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Pulmonary Hypertension

Online Health Chat with Gustavo Heresi-Davila, MD and Nicholas Smedira, MD

September 26, 2012

Introduction

Cleveland_Clinic_Host: Pulmonary hypertension is a rare and serious lung disorder. It occurs in people of all ages, races and ethnic backgrounds. This condition is much more common in young adults, and is approximately twice as common in women as in men. One of the more severe causes of pulmonary hypertension is due to unresolved blood clots in the lungs, which is called chronic pulmonary thromboembolic disease (CTEPH). Although CTEPH can cause serious illness and can even be fatal in certain cases, it can be cured in some patients through a surgical procedure that removes the obstruction in the lung. Other causes of pulmonary hypertension may require a lung transplant to treat the patient.

For More Information

On Cleveland Clinic
Cleveland Clinic's Sydell and Arnold Miller Family Heart & Vascular Institute is one of the largest cardiovascular and thoracic specialty groups in the world, providing patients with expert medical management and a full range of therapies. Our areas of expertise combine research, education and clinical practice to provide innovative and scientifically based treatments for cardiac, vascular, esophageal and thoracic disease in 24 departments and centers. The commitment of our physicians and scientists to the prevention and cure of cardiovascular disease has led to innovative care, better outcomes and improved quality of life for patients. U.S. News & World Report has ranked Cleveland Clinic as the No.1 heart program in America every year since 1995.

At Cleveland Clinic's Respiratory Institute, we provide world-class patient care by combining our strengths in clinical expertise, research and education. Serving nearly 90,000 patients annually, the Respiratory Institute’s unsurpassed expertise and experience attracts patients from all over the world who seek a definitive diagnosis and cutting-edge treatment, often for a rare or complex lung disorder. With more than 60 pulmonologists, allergists/immunologists and critical care specialists, the Respiratory Institute diagnoses and treats a wide range of lung, allergy and breathing-related conditions. The Respiratory Institute includes these departments and centers: Pulmonary Hypertension, Respiratory Therapy, Sarcoidosis Center, Interstitial Lung Disease, Alpha-1 Antitrypsin Deficiency Center, Asthma Center, Bronchoscopy and Lung Cancer.

Among respiratory programs in the United States for 2012, Cleveland Clinic’s Respiratory Institute is ranked third in the nation by U.S.News and World Report.

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A remote second opinion may also be requested from Cleveland Clinic through the secure Cleveland Clinic MyConsult® website. To request a remote second opinion, visit eclevelandclinic.org/myConsult


For Appointments

To make an appointment with Gustavo Heresi-Davila, MD, or any of the other specialists in Pulmonary and Critical Care Medicine at Cleveland Clinic, please call 216.444.6697 or toll-free 800.223.2273, ext. 55763. You can also visit us online at http://my.clevelandclinic.org/respiratory.

To make an appointment with Nicholas Smedira, MD, or any of the other cardiothoracic surgeons at Cleveland Clinic, please call 216.445.9288 or toll-free 866.289.6911. You can also visit us online at http://my.clevelandclinic.org/heart


About the Speakers

Gustavo Heresi-Davila, MD, is a staff physician in the Department of Pulmonary, Allergy, and Critical Care Medicine at Cleveland Clinic. His specialties include pulmonary hypertension, CTEPH, acute respiratory distress syndrome (ARDS), pneumonia, pulmonary fibrosis, scleroderma, and sepsis.

Dr. Heresi-Davila completed his fellowship in pulmonary and critical medicine at the Cleveland Clinic following his residency in internal medicine at Jackson Memorial Hospital in Miami. Dr. Heresi-Davila graduated from medical school at Universidad Nacional de San Agustin Facultad de Medicina in Arequipa, Peru. Dr. Heresi-Davila is board-certified in the fields of internal medicine, critical care medicine and pulmonary disease.

Cleveland_Clinic_Host: Welcome to our Online Health Chat with Cleveland Clinic specialists Dr. Gustavo Heresi-Davila and Dr. Nicholas Smedira. We are thrilled to have them here today for this chat on Pulmonary Hypertension.

