Pulmonary hypertension (PH) is a lung disorder in which the blood pressure in the pulmonary arteries rises above normal levels.

There are many causes of pulmonary hypertension.

Known causes include:

  • Vascular diseases such as scleroderma, lupus or Crest syndrome
  • Congenital heart diseases such as ventricular and atrial septal defects
  • HIV infection
  • Liver disease

The increased pressure causes your body to have to work harder to pump blood through the lungs, and as a result, the right side of the heart becomes enlarged. As the disease progresses, right heart failure may develop.

There are several types of Pulmonary Hypertension:

  • Pulmonary arterial hypertension (PAH): This is a condition where the arteries that supply blood to the lungs become narrow. This increases the resistance to blood flow, resulting in higher lung pressures. 
  • Pulmonary Hypertension Due to Left Heart Disease: A condition where disorders of the left side of the heart, such as left heart failure, heart stiffening and valvular heart disease can result in PH.
  • Pulmonary Hypertension Due to Lung Disease: This is a condition where diseases that affect the lung tissue result in pressure elevation. These include obstructive sleep apnea, chronic obstructive pulmonary disease, and interstitial lung disease.
  • Chronic Thromboembolic Pulmonary Hypertension (CTEPH): This is a condition where blood clots result in narrowing of the pulmonary arteries. If the blood clots do not fully dissolve, they can cause PH.
  • Idiopathic Pulmonary Arterial Hypertension: When pulmonary hypertension occurs without a known cause, it is called idiopathic pulmonary arterial hypertension (IPAH). This means that there are no other diseases of the heart or lungs causing the high blood pressure. Some cases are inherited, called familial PAH.
  • Miscellaneous: This includes a heterogeneous group of disorders, including kidney disease, and anemias, which can cause PH of varying severity.

Pulmonary Hypertension Diagnosis

We perform a series of tests to show how your heart and lungs are working, in order to help us diagnose pulmonary hypertension. These tests help determining the cause of PH, the severity of PH, and help in developing a treatment plan. Tests include electrocardiogram, ventilation/perfusion scan, right heart cardiac catheterization and echocardiogram. Learn more about cardiac diagnosis.

Pulmonary Hypertension Symptoms

Early on, you may have subtle or few symptoms, making it challenging to diagnose PH. Symptoms may include:

  • Shortness of breath
  • Chest pain, especially during physical activity
  • Weakness
  • Fatigue
  • Fainting
  • Lightheadedness during exercise
  • Dizziness (especially when climbing stairs or upon standing up)
  • Leg and ankle swelling
  • Physical examination that shows: enlargement of the neck veins, normal lungs, a heart murmur, enlargement of the liver and swelling due to fluid retention.
Pulmonary Hypertension Treatment

PH is a chronic condition that can become life threatening. Treatment options include:

  • Medications: There are many medication options available to you. Our patients have many more options than in the past as well as improved life expectancy. The goal of medical treatment is to slow the progress of the disease and improve your symptoms. We will discuss the possible medications with you and create a personalized plan for you.
  • Clinical trials: Our patients have the opportunity to participate in clinical trials, which means access to the latest drugs and therapies before they are available to the general public.
  • Pulmonary thromboendarterectomy (PTE): This is the only known cure for chronic thromboembolic pulmonary hypertension. We are one of the few medical centers in the country offering this procedure. Learn more about PTE.
  • Lung transplant: If the medications are not providing sufficient relief, we will discuss a lung transplant evaluation. Learn more about lung transplant.