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.
How pulmonary hypertension is diagnosed
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.
Signs and symptoms of pulmonary hypertension include:
- A bluish discoloration of the lips
- Chest pain
- Difficulty breathing or shortness of breath with activity
- Fatigue (usually with activity is the first symptom to develop)
- Fainting, especially with exercise that increases heart rate
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.
Pulmonary hypertension conditions and causes
We treat all types of pulmonary hypertension, including:
- Group 1 pulmonary arterial hypertension: Inherited, caused by congenital heart disease, connective tissue disease or a new genetic issue. Group 1 includes persistent pulmonary hypertension of the newborn.
- Group 2 pulmonary hypertension: Caused by the heart failing on the left side and pressure backing up into the lungs.
- Group 3 pulmonary hypertension: Caused by any disease that affects the air spaces and air tubes in the lungs including developmental abnormalities.
- Group 4 pulmonary hypertension: Caused by blood clots in the blood vessels in the lungs (pulmonary embolism).
- Group 5 pulmonary hypertension: Caused by a combination of many rare diseases including sickle cell disease, cancer, liver and kidney diseases.
Learn more about the types of pulmonary hypertension.
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.
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.
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.
Meet Our Team
Meet our specialists with experience in caring for children with pulmonary hypertension.
For more information or appointments, please call the PH nurse coordinator.