Every year, nearly 1 in 500 babies are born with pulmonary hypertension, a potentially fatal heart and lung disease. Now, the first ever guidelines for diagnosis and treatment have been published in the journal Circulation.
The new guidelines are the result of collaboration between the American Heart Association (AHA) and American Thoracic Society (ATS) and were created by an interdisciplinary team of 27 physicians, based on data from more than 600 studies.
Existing guidelines for diagnosis and treatment of pulmonary hypertension in adults are not considered applicable to children because of the different underlying causes of the disease.
Children with pulmonary hypertension typically have blockages in their pulmonary arteries, making it harder for the heart’s right ventricle to pump blood through the lungs.
They may experience shortness of breath and fainting, and may appear blue due to lack of oxygen.
While pulmonary hypertension can be an isolated condition caused by circulatory problems in the lungs, it often occurs as a complication of other serious heart or lung health issues. These include congenital heart disease, bronchopulmonary dysplasia, which is a form of chronic lung disease, and congenital diaphragmatic hernia.
Children with diaphragmatic hernias are born with a hole in the diaphragm that causes the abdominal organs to shift in the chest, preventing the lungs from developing properly.
Untreated, pulmonary hypertension can lead to disability or death from heart failure.
Guidelines aim to raise level of care
The new guidelines aim to provide a foundation for the best possible care in cases of pediatric pulmonary hypertension.
They offer practical advice about:
Classifying the types of pulmonary hypertension
Proven and emerging medical and surgical therapies
Appropriate and approved treatments and dosages
Optimal comprehensive care of the patient, including advice on supportive care, social aspects of care and practical advice on exercise and travel restrictions
Advice on the role of pulmonary hypertension centers offering specialized treatment.
The guidelines also highlight areas where more research is needed and offer advice for parents and health care providers about difficult issues.
uch issues include the use of blood thinners, how to determine whether a child with pulmonary hypertension can safely engage in exercise or travel on a plane, and how high altitude can cause or worsen pulmonary hypertension.
Dr. Stephen Archer, head of medicine at Queens University in Canada and co-chair of the guideline’s writing committee, says that children with pulmonary hypertension may experience lifelong health issues or die prematurely, especially those who are not correctly diagnosed and managed.
However, with the proper diagnosis and treatment at a specialized center, he says the outlook for many of these children is excellent.
“When children are diagnosed with pulmonary hypertension, parents often feel hopeless. However, it is 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. Steven Abman is the guidelines committee co-chair, a pediatric pulmonologist, and professor of pediatrics at the University of Colorado School of Medicine and Children’s Hospital in Denver. He notes that while the new guidelines provide a foundation for taking care of children with pulmonary hypertension, there remains a huge need for more specific data and research to further improve outcomes for children with this condition.