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 (PAH), 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 and critical care physicians, advanced practice nurses, research nurse coordinators and research fellows. We also collaborate closely with the departments of cardiovascular medicine, cardiovascular imaging, cardiothoracic surgery, and lung transplantation as well as specialists in hepatology, liver transplantation, sleep medicine 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 annual 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.
Make an Appointment
To make an appointment with a Cleveland Clinic pulmonary hypertension specialist, call 216.445.5763 or toll-free 1.800.223.2273 (ext. 55763).
Pulmonary hypertension (PH) is a group of diseases characterized by high pulmonary artery pressures and pulmonary vascular resistance. Pulmonary hypertension can be idiopathic (primary) 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 in the 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 (i.e., idiopathic pulmonary hypertension)
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 the Cleveland Clinic, our patients are on various advanced pulmonary hypertension therapies, such as intravenous epoprostenol (Flolan and Veletri), intravenous and inhaled treprostinil (Remodulin and Tyvaso), oral bosentan and ambrisentan (Tracleer and Letairis), oral sildenafil and tadalafil (Revatio and Adcirca). 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.
Learn more about symptoms, causes, diagnostic tests and treatments for pulmonary hypertension and other pulmonary vascular diseases.
Find additional information on the following helpful websites.
- American Heart Association
- Cleveland Area Pulmonary Hypertension Support Group
- Pulmonary Hypertension Association
Research & Clinical Trials
We have several ongoing investigator-initiated research projects aimed at understanding the pathophysiology of pulmonary hypertension (PH).
Pulmonary Hypertension Research
A main research focus of the Pulmonary Hypertension Program is nitric oxide in pulmonary hypertension. Nitric oxide is a gas that can be detected in the exhaled breath of humans and is found in particularly high concentrations in the upper respiratory tract (nasal and sinus passages). Cleveland Clinic Respiratory Institute physicians and researchers have discovered patients with pulmonary arterial hypertension (PAH) have low levels of nitric oxide in their exhaled breath, and replacing the nitric oxide with the use of a vasodilator seems to be treating the problem well.
Other research interests include the non-invasive evaluation of pulmonary hypertension, the association with metabolic syndrome (obesity), endothelial dysfunction and biomarkers of pulmonary hypertension.
Currently, we have ongoing grants from the National Institutes of Health and from the Gilead Scholars Program to examine the use of a s-nitrosothiol analyzer for the clinical diagnosis of cardiovascular disease (NIH: SBIR PA-09-080 – R43/R44) and myeloperoxidase in pulmonary hypertension.
Pulmonary Hypertension Clinical Trials
We also participate in several multicenter clinical trials evaluating new therapies for the treatment of pulmonary hypertension. Click on the title of the clinical trial to view more information, such as principal investigator, sponsor, purpose of the trial and patient population:
A randomized, double-blind, placebo-controlled phase II study to investigate the efficacy and safety of Riociguat (0.5mg, 1.0mg, 2.0mg, and 2.5mg TID) in patients with symptomatic pulmonary hypertension associated with idiopathic interstitial pneumonias (IIP)
To evaluate the efficacy and safety of 26-weeks of treatment with riociguat vs. placebo in patients with symptomatic PH associated with IIP. For more information please contact Kasi Timmerman at 216-444-2140
A multinational, multicenter study to assess the effects of oral Sildenafil on mortality in adults with pulmonary arterial hypertension (PAH)
This is a randomized, double-blind, parallel-group study in adult patients with PAH that is designed to assess mortality during long-term treatment with sildenafil at three doses. For more information please contact Kasi Timmerman at 216.444.2140
A phase 2, dose-ranging, randomized, double-blind, placebo-controlled study of GS-4997 in subjects with pulmonary arterial hypertension
This study will compare the efficacy, safety, and tolerability of 3 doses of GS-4997 to placebo in subjects with PAH. Study drug will be administered on the background of stable PAH therapy. The study will consist of screening followed by a 24-week blinded placebo-controlled treatment period (Period 1), and a long-term blinded GS-4997 treatment period (Period 2). For more information please contact Kasi Timmerman at 216.444.2140
Pulmonary Vascular Complications of Liver Disease-2 (PVCLD2)
The purpose of this study is to determine if certain genes, hormones, or other factors predict the risk of developing lung vessel disease in patients with liver disease and whether they determine outcome. For more information, please contact Mario Becerra at 216.445.7599.
For more information about clinical trials for pulmonary hypertension at Cleveland Clinic, contact Kasi Timmerman at 216.444.2140
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.