Pediatric Pulmonary Hypertension Program

Comprehensive care for children of all ages with pediatric pulmonary hypertension (PH), a rare disease that causes high blood pressure in the pulmonary arteries

What is pediatric pulmonary hypertension?

Pulmonary hypertension (PH) is a rare and often progressive disease resulting in high pressure in the blood vessels of the lungs. These vessels (pulmonary arteries and their smaller branches) can become dysfunctional, stiffened, or relatively narrowed and over time make the right side of the heart work harder. In severe cases, the small blood vessels in the lungs become damaged beyond repair. Additionally, blood clots can form in the vessels, further damaging the lungs.

There are different forms of PH; it may be genetic or acquired. It can affect children with congenital heart defects, infants with chronic lung disease and may be secondary to other conditions such as sleep apnea, blood clots, autoimmune disorders and liver disease.

Pulmonary hypertension conditions and causes

At Johns Hopkins All Children’s Hospital in St. Petersburg, Florida, we treat all types of pulmonary hypertension. The different types of pulmonary hypertension are broken down into different “groups” depending on their causes and other factors:

Group 1

Group 1 is called pulmonary arterial hypertension (PAH). It occurs when the arteries in the lungs become thickened, narrow or stiff, causing the right side of the heart to work harder to push blood through these arteries. This inhibits the heart from pumping enough blood through the lungs to go to the rest of the body.

PAH can have several causes. It can be inherited; caused by congenital heart disease, liver disease, connective tissue diseases, or a new genetic issue; heritable secondary to mutations; or induced by drugs or toxins. It may also be idiopathic, meaning the cause is unknown.

Group 1 includes persistent pulmonary hypertension of the newborn (PPHN). PPHN can be associated with several conditions, including:

• Congenital diaphragmatic hernia
• Meconium aspiration syndrome (MAS)
• Perinatal asphyxia
• Pulmonary dysplasia
• Respiratory distress syndrome (RDS)

Congenital heart defects that may lead to pulmonary arterial hypertension because of the damage they can cause to blood vessels include:

• Atrial septal defects
• Patent ductus arteriosus
• Transposition of the great arteries
• Ventricular septal defects

Group 2

Group 2 is caused by the heart failing on the left side, such as problems with how the heart squeezes or relaxes, or with the valves on the left side of the heart. Because of this, blood flow backs up and causes increased pressure in the lungs.

Heart conditions that may lead to this type of pulmonary hypertension include:

• Dilated cardiomyopathy
• Mitral stenosis
• Pulmonary vein stenosis

Group 3

Group 3 is caused by lung disease. This includes any disease that affects the air spaces and air tubes in the lungs including developmental abnormalities. Causes could include chronic lung diseases such as obstructive lung diseases in which the lung airways are narrowed and exhaling is difficult (COPD, emphysema, etc.); restrictive lung diseases in which the lungs don’t expand as easily when inhaling (interstitial lung disease, pulmonary fibrosis, etc.); or sleep apnea.

Low oxygen levels can also cause this group of PH, as well as conditions that impede lung growth such as low birth weight, prematurity, or congenital diaphragmatic hernia. These causes can all lead to arteries in the lungs becoming tightened, causing high blood pressure in the lungs.

Group 4

Group 4 is called chronic thromboembolic pulmonary hypertension (CTEPH), and is caused by blood clots in the blood vessels in the lungs (called “pulmonary embolism”). Scar tissue in the blood vessels of the lungs from the blood clots blocks normal blood flow, making the right side of the heart work harder.

Group 5

Group 5 is caused by a combination of many rare diseases including sickle cell disease, cancer, liver and kidney diseases.

Groups 1 and 3 are seen most often in children, however our team has experience treating patients with all types of pulmonary hypertension.

How pulmonary hypertension is diagnosed in children and infants

Pulmonary hypertension is most often initially diagnosed with an echocardiogram (an ultrasound of the heart).

Cardiac catheterization, where a thin tube catheter is guided to directly determine pressures and measure blood flow may be needed as well. This can help to confirm the diagnosis and determine the severity of pulmonary hypertension. Cardiac catheterization is performed by the expert team in our Interventional Cardiology program.

If appropriate depending on your child’s age, condition and other factors, we may also recommend an exercise stress test or other tests.

Signs and symptoms of pediatric pulmonary hypertension include:

• A bluish discoloration of the lips
• Chest pain
• Difficulty breathing or shortness of breath with activity
• Dizziness
• Fatigue (usually with activity is the first symptom to develop)
• Fainting, especially with exercise that increases heart rate

Treatment and services for pulmonary hypertension

We work to treat the underlying condition that may be causing pulmonary hypertension, such as a congenital heart defect, lung disease or other condition. We work with the appropriate specialists needed for your child’s condition, such as cardiology and pulmonology.

Therapies to treat pulmonary hypertension are used to lower pressure in the lungs and help blood flow more easily. This may include:

• Oxygen to help with breathing issues and relax the arteries in the lungs
• Medications to help promote dilation of blood vessels in the lungs
• Anticoagulants to reduce clotting
• Diuretics to reduce fluid in the body and help the heart work better
• Additional medications to improve heart function and blood flow

Our multidisciplinary team provides continuity of care and seamless transition between inpatient and outpatient care, and includes a pulmonary hypertension nurse coordinator who helps facilitate case management.

Why Choose Johns Hopkins All Children’s

Our multidisciplinary care team includes pediatric cardiologists and pulmonologists who focus on pulmonary hypertension, interventional cardiology, neonatology and specialized nurses and pharmacists. This team works together to diagnose and treat patients, forming comprehensive, coordinated care plans.

The services we offer include:

• Multidisciplinary Pulmonary Hypertension Clinic
• Comprehensive evaluation and diagnosis
• Individualized treatment plans
• Advanced imaging
• Cardiac catheterization
• Exercise stress tests
• Six-minute walk tests
• Cardiovascular surgery
• Active participation in clinical trials and research

Resources for families

The Pulmonary Hypertension Association offers a number of helpful resources for families, including living with pediatric PH, school and PH, and information specific to teens and young adults. Learn more.