“These children suffer with health issues throughout their lives or die prematurely — particularly if they’re not properly diagnosed and managed," Dr. Stephen L. Archer, the head of medicine at Queens University, Kingston, Ontario, Canada and co-chair of the guideline writing committee, said. "But with the proper diagnosis and treatment at a specialized center for pulmonary hypertension, the prognosis for many of these children is excellent."
About two of every 1,000 babies born in the United States each year suffers from pulmonary hypertension, which is a potentially deadly heart and lung disease. The condition is usually marked by blockages in the blood vessels of the lungs, which leads to shortness of breath, fainting and cyanosis (blue skin).
Typically, the condition is secondary to congenital heart disease, chronic lung disease or congenital diaphragmatic hernia. It can lead to disability or death from heart failure.
The guidelines include information about how to classify the many kinds of pulmonary hypertension; details on current and in-progress medical and surgical treatments; treatments that are approved for children and suggested dosages; suggestions for comprehensive care; and tips for evaluating pulmonary hypertension centers
that specialize in treating the disease.
"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 of children with pulmonary hypertension,” Dr. Steven H. Abman, the guidelines committee co-chair who is also a pediatric pulmonologist, said.