Nicholas G. Smedira, MD, is a staff cardiothoracic surgeon in the Department of Thoracic and Cardiovascular Surgery and Director of Quality for the Sydell and Arnold Miller Family Heart & Vascular Institute. He specializes in heart and heart-lung transplantation, ventricular assist devices, extracorporeal membrane oxygenation (ECMO), heart failure surgery, aortic and mitral valve repair and replacement, off-pump coronary bypass grafting, myectomy and the surgical treatment of left ventricular outflow tract obstruction, treatment of pulmonary hypertension caused by chronic pulmonary emboli, reoperations, and ascending aortic replacement. Dr. Smedira has performed more than 5,000 heart operations since 1995, and his accomplishments include the first implantation of the SynCardia Total Artificial Heart after FDA approval, the first combined heart-liver transplant in Ohio and the first ever successful bridge of a patient with a total artificial heart until he received a heart and liver transplant. Dr. Smedira is certified by the American Board of Thoracic Surgery and the American Board of Surgery.

Dr. Smedira received a fellowship in cardiothoracic surgery at Cleveland Clinic’s Heart Center, where he was appointed as a staff cardiac surgeon in 1995. Dr. Smedira also received fellowships in cardiothoracic surgery, critical care medicine and research from the University of California San Francisco Medical Center, where he also completed his residency in general surgery and became chief surgical resident. Dr. Smedira graduated from medical school with honors from the University of Rochester School of Medicine and Dentistry, Rochester, NY.


Let’s Chat About Pulmonary Hypertension

Cleveland_Clinic_Host: Welcome to our Online Health Chat with Cleveland Clinic specialists Dr. Paul Saluan and expert Adam Lake. We are thrilled to have them here today for Concussions and Sports Injuries in School-aged Athletes.


Diastolic Dysfunction and Pulmonary Hypertension

SunnyFL: How does diastolic dysfunction cause pulmonary hypertension? How do you test for diastolic dysfunction?
Dr__Smedira: The most common way to diagnose diastolic dysfunction is with an echocardiogram. We often think of diastolic dysfunction as the heart being unable to relax or that it is stiff. By being stiff, when the heart fills with blood, the pressure inside the heart rises and this is what causes pulmonary hypertension.

SunnyFL: My pulmonologist thinks I have diastolic dysfunction that is causing my pulmonary hypertension, but my cardiologist doesn't agree. He thinks it is my autoimmune disease. I have another echocardiogram scheduled in November. Should that give me the answer then?
Dr__Heresi: An echocardiogram can give some clues, but you really need a heart catheterization to distinguish different types of pulmonary hypertension.


Pulmonary Hypertension Diagnosis

mlg944: I am 53-year-old male with hypertension and on medications for that. Lately I've had the following symptoms while doing light yard work. I get winded and dizzy, so I have to stop and take a break. When I measure my blood pressure when I immediately come in the house, it is 108/63 with a pulse of 120. I experience heavy breathing and coughing for 24 hours. I measured my blood pressure five minutes after working outside and had a blood pressure of 100/58 with a pulse of 136. Ten minutes after working, I measured my blood pressure at 100/64 with a pulse of 118. Twenty five minutes after working outside, I had a blood pressure of 100/65 with a pulse of 112. Forty minutes after working outside, I had a blood pressure of 110/69 with a pulse of 102 - are these symptoms of pulmonary hypertension
Dr__Heresi: Not necessarily. Shortness of breath, dizziness and fast pulse rate can be seen with many other medical conditions. Pulmonary hypertension is one of them, but it would not be the most common. Checking for common things first like asthma and heart failure is best, but then move on to an echogram quickly if nothing is evident after the first evaluation.

mlg944: Is the echocardiogram considered a stress test?
Dr__Heresi: It can be. A stress echocardiogram would be a good idea to rule out coronary blockages that may need a coronary angiogram to confirm. That echo also would be a good screen for PH, which would need a right heart catheterization to be confirmed.

boocifur: Can anyone with lung disease ever be classified as Type 1 PH?
Dr__Heresi: If the lung disease is severe, no. But if the lung disease is mild and the pulmonary hypertension is very severe, you can view that as Type 1 PH.

browna20: What is the difference between primary and secondary pulmonary hypertension?
Dr__Heresi: Secondary means that we are able to identify a reason for the pulmonary hypertension, e.g., pulmonary fibrosis, blood clots, etc. Primary means we are not able to find a reason for the pulmonary hypertension. This is referred to as idiopathic nowadays.


Type 1 Pulmonary Hypertension (PAH)

maynard: Can you explain the difference between Type 1 pulmonary hypertension (PAH) and Type 5 PH?
Dr__Heresi: Type 1 PH is pulmonary arterial hypertension (PAH), which includes idiopathic disease. PAH is associated with connective tissue disease, HIV, congenital heart disease, etc. This type is the one where current PAH therapies are approved for. Type 5 PH is just a miscellaneous category that includes several medical conditions known to be associated with PH, but without a clear mechanism. These conditions include sarcoidosis, fibrosing mediastinitis, etc.


