Pulmonary Hypertension

Pulmonary hypertension is high blood pressure in the pulmonary arteries in the lungs. With symptoms typical of more common diseases, and a variety of causes, this potentially fatal disease is complex to diagnose and treat.

Causes of Pulmonary Hypertension

Pulmonary hypertension can have no known cause, can be genetic, can be caused by drugs or toxins, or can occur because of an underlying disease or issue, including:

  • Congenital heart disease
  • Left heart disease
  • Blood clots in the lungs (pulmonary embolism)
  • Lung disease
  • HIV
  • Liver disease
  • Sickle cell disease
  • Metabolic disorders
  • Sleep disorders, such as sleep apnea
  • Connective tissue diseases, such as scleroderma

Diagnosis and Types of Pulmonary Hypertension

Properly diagnosing this disease, as well as classifying the exact type of pulmonary hypertension, is critical for the best treatment options.

Types of pulmonary hypertension include:

  • Pulmonary arterial hypertension (PAH): A disease of the blood vessels of the lungs, which become thick and narrow, causing elevation in pressure.
  • Pulmonary venous hypertension (PVH): An elevation in blood pressure in the pulmonary veins, most often caused by congestive heart failure.
  • Chronic thromboembolic pulmonary hypertension (CTEPH): Pulmonary hypertension associated with the body’s inability to clear blood clots from the lungs (known as chronic thromboembolic obstruction). The disease is often present without any known history of previous pulmonary embolism.
  • Exercise-induced pulmonary hypertension: A condition in which the pulmonary arteries are normal at rest, but increase abnormally with exertion.

The earlier a patient is diagnosed with pulmonary hypertension, the better the chances for an optimal treatment solution. With shortness of breath being the most common symptom of pulmonary hypertension, it’s easy to get misdiagnosed.

We have multiple techniques to diagnose pulmonary hypertension, including:

  • Right-heart catheterization
  • Pulmonary function test (to measure lung function)
  • Ventilation/perfusion scan (to measure air and blood flow to the lungs)
  • Electrocardiogram (EKG)
  • Blood test
  • Chest X-ray
  • Stress test

Pulmonary Hypertension Treatment Options

While there is no cure for pulmonary hypertension, thanks to advances in the treatment over the past decade, more effective options are available to patients than ever before.

Our breadth of experience with all types, stages and severities of pulmonary hypertension means we have the ability to properly treat our patients using the highly specialized and complicated medical therapies for this condition.

Due to the complex nature of this disease, every patient’s treatment protocol must be tailored and carefully monitored on a regular basis. Our physicians and staff are skilled in determining which medical therapies will work best for individual patients, and to adjust treatment as needed. We are also involved in multiple investigational trials for new treatments, which means our patients have access to the latest and best options.


Our treatments include:

  • High-dose calcium channel blocker therapy:  Increases the supply of blood and oxygen to the heart, while reducing its workload.
  • Prostacyclin therapy: Given intravenously, subcutaneously or inhaled, prostacyclins help dilate (open) blood vessels in the lungs and improve the function of the heart. This is a complex and aggressive treatment.
  • Oral PAH specific therapy, including endothelin receptor antagonists and phosphodiesterase type 5 inhibitors: A targeted therapy to slow the progression of PH and possibly reverse some of the damage to the heart and lungs.
  • Pulmonary endarterectomy (PEA): A surgical procedure to remove blood clots from the lungs of patients with chronic thromboembolic pulmonary hypertension (CTEPH). The University of Michigan is currently the only institution in the state, and one of only a few in the region, to offer this extremely complex therapy.
  • Balloon pulmonary angioplasty (BPA): A procedure in which a deflated balloon is directed to the diseased lung vessels and inflated to break up blood clots and restore blood flow. For more information, visit the Balloon pulmonary angioplasty (BPA) page.
  • Lung transplantation
  • Clinical Trials: Investigational studies providing patients with access to the latest pulmonary hypertension treatment and management therapies.
Pulmonary Hypertension Logo

Accredited Through the Pulmonary Hypertension Care Centers Program

The Pulmonary Hypertension Program at the University of Michigan Frankel Cardiovascular Center is the largest and most experienced program in the state, and one of the largest in the country. We are a comprehensive, single resource for the care and treatment of patients who live with this challenging disease. In addition, we are accredited as a Center of Comprehensive Care (CCC) through the Pulmonary Hypertension Care Centers (PHCC) program.

Pulmonary Hypertension Research and Clinical Trials

Within the last decade, great advances have been achieved in improving existing treatments as well as the creation of new options for pulmonary hypertension. The University of Michigan has been involved in many of the clinical trials that have led to these discoveries. We lead the state in experience and volume of current enrolling clinical trials, and our physicians are often in leadership roles on these studies.

Research gives patients direct access to the latest therapies to treat and manage pulmonary hypertension. Clinical trials include TROPHY-1, an evaluation of the TIVUS System for pulmonary artery denervation in patients with pulmonary hypertension, and Novel Screening Strategies for Scleroderma-Associated Pulmonary Arterial Hypertension, a study examining whether tests performed during exercise can improve the ability to detect early pulmonary arterial hypertension in patients with scleroderma.

U-M also serves as the Data Coordinating Center for the Pulmonary Hypertension Breakthrough Initiative, a network of multidisciplinary, collaborative transplant and research centers that distributes stored clinical specimens and relevant data to researchers for use in groundbreaking research to better understand pulmonary arterial hypertension.

Patient Resources

Make an Appointment

To schedule an appointment to discuss your pulmonary hypertension or any other heart and vascular issue, call us at 888-287-1082. Visit our Make an Appointment page for more information about what to expect when you call us.