Pulmonology

Pulmonary Hypertension

Our program diagnoses, manages and treats pediatric pulmonary hypertension in our Pulmonary Hypertension Clinic and Infant Pulmonary Clinic and offers the latest treatments and FDA-approved therapies. Our multidisciplinary care team includes a pulmonologist and cardiologist who work together diagnosing and treating patients and discussing long-term care plans.

Pulmonary hypertension (PH) is a progressive lung disease that causes high blood pressure in the pulmonary arteries. The pulmonary arteries shrink over time and make the right side of the heart work harder to force blood through the narrowed arteries. In severe cases, the small blood vessels in the lungs become damaged beyond repair. Additionally, blood clots can form in the vessels causing a loss of air spaces and blood vessels in the lungs.

Conditions treated in clinic include:

  • Group 1 pulmonary arterial hypertension (PAH): caused by congenital heart disease, connective tissue disease or a genetic issue
  • 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 
  • 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 

Signs and symptoms of pulmonary hypertension:

  • 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 heartrate 
  • Swelling and/or discoloration in the lower legs or ankles