At the Cleveland Clinic Respiratory Institute, our Pulmonary Hypertension Program is one of the nation's largest and most comprehensive treatment programs for patients with pulmonary hypertension. Our commitment is to provide patients with the best medical advice and to evaluate you in a timely manner with compassion and courtesy.
Patient Care–Patients First
For nearly 20 years, we have been caring for patients with all forms of pulmonary hypertension, including idiopathic pulmonary arterial hypertension (IPAH), chronic thromboembolic pulmonary hypertension, portopulmonary hypertension and pulmonary hypertension associated with connective tissue diseases.
Our physicians and nurses have special expertise and interest in pulmonary hypertension, and are dedicated to the evaluation and care of patients with pulmonary hypertension. Teams are comprised of pulmonary, cardiologists, critical care physicians, advanced practice nurses, registered nurses and research coordinators. We also collaborate closely with the departments of cardiovascular medicine, cardio-thoracic imaging, cardiothoracic surgery, and lung transplantation as well as specialists in hepatology, liver transplantation, sleep medicine, hereditary hemorrhagic telangiectasia (HHT) and rheumatology. As a result, our patients receive the most comprehensive, timely care as well as benefit from cutting-edge research and the best treatment options available for pulmonary hypertension.
As part of our commitment to excellence in patient care, research and education, we bring together local, national and international experts during our Pulmonary Hypertension Symposium. This event provides patients and medical professionals with a unique opportunity to learn about the most recent advancements in the diagnosis and treatment of pulmonary hypertension.
View Our Treatment Outcomes
Our Outcomes offer detailed information and data to help patients and their physicians make informed decisions about treatment for a wide-range of respiratory issues.
Pulmonary hypertension (PH) is a group of diseases characterized by high pulmonary artery pressures and pulmonary vascular resistance. Pulmonary hypertension can be idiopathic or secondary to an identifiable underlying pulmonary, cardiac or systemic disease.
Idiopathic pulmonary arterial hypertension (IPAH), previously known as primary pulmonary hypertension, is a progressive disease that affects predominantly young individuals, is more common in females, and traditionally had an average survival rate of two to three years from the time of diagnosis. Thanks to the availability of targeted or advanced therapies for pulmonary hypertension, this survival rate has improved in recent years.
Types of Pulmonary Hypertension
- PH originating at the pulmonary arteries (i.e., pulmonary arterial hypertension or PAH)
- PH originating in the heart (i.e., pulmonary venous hypertension or PVH)
- PH originating from abnormal lung tissue or due to poor oxygenation
- PH due to clots in the pulmonary arteries (i.e., chronic thromboembolic pulmonary hypertension or CTEPH)
- PH due to unclear mechanisms
Causes of Pulmonary Hypertension
Pulmonary hypertension is associated with several conditions including:
- Unknown causes (i.e., idiopathic)
- Scleroderma, lupus or other inflammatory diseases
- Heart, lung, liver, kidney and blood diseases
- Blood clots - this condition is called chronic thromboembolic pulmonary hypertension (CTEPH)
Treatments for Pulmonary Hypertension
At Cleveland Clinic, our patients are on various advanced pulmonary hypertension therapies, such as intravenous epoprostenol (Flolan and Veletri), intravenous, oral and inhaled treprostinil (Remodulin and Tyvaso), oral bosentan, macitentan, and ambrisentan (Tracleer, Opsumit and Letairis), oral sildenafil and tadalafil (Revatio and Adcirca), selexipeg( Uptravi), or riociquat(Adempas). Many patients are on combination therapy.
The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is a complex surgery called pulmonary thromboendarterectomy, which can potentially cure this disease. Cleveland Clinic is one of the few centers in the country with expertise in this procedure.
More Information from our Health Library
Learn more about symptoms, causes, diagnostic tests and treatments for pulmonary hypertension and other pulmonary vascular diseases in our Health Library.
Appointments & Locations
Make an Appointment
To make an appointment with a Cleveland Clinic pulmonary hypertension specialist, call 216.636.5327 or toll-free 1.800.223.2273 (ext. 65327).
Research & Clinical Trials
We have several ongoing investigator-initiated research projects aimed at understanding the pathophysiology of pulmonary hypertension (PH). Please contact our office as research options change.
For Medical Professionals
We offer elective, two-to-four-week rotations that include clinical or research experience for residents, pulmonary fellows, cardiology fellows and community physicians, who want to learn more about pulmonary hypertension, as well as pulmonary hypertension experiences for allied health professionals. For more information, visit the Center for Advanced Skills Training.
Disease Management Project
Cleveland Clinic publishes an online medical reference, the Disease Management Project, which provides nationally-established treatment guidelines for the most common diseases and conditions.
View an overview of treatment guidelines for:
View helpful publications written by Cleveland Clinic Respiratory Institute physicians on the topic of pulmonary hypertension.
- Hypoxia-inducible Factors in Human Pulmonary Arterial Hypertension: A Link to the Intrinsic Myeloid Abnormalities
Farha S, Asosingh K, Xu W, Sharp J, George D, Comhair S, Park M, Tang WH, Loyd JE, Theil K, Tubbs R, Hsi E, Lichtin A, Erzurum SC. Blood. 2011 Mar 31;117(13):3485-93.
- Update on Pulmonary Vascular Diseases 2010
Dweik RA, Erzurum SC. Am J Respir Crit Care Med. 2011 Jul 1;184(1):26-31.
- Plasma Levels of High-density Lipoprotein Cholesterol and Outcomes in Pulmonary Arterial Hypertension
Heresi GA, Aytekin M, Newman J, DiDonato J, Dweik RA. Am J Respir Crit Care Med.2010;182(5):661-8.
- Pulmonary Hypertension in COPD: Epidemiology, Significance, and Management: Pulmonary Vascular Disease: The Global Perspective
Minai OA, Chaouat A, Adnot S. Chest. 2010 Jun;137(6 Suppl):39S-51S.
- Somatic Chromosome Abnormalities in the Lungs of Patients with Pulmonary Arterial Hypertension
Aldred MA, Comhair SA, Varella-Garcia M, Asosingh K, Xu W, Noon GP, Thistlethwaite PA, Tuder RM, Erzurum SC, Geraci MW, Coldren CD. Am J Respir Crit Care Med. 2010 Nov 1;182(9):1153-60. Epub 2010 Jun 25.
- Strategic Plan for Lung Vascular Research: An NHLBI-ORDR Workshop Report
Erzurum S, et al. Am J Respir Crit Care Med. 2010 Dec 15;182(12):1554-62. Epub 2010 Sep 10. Review.
- Sarcoidosis-associated Pulmonary Hypertension. One Size Does Not Fit All
Heresi GA, Dweik RA. Chest. 2009 Jun;135(6):1410-2.
- High Levels of Hyaluronan in Idiopathic Pulmonary Arterial Hypertension
Aytekin M, Comhair SA, de la Motte C, Bandyopadhyay SK, Farver CF, Hascall VC, Erzurum SC, Dweik RA. Am J Physiol Lung Cell Mol Physiol. 2008 Nov;295(5):L789-99. Epub 2008 Sep 5.