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
- Lung disease
- 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 (i.e. pulmonary arterial hypertension, thromboembolic hypertension, pulmonary venous hypertension) is critical for the best treatment options. 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.
Our multidisciplinary team of experts is dedicated to studying and treating pulmonary hypertension. We have the expertise to comprehensively evaluate and diagnose, utilizing right-heart catheterization, pulmonary function test (lung function measures), ventilation perfusion lung scan (to measure air and blood flow to the lungs) EKG, blood test, chest x-ray and a stress test.
Pulmonary Hypertension and 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 program offers a broad array of treatments including medical therapies, clinical trials, lung transplantation and pulmonary thromboendarterectomy. 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 which patients, and to adjust them as needed. We are also very 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 – to increase the supply of blood and oxygen to the heart, while reducing its workload.
- Prostacyclin therapy - given intravenous, subcutaneous, 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 for which we have the most experience in the state.
- Oral PAH specific therapy, including endothelin receptor antagonists and phosphodiesterase type 5 inhibitors
- Pulmonary endarterectomy – a surgery to remove blood clots for thromboembolic pulmonary hypertension patients. The University of Michigan is currently the only institution in the state, and one of only a few in the region, to offer this therapy.
- Lung transplantation
- Clinical Trials- Investigational therapies for PAH that are not yet broadly available.
The Pulmonary Hypertension Program at the University of Michigan Samuel and Jean 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.
PH 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. A new pulmonary arterial hypertension quality of life tool is being studied in the SYMPHONY trial. Other clinical trials for pulmonary hyptertension patients include the MELODY-1 trial, a 12-week study to evaluate the safety and tolerability of the drug drmacitentan (Opsumit®) in subjects with pulmonary hypertension due to left heart disease), and Novel Screening Strategies for Scleroderma-Associated Pulmonary Arterial Hypertension, a study examining whether tests performed during exercise can improve the ability to find early pulmonary arterial hypertension in patients with Scleroderma.
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.