Blood Clots and Pulmonary Hypertension

james4: How often do blood clots cause pulmonary hypertension? What can be done about them?
Dr__Heresi: Blood clots are probably one of the most under-recognized causes of pulmonary hypertension, although precise estimates are not available. With a fresh acute clot, blood thinners are indicated and usually suffice. However, in about four percent of people after an acute fresh pulmonary clot, the clot can get stuck in the lungs and become scarred. This causes permanent pulmonary hypertension (type 4), which can be potentially cured by an operation called pulmonary thromboendarterectomy (PTE), which essentially removes the old clots.

james4: How can I know if my pulmonary hypertension is linked to blood clots?
Dr__Heresi: Based on symptoms and your history alone, it is not possible. At Cleveland Clinic we always screen for PH due to blood clots (chronic pulmonary thromboembolic disease, or CTEPH) with a VQ (ventilation/perfusion pulmonary) scan. We evaluate patients with CT scans, MRI and pulmonary angiography. These tests are needed to make a diagnosis of CTEPH and to assess patients prior to the operation.


Orthostatic Hypotension and Severe Tricuspid Regurgitation

Wynn: If my husband has autonomic dysfunction (possibly from pituitary adenoma pressure) as well as severe tricuspid regurgitation, which would you expect to be treated first?
Dr__Smedira: These are exceedingly complex issues that would require a great deal more information and direct consultation with the specialist(s). We would be happy to review the information through an e-consult or second opinion.

Wynn: My husband has a healthy lifestyle and a good weight. He is a non-smoker. However, friends are recommending we switch to a vegan (preferably raw food), low oil diet. Is there any medical verification that this change could be beneficial?
Dr__Heresi: Not to my knowledge.

 

Desquamative Interstitial Pneumonia Diagnosis

dthoresen: Is there a relationship between desquamative interstitial pneumonia (DIP) and pulmonary hypertension?
Dr__Heresi: Desquamative interstitial pneumonia (DIP), as any other interstitial lung disease, can lead to pulmonary hypertension, especially when associated with low oxygen levels. That being said, we do not see pulmonary hypertension commonly in DIP. Smoking cessation is key in the treatment of  DIP.

dthoresen: I never smoked.
Dr__Heresi: In that case, the diagnosis of DIP is suspect. Did they tell you desquamative interstitial pneumonia, or did you mean something else?

dthoresen: Cleveland Clinic told me based on video-assisted thoracoscopy.
Dr__Heresi: That is unusual, but certainly possible. Same comments about pulmonary hypertension though.

 

Pulmonary Hypertension Treatment

howjimbo: Are there any new medications available and what is the life expectancy of a patient with pulmonary hypertension with medications available today?
Dr__Heresi: With current pulmonary hypertension drugs, the three-year survival is about 65 percent to 67 percent. There are currently no new drugs available. However, a clinical trial on a drug called riociguat has been recently concluded, and the results will be released next month. At least three other compounds are under investigation, but the clinical trials have not been competed yet.

LucyJ: If a patient is on intravenous medications for pulmonary hypertension, will he always have side effects and to what extent?
Dr__Heresi: Not always. Side effects are highly variable, and some patients have a lot, while some don't. Now most people on intravenous medications will have some side effects, but doses are usually adjusted to find the right balance between benefit and side effects.

phylisk: What can a person do the make pulmonary hypertension better?
Dr__Heresi: The best thing to do is to follow with a pulmonary hypertension specialist. Otherwise, eating a healthy diet, not smoking, losing weight and light exercise (if allowed by your pulmonary hypertension expert) are always a good idea.

phylisk: How can you prolong your life with pulmonary hypertension?
Dr__Heresi: Current pulmonary hypertension medications can prolong life. Following up with a pulmonary hypertension expert is the best thing you can do.


Valve Leakage and Pulmonary Hypertension

tbradley: I had open heart surgery last December (aortic valve replacement with mechanical valve and triple bypass). Now it appears that I have leakage around my valve and regurgitation, which they say has caused pulmonary hypertension (PH). I can find nothing in the literature linking these two conditions (valve leakage and pulmonary hypertension) together, but my current symptoms do fit PH (shortness of breath and some pain—mostly when walking). How rapidly should I have a new operation if indeed the leakage is causing the pulmonary hypertension? I am in the process of scheduling a catheterization for as early as possible, but do need to be weaned off Coumadin® first.
Dr__Smedira: A leaky aortic valve can be a cause of pulmonary hypertension. A new operation would be indicated if the leak is severe and the risk of doing a re-operation is relatively low. The catheterization should measure pressures inside the heart, and this should help guide therapies.

