October 4, 2023

High blood pressure in the lungs can affect people of all ages.

The word hypertension is most commonly associated with high blood pressure in adults, as measured in the arms or legs. However, another type of the disease, pulmonary hypertension, can affect the lungs, causing health concerns for people of all ages, including the tiniest infants.

Pulmonary hypertension—sometimes referred to as PH—is a rare and complex condition characterized by stiff or narrow blood vessels in the lungs, which may be damaged or even reduced in density. Because these lung blood vessels don’t operate as they should, the heart must work harder to pump blood through them.

“The blood that flows through the lungs is typically under about five times lower pressure than the blood that flows through the rest of the body. The right side of the heart pushes blood into the lungs, but it isn’t programmed to deal with high pressure. That pressure is what can cause serious health concerns, including heart failure,” said Dr. Eric Austin, a pediatric pulmonologist and pulmonary hypertension specialist at Monroe Carell Jr. Children’s Hospital at Vanderbilt.

A rare disease with many causes

It’s estimated that this condition affects about 1-3% of Americans each year. Because it is rare, precise data about its prevalence is lacking. It is, however, known to have a variety of causes, with about 30% of cases occurring in patients with severe lung disease. Among children, that is most commonly associated with preterm birth.

It’s estimated that PH affects approximately 1-3% of Americans each year.

“With babies now being born as many as 20 weeks before their due date, we see infants not only with premature lungs and airways, but also immature lung blood vessels, making them susceptible to PH,” Austin said.

Other groups with a tendency toward the disease are those with a genetic predisposition, people with Down syndrome and other syndromic conditions, and children with a congenital heart disease

Getting the right diagnosis

Some children with pulmonary hypertension may initially be misdiagnosed with asthma or other conditions, Austin said, and will typically experience shortness of breath, difficulty participating in physical activities, and passing out or feeling like they will pass out. 

Once PH is confirmed through an echocardiogram, heart catheterization and other studies, patients are placed on medications to lower the blood pressure in the lung circulation. They are also treated for any accompanying medical conditions, which is often the case.

Living with pulmonary hypertension

Although some people with the condition may eventually come off therapy, many remain on medication throughout their lives, while some may also seek surgical options such as lung transplants.

“Discovering your child has PH is scary, but with experienced treatment teams and advances in research, we’ve been quite successful in helping children lead rich and fulfilling lives,” Austin said.

Comprehensive care

The Pediatric Pulmonary Hypertension Program at Monroe Carell Jr. Children’s Hospital at Vanderbilt is designated a Pulmonary Hypertension Comprehensive Care Center by the Pulmonary Hypertension Association, making it one of only eight pediatric programs in the country to hold this distinction.

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