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Pulmonary Hypertension

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.

More Information

Learn more about symptoms, causes, diagnostic tests and treatments for pulmonary hypertension and other pulmonary vascular diseases.

Web Resources

Find additional information on the following helpful websites.

The following Cleveland Clinic Respiratory Institute physicians specialize in the treatment of pulmonary hypertension and other pulmonary vascular diseases:

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), or email Jessica Klinko, Medical Secretary, at klinkoj@ccf.org.

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 Multi-Center, Double-Blind, Randomized, Placebo-Controlled, Parallel-Group, Phase 3 Study To Evaluate The Effects of Macitentan On Exercise Capacity in Subject With Eisenmenger Syndrome

A multi-center, double-blind, randomized, placebo-controlled, parallel-group, Phase 3 study to demonstrate that macitentan improves exercise capacity in comparison with placebo in subjects with Eisenmenger Syndrome (ES). For more information, please contact Kasi Zimmerman at 216.444.2140

A Clinical Study to Determine Safety, Tolerability and Efficacy of Pulsed, Inhaled Nitric Oxide (iNO) Versus Placebo as Add-On Therapy in Symptomatic Subjects with Pulmonary Arterial Hypertension (PAH)

A study to determine if inhaled nitric oxide (iNO) given through a special delivery device (INOpulse® DS) is safe and efficacious in treating Pulmonary Arterial Hypertension (PAH).The primary endpoint is change in pulmonary vascular resistance (PVR) (dynes. sec/cm-5) from baseline to EOS Part 1, sponsored by Ikaria. For more information, please contact Kasi Timmerman at 216.444.2140

A Multi-Center, Open-Label, Single-Arm, Phase 3b Study of Macitentan in Patients With Pulmonary Arterial Hypertension to Psychometrically Validate the PAH-SYMPACT Instrument.

A multi-center, open-label, single-arm, Phase 3b study to demonstrate the final content validity, the psychometric characteristics of reliability and construct validity, and the ability of the PAH-SYMPACT instrument. 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.

» Registry of Sarcoidosis-Associated Pulmonary Arterial Hypertension

For more information about clinical trials for pulmonary hypertension at Cleveland Clinic, call Katie Zak at 216.636.2421

Education

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.

References

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:

Recommended Readings

View helpful publications written by Cleveland Clinic Respiratory Institute physicians on the topic of pulmonary hypertension.

Reviewed: 07/11

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