Caring for children with pulmonary hypertension (PH) – a rare condition marked by high blood pressure in the lungs – can be extremely challenging. If not appropriately diagnosed and treated, PH can cause long-term heart damage and is ultimately fatal. In cases of severe PH, children often talk and act normal but are at high risk of sudden cardiac death.

“Those are the patients that make us the most nervous, but where I can also be the most helpful, to come in, do a comprehensive assessment, say what the patient’s risk factors are and make a treatment plan,” says Megan Griffiths, M.D., Pediatric Cardiologist at Children’s Health℠ and Assistant Professor at UT Southwestern.

Dr. Griffiths launched the Pulmonary Hypertension Program within the Children’s Health Heart Center in September 2022. In addition to managing severe cases, she is working with her colleagues in cardiology, pulmonology and neonatology to care for babies born with PH and children with chronic PH using the latest diagnostic approaches and treatments.

The big picture: Rare disease, but common comorbidity

PH affects about 25 to 30 children per million in the U.S. each year. Primary PH – also known as idiopathic, or without a known cause – is very unusual in children.

“But as a complication of other diseases, PH is not that rare,” says Dr. Griffiths, who recently completed a fellowship in pulmonary hypertension at Columbia University before joining Children’s Health.

Dr. Griffiths estimates that 10 to 20% of extremely premature babies (born at less than 28 weeks) whose lungs are not fully formed develop PH. They represent the largest patient group in the program. Many of the patients are also children with congenital heart disease.

Other risk factors for pediatric PH include heart, kidney and other organ transplantation, treatment for cancer, and other diseases and conditions such as Down syndrome and sickle cell disease. PH can also be a complication of COVID-19 because the infection is associated with thrombosis, which can cause high pulmonary blood pressure.

Despite the severity of PH, it often appears as nonspecific and even mild symptoms – fatigue, fainting and shortness of breath during activity. Children tend to compensate for these by limiting their activity. When they do receive medical care, they are sometimes mistakenly treated for asthma, Dr. Griffiths says. The average time to receive a PH diagnosis from symptom onset is two years.

However, Dr. Griffiths notes that physicians have become better at diagnosing the condition. New recommendations to screen premature infants with lung disease for PH have helped, too. Babies in the Children’s Health NICU receive routine PH screening by an echocardiogram.

Recently, Dr. Griffiths saw a 4-year-old child who had PH because of congenital heart disease. The child had always been lethargic, but physicians had not been able to determine if the symptoms were entirely because of her heart defect. The child finally received a diagnosis of PH and started receiving IV therapy.

“She’s like a new child, able to keep up with her siblings,” Dr. Griffiths says. “I smile every time I see her.”

Key details: How advances in diagnosis and treatments are helping children

The Pulmonary Hypertension Program sees about 15 patients with PH every day. They range in age from newborns to 21-year-olds. Children are typically referred to the program because a cardiologist, or sometimes the child’s pediatrician, suspects PH.

The program carries out a comprehensive assessment that includes imaging and blood tests of the lungs, heart and other organs. Dr. Griffiths pays special attention to the pulmonary CT imaging because it can reveal blood clots in the lungs. If a patient has lung disease along with PH, Dr. Griffiths works with her pulmonology colleagues to treat both conditions.

The best way to definitively diagnose PH is by cardiac catheterization to measure pressure in the pulmonary blood vessels and blood flow from the heart into the lungs. Children’s Health is one of very few hospitals where patients receive cardiac catheterization inside an MRI scanner, Dr. Griffiths says. This makes it possible to accurately measure blood flow and determine the best treatment for children with congenital heart disease and unrepaired shunts, or irregular blood flow patterns.

Since starting the program, Dr. Griffiths has been working to expand the use of newer treatment options. Instead of the standard pills that many physicians are familiar with – sildenafil and bosentan – she prescribes tadalafil for both new and chronic patients that do not have signs of heart failure. This medication has less frequent dosing, lower toxicity and can be more effective. “It is easier for kids to take and for families to manage,” she says.

Children with severe PH and signs of heart failure require inhaled or IV medications. While inhaled nitric oxide is a standard medication, Dr. Griffiths and the pharmacists on her team have the expertise to provide inhaled or IV treprostinil and other medications, which she says patients may respond better to.

For example, a newborn baby with severe PH and heart failure was recently transferred to the program. Dr. Griffiths and her colleagues treated the baby with epoprostenol and were able to avoid putting the infant on ECMO.

Why Children’s Health: Getting kids on track for healthy lives

Children’s Health is one of only a few centers in a large region to provide advanced PH care. Dr. Griffiths encourages cardiologists and pediatricians who think a child may have PH to refer to her program. Patients who are experiencing serious symptoms can be transferred to the program where Dr. Griffiths and her team will work to stabilize them.

In some cases, children see a local cardiologist, pulmonologist or other specialist and come back to Children’s Health for medication or if their condition worsens. Although there is no cure for PH in many cases, children are able to have longer, good and functional lives because of treatment advances.

Looking ahead, Dr. Griffiths plans to share data on patient outcomes through the Pediatric Acute Care Cardiology Collaborative (PAC3), a network of clinicians and hospitals, which is co-directed by Nicolas Madsen, M.D., M.P.H., who is Co-Director of the Heart Center and Chief of Cardiology at Children’s Health.

Dr. Griffiths aims for Children’s Health to become an accredited pediatric PH center in the U.S. There are currently only eight of these centers, and none of them are in North Texas. For Dr. Griffiths, it would mean more progress in her mission to help kids with PH live the fullest possible lives.

“When I was working in New York, I remember treating children who had to travel there from Texas for PH treatment,” she says. “I’m very happy that we’re now able to give world-class care to these kids, much, much closer to their homes.”

Learn more about the innovative care at the Children’s Health Heart Center.>>