Pulmonary hypertension (PH) is a disorder in which blood vessels in the lungs become narrow, impeding blood flow. As a result, blood pressure in the lungs rises above normal. This forces the right side of the heart to work harder than it should to pump blood out through the lungs.
Pulmonary hypertension often worsens over time. Without treatment, it can advance to the point where the right side of the heart fails. All children with PH should undergo a thorough evaluation from a physician and team with expertise in PH. The evaluation should include a search for the underlying cause of PH to determine the best treatment options.
Treatments can ease symptoms and slow the disease's progression, but there is currently no cure for PH. However, a great deal of research is under way, and the long-term prognosis is considerably better than it was even five years ago.
Causes of Pulmonary Hypertension
A number of conditions can cause or contribute to pulmonary hypertension. They include:
Sometimes we can't find a cause. This is called idiopathic pulmonary hypertension.
Symptoms of pulmonary hypertension are often minor at first but can gradually worsen over time. Other conditions can cause similar symptoms, which makes early detection challenging. If your child experiences any sign of pulmonary hypertension, consult his or her doctor right away. The most common are:
The first steps to diagnosing PH are to collect a medical history and perform a complete physical exam. Additional testing may be required to help determine whether your child has PH and what may be causing it, including:
The first step in treatment is usually to identify the underlying cause of PH and try to correct it. Other treatment options may include supplemental oxygen and medications that are taken orally, inhaled or delivered by continuous infusion under the skin or into a vein. The medications we currently use include:
Reviewed by health care specialists at UCSF Benioff Children's Hospital.
Pulmonary Hypertension Program
1825 Fourth St., Sixth Floor
San Francisco, CA 94158
Phone: (415) 476-3679
Fax: (415) 476-5363
Appointment information