miscellaneous causes have been ruled out.

The guideline emphasizes that children thought to have PH should be evaluated and treated at comprehensive, multidisciplinary clinics at specialized pediatric centers. “When children are diagnosed, parents often feel helpless. However, it’s important that parents seek doctors and centers that see these children on a regular basis and can offer them access to new molecular diagnostics, new drug therapies, and new devices, as well as surgeries that have recently been developed,” Dr. Stephen L. Archer, cochair of the guideline committee and head of medicine at Queen’s University, Kingston, Ont., said in the statement.

“These children suffer with health issues throughout their lives or die prematurely, particularly if they’re not properly diagnosed and managed. But with the proper diagnosis and treatment at a specialized center for PH, the prognosis for many of these children is excellent,” he noted.

Properly classifying the type of PH is a key first step in determining treatment. The guideline addresses numerous methods for diagnosing and monitoring PH, including imaging studies, echocardiograms, cardiac catheterization, brain natriuretic peptide and other laboratory testing, 6-minute walk distance (at appropriate ages), sleep studies, and genetic testing. It specifically deals with persistent PH of the newborn and PH arising from congenital diaphragmatic hernia; bronchopulmonary dysplasia or other lung diseases; heart disease such as atrial-septal defect or patent ductus arteriosus; and systemic diseases such as hemolytic hemoglobinopathies and hepatic, renal, or metabolic illness; as well as idiopathic PH and PH related to high-altitude pulmonary edema.

Regarding ongoing outpatient care, the guideline recommends that children with PH receive influenza and pneumococcal vaccinations and prophylaxis for respiratory syncytial virus (if they are eligible), as well as antibiotic prophylaxis to prevent subacute bacterial endocarditis in those who are cyanotic or have indwelling central lines. Growth must be monitored rigorously, and infections and respiratory illnesses must be recognized and treated promptly. Any surgeries require careful preoperative planning and should be performed at hospitals with expertise in PH.

The guideline includes an extensive section on pharmacotherapy for childhood PH, including the use of digitalis, diuretics, long-term anticoagulation, oxygen therapy, calcium channel blockers, phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, intravenous and subcutaneous prostacyclin therapy, and the transition from parenteral to oral or inhaled treatment.

In addition, the guideline addresses exercise and sports participation, travel restrictions, and contraceptive counseling for adolescent patients. Finally, “given the impact of childhood PH on the entire family, [patients], siblings, and caregivers should be assessed for psychosocial stress and be readily provided support and referral as needed,” the guideline recommends.

A copy of the guideline is available at

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