PH is commonly seen as a consequence of idiopathic pulmonary fibrosis. In that context, PH drugs are really not indicated. I would agree with an evaluation for lung transplantation. Cleveland Clinic also has two ongoing clinical trials for IPF. Enrollment into one of them may be another option as well.

tbradley: Two cardiologists have told me that if the valve leakage is fixed, it is possible that the pulmonary hypertension will go away or at least subside. I thought pulmonary hypertension was permanent, that's why I'm most concerned about rapid surgical intervention. Could fixing the leakage stop or even reverse the pulmonary hypertension?
Dr__Heresi: The vast majority of patients with pulmonary hypertension from leaky valves will see dramatic reversals in the pulmonary hypertension when the leak is stopped. If the valves have been leaking for many years, there is the possibility that pulmonary hypertension will not completely normalize.

 

Pediatric Pulmonary Hypertension Treatment

Readyforchange: I have a 12-year-old son that was diagnosed with pulmonary hypertension and pulmonary hemosiderosis. He was just recently referred to an adult-pulmonologist who put him on Letairis® and Adcirca®. He was given a cardio-catheter, in which the pediatric cardiologist told us the numbers changed slightly for the better. Although he made a little progress the pulmonologist feels it's not enough. He wants to try Remodulin®. I did a little research and didn't like the outcome. So I guess my question is there any other medication out there for this illness before this drastic step?
Dr__Heresi: If your son is already on both Letairis® and Adcirca®, then the only option left for medical therapy for PH is a parenteral prostacyclin like Remodulin®. However, if he also has pulmonary hemosiderosis, which can cause significant pulmonary fibrosis, he may need to be evaluated for lung transplantation.

There is an upcoming Health Chat on Interstitial Lung Disease (ILD) on Wednesday, November 7 at noon with Drs. Daniel Culver and Joseph Parambil that might be of interest to you.


Pulmonary Fibrosis Treatment

Nivlag1: I have idiopathic pulmonary fibrosis (IPF) since May 2007. My pulmonary doctor recently said he is sure have pulmonary hypertension (PH), but does not want to send me to a PH doctor as "they are very aggressive in their treatment." He wants me to concentrate on continuing with weight loss, so I can be evaluated for transplant. What do you think of that? It worries me, but I don't know what to do and have heard the PH medications are very expensive.
Dr__Heresi: PH is commonly seen as a consequence of IPF. In that context, PH drugs are really not indicated. I would agree with an evaluation for lung transplantation. Cleveland Clinic also has two clinical trials ongoing for IPF. Enrollment into one of them may be another option as well.


Exercise and Pulmonary Hypertension

howjimbo: What is the best and safest exercise for people with pulmonary hypertension (PH)?
Dr__Heresi: First of all, you need to be stabilized on PH medications. Then, if your PH physician agrees, a stint with pulmonary rehabilitation is usually helpful. Then, aerobic exercise is the best (e.g., treadmill or light jogging). Also, be mindful if you need oxygen and make sure to use it. Finally, no weightlifting exercise.


Pulmonary Thromboembolectomy

james4: Do all heart centers perform pulmonary thromboembolectomies (PTE)?
Dr__Heresi: Only a few heart centers in the United States have the experience and capability to perform PTE. At Cleveland Clinic, specialists in pulmonary hypertension, clotting disorders, cardiac surgery and perfusionists (who manage the heart-lung machine) work together to perform this complex operation. Our 30-day mortality over the past two years is zero.


Prognosis Pulmonary Hypertension and COPD

phylisk: What should you do to make people understand that you can not wait on them hand and foot with COPD and pulmonary hypertension?
Dr__Heresi: This is difficult, but the more the patient moves—the better it is. Good luck!


Closing

Cleveland_Clinic_Host: I'm sorry to say that our time with Cleveland Clinic specialists Gustavo Heresi-Davila, MD and Nicholas G. Smedira, MD is now over. Thank you, Dr. Heresi-Davila and Dr. Smedira for taking the time to answer our questions today about pulmonary hypertension.
Dr__Heresi: Thank you very much for all of your questions today. Please know that we are here to help you!
Dr__Smedira: Thank you and good luck!


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