By: Caitlin Haxel, MD, FAAP & Julie Glickstein, MD, FAAP
Pulmonary hypertension is high blood pressure in the arteries of the lungs. It is a rare, progressive disease affecting people of all ages.
How pulmonary hypertension affects infants and children:
Children are either born with narrowing in the lung arteries or the arteries become thicker and narrower over time. The narrowing in the arteries causes increased pressure. The right ventricle pumps blood to the lungs to receive oxygen. In pulmonary arterial hypertension, a type of pulmonary hypertension, the right ventricle needs to pump against the increased pressure in the lung arteries. This can lead to heart problems over time, especially with the right ventricle. The walls of the right ventricle can become much thicker than normal, and the function of the right ventricle can decrease over time.
Pulmonary hypertension in newborns:
Persistent pulmonary hypertension of the newborn (PPHN) is the term used when babies are born with the disease. Normal transitions in circulation that should occur after birth do not, and this can lead to symptoms such as fast breathing, difficulty breathing, and/or low oxygen saturation.
Pulmonary hypertension in children:
Pulmonary hypertension can develop in children due to an unknown cause (idiopathic PH) or due to another medical problem including congenital heart or lung disease.
Symptoms of pulmonary hypertension:
Symptoms of pulmonary hypertension depend on the exact cause, and a complete work-up is needed to figure out what that underlying cause is.
In children, there can be a range of symptoms including:
Fatigue with activity
Shortness of breath with activity
Fainting, particularly with activity
Swelling of the legs or ankles
What type of doctor treats children with pulmonary hypertension?
If your pediatrician is concerned about your child's symptoms, you may be referred to a pediatric pulmonary hypertension specialist for an evaluation―usually a pediatric cardiologist or pulmonologist with extensive training in pulmonary hypertension who treats many children with the condition.
Diagnosing pulmonary hypertension:
To confirm the diagnosis, your child's doctors will review the results of a physical exam and various tests taken at the appointment.
Examples of tests include:
Electrocardiogram (tracing of the electrical activity in the heart)
Echocardiogram (ultrasound of the heart)
Six-minute walk test or cardiopulmonary exercise testing (to understand how the body responds to exercise)
Ventilation-perfusion lung scintigraphy (looks at the circulation of blood and air in the lungs)
More advanced imaging:
Chest/cardiac computed tomography (CT) (uses radiation to look at the lungs and blood vessels)
Chest/cardiac magnetic resonance imaging (MRI) (uses magnetic fields to look at the lungs and blood vessels)
Cardiac catheterization (procedure to measure the pressure in the blood vessels in the lungs)
Lung biopsy (invasive procedure to get a small amount of lung tissue for testing)
Treatments for pulmonary hypertension:
Whenever possible, it is important to identify and treat the underlying problem. If your child has a heart valve problem that is causing the pulmonary hypertension, for example, then treatment would first involve repair to the valve.
There are many medications used to decrease the pressure in the lung blood vessels that improve the quality of life for infants and children with pulmonary hypertension. Warfarin (Coumadin), digoxin, and vasodilators, such as nifedipine, intravenous prostacyclin, or both are examples. These medications can be taken by mouth, inhaled into the lungs, or given through an IV into the blood vessels. Everyone responds differently to treatment, so it is important to talk with your child's doctors to determine what is right for your child.
Outlook for children with this disease:
There is no cure for pulmonary hypertension, and some children eventually require lung or heart-lung transplants. However, new treatments are available to help prevent the disease from progressing and new research is being done all the time to hopefully result in more options.
About Dr. Haxel:
Caitlin Haxel, MD, FAAP, is a pediatric cardiology fellow focusing on advanced imaging and fetal cardiology at the Children's Hospital Colorado in Aurora, Colorado. She completed her medical education at State University of New York in Brooklyn, New York followed by her pediatric residency and pediatric cardiology fellowship at New York Presbyterian-Morgan Stanley Children's Hospital at Columbia University Medical Center in New York, NY. Within the American Academy of Pediatrics, Dr. Haxel is a member of the Section on Cardiology and Cardiothoracic Surgery Publications and Communications Committee.
About Dr. Glickstein:
Julie Glickstein, MD, FAAP is a Professor of Pediatrics at the Children's Hospital of New York Presbyterian/Columbia University. She is a member of many professional organizations including the American Heart Association, the American College of Cardiology, the American Society of Echocardiography, the New York Pediatric Cardiology Society of Greater New York, and the Fetal Heart Society. Within the American Academy of Pediatrics, she sits on the Society of Pediatric Cardiology Training Program Directors, is a member of the Section on Cardiology and Cardiothoracic Surgery Publications and Communications Committee, as well as the Congenital Heart Disease Public Health Workgroup.