Overview

Overview

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 imagingcardiothoracic 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).

Health Information

Health Information

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.

Pulmonary Hypertension

Medications Nutrition
Doctors

Doctors

Research & Clinical Trials

Research & Clinical Trials

We have several ongoing investigator-initiated research projects aimed at understanding the pathophysiology of pulmonary hypertension (PH).

Pulmonary Hypertension Clinical Trials

Pulmonary Vascular Complications of Liver Disease-2 (PVCLD2)

Sponsored by Perelman School of Medicine at the University of Pennsylvania as a subcontract of the NHLBI, 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. ELIGIBILITY: Patients age ≥ 18 years with chronic portal hypertension from intrinsic liver disease or portal vein disease, documented by clinical history or liver biopsy, referral for evaluation for liver transplantation (LT) or portopulmonary hypertension (or a known diagnosis of portopulmonary hypertension). Exclusion criteria include having an active infection, active or recent (< 2 weeks) gastrointestinal bleeding, lung transplant or LT recipients, being pregnant.

PRINCIPAL INVESTIGATOR: Gustavo Heresi, MD

STUDY COORDINATOR: Holly Radice | 216.444.2140


A randomized, double-blind, placebocontrolled, prospective, multicenter, parallel group study to assess the safety and efficacy of macitentan in patients with portopulmonary hypertension

Sponsored by Actelion Pharmaceuticals Ltd., this study’s main objective is to evaluate the effect of macitentan on pulmonary vascular resistance (PVR) as compared with placebo in patients with portopulmonary hypertension (PoPH). ELIGIBILITY: Men or women > 18 years with symptomatic PoPH, mPAP > 25 mm Hg, PAWP < 15 mm Hg, PVR > 4 Wood units. Exclusion criteria include severe hepatic impairment, severe obstructive or restrictive lung disease, undergoing dialysis, history of liver transplant, hepatocellular carcinoma, schistosomiasis infection, GI bleeding < 3 months prior to randomization, treatment with ERA within 3 months prior to randomization, treatment with interferon within 3 months prior to randomization, treatment with strong CYP3A4 inhibitors.

PRINCIPAL INVESTIGATOR: Adriano Tonelli, MD

STUDY COORDINATOR: Holly Radice | 216.444.2140


International CTEPH Registry

The primary objective is to describe the epidemiology of CTEPH and mode of diagnosis and treatment worldwide and determine long-term outcome as measured by NYHA class and survival. Sponsored by International CTEPH Association (ICA). ELIGIBILITY: Patients must be newly diagnosed with CTEPH; have been treated with anticoagulation for at least 3 months before diagnosis of CTEPH; mPAP > = 25 mm Hg at rest, abnormal VQ scan, pulmonary angiogram; CT pulmonary angiogram; or MR pulmonary angiogram confirming chronic thromboembolic disease as recommended by standard guidelines.

PRINCIPAL INVESTIGATOR: Gustavo Heresi, MD

STUDY COORDINATOR: Kasi Timmerman | 216.444.2140


U.S. CTEPH REGISTRY

The primary objective is to characterize the demographics, evaluation; and clinical course of WHO Group IV pulmonary hypertension, CTEPH. Sponsored by University of California, San Diego. ELIGIBILITY: Must be a permanent resident of the United States; mPAP > 25 at rest and PAWM < 15; radiologic confirmation that chronic thromboembolic disease is the cause of the pulmonary hypertension; one or more mismatched perfusion defect(s) by lung ventilation/perfusion scan and confirmation of chronic thromboembolic disease by evidence of bands/webs, vessel narrowing or occlusion seen on CT pulmonary angiogram (CTA), conventional angiography or R angiography (MRA); patients must be diagnosed with CTEPH within 6 months of being considered for study; patients cannot have an underlying disorder with an anticipated life expectancy of less than 2 years.

PRINCIPAL INVESTIGATOR: Gustavo Heresi, MD

STUDY COORDINATOR: Kasi Timmerman | 216.444.2140


A Phase 3, placebo controlled, double-blind, randomized, clinical study to determine efficacy, safety and tolerability of pulsed, inhaled nitric oxide (iNO) versus placebo in symptomatic subjects with pulmonary arterial hypertension (PAH)

Sponsored by Bellerophon Pulse Technologies, this study’s main objective is to evaluate the efficacy of inhaled nitric oxide (iNO) using the iNOpulse device as compared to placebo inpatients with PAH.

Eligibility: Men or women 18-80 years  of age diagnosed with WHO group 1 PAH who are on a stable dose of at least one PAH specific therapy, who are using oxygen therapy, mPAP > 25 mmHg, PCWP < 15, PVR > 5 wood units. Exclusion criteria include moderate to severe restrictive lung disease, undergoing dialysis, the use of a CPAP or BiPAP device, and Riociguat as therapy.

PRINCIPAL INVESTIGATOR: Gustavo Heresi, MD

STUDY COORDINATOR: Holly Radice | 216.444.2140


A Phase 2, Randomized, Double-BlInd, Placebo-Controlled Study of UBEnimex in Patients with Pulmonary ARTerial HYpertension (WHO Group 1) (LIBERTY)

Sponsored by Eiger Pharmacuticals, this study’s main objective is to evaluate the safety and efficacy of Ubenimex as compared with placebo in patients with Pulmonary Arterial Hypertension.

Eligibility: Men or women 18-75 years of age diagnosed with WHO group 1 PAH, on a stable dose of PAH specific therapy for at least 3 months prior to screening, mPAP>25 mmHg, PCWP<15, PVR>3.75 wood units. Exclusion criteria include significant restrictive lung disease, history of portal hypertension or liver disease, and having an active infection.

PRINCIPAL INVESTIGATOR:  Kristin Highland, MD, MS

STUDY COORDINATOR: Holly Radice | 216.444.2140


A Study of the Efficacy and Safety of Bardoxolone Methyl in Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

Sponsored by Reata Pharmaceuticals the study’s main objective is to assess the efficacy and safety of bardoxolone methyl relative to placebo in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). 

ELIGIBILITY: Symptomatic pulmonary hypertension WHO/NYHA FC class II and III; WHO Group I PAH associated with connective tissue disease; Had a diagnostic right heart catheterization performed and documented within 36 months prior to Day 1 that confirmed a diagnosis of PAH according to all the following criteria: a. Mean pulmonary artery pressure ≥ 25 mm Hg (at rest); b. Pulmonary capillary wedge pressure (PCWP) ≤ 15 mm Hg; c. Pulmonary vascular resistance > 240 dyn•sec/cm5 or > 3 mm Hg/liter (L)/minute.

PRINCIPAL INVESTIGATOR:  Kristin Highland, MD, MS

STUDY COORDINATOR: Mary Beukemann | 216.444.2140

For Medical Professionals

For Medical Professionals

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.