November 6, 2013
Pulmonary hypertension is a rare and serious lung disorder. It occurs in individuals of all ages, races, and ethnic backgrounds although it is much more common in young adults and is approximately twice as common in women as in men. Symptoms of pulmonary hypertension do not usually occur until the condition has progressed. The first symptom of pulmonary hypertension is usually shortness of breath with everyday activities, such as climbing stairs. In more advanced stages of the disease, even minimal activity will produce some of the symptoms. Additional symptoms include irregular heart beat (palpitations or strong, throbbing sensation), racing pulse, passing out or dizziness, progressive shortness of breath during exercise or activity, and difficulty breathing at rest. Eventually, it may become difficult to carry out any activities as the disease worsens. Appropriate diagnosis and analysis of the problem is necessary before starting any treatment. Treatment varies per individual based on the different underlying causes but generally includes taking medications; making lifestyle and dietary changes; having surgery, if necessary; and seeing your doctor regularly.
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. 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)
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 medication therapies for pulmonary hypertension, this survival rate has improved in recent years. Additionally, a complex surgery called pulmonary thromboendarterectomy can be used to treat—and potentially cure—chronic thromboembolic pulmonary hypertension (CTEPH).
About the Speaker
Adriano R. Tonelli, MD is a staff physician in Cleveland Clinic’s Department of Pulmonary, Allergy, and Critical Care Medicine. He is board-certified in internal medicine with additional specialty certification in pulmonary disease and critical care medicine. Dr. Tonelli’s clinical interests include pulmonary hypertension, pulmonary embolism, and scleroderma. He is a KL2 Clinical Research Scholar with a focus on pulmonary hypertension and a special interest in noninvasive diagnostic modalities and pulmonary hypertension in the context of liver diseases. Dr. Tonelli sees patients at Cleveland Clinic main campus.
Dr. Tonelli completed his pulmonary and critical care fellowship at University of Florida, Gainesville, Florida. He completed his residency in internal medicine at Sparrow Hospital, Michigan State University, in Lansing, Mi. Dr. Tonelli also completed fellowships in electrophysiology and cardiology at Fundacion Favaloro, Buenos Aires, Argentina. He graduated as valedictorian of his medical school class at the Universidad Nacional Cuyo, in Mendoza, Argentina.
Let’s Chat About Pulmonary Hypertension
Pulmonary Hypertension Diagnosis: Signs and Symptoms
donrobi: Does early identification of pulmonary hypertension provide an opportunity to cure the disease? If so, how can it be diagnosed early?
Adriano_Tonelli,_MD: There are no cures for the disease at this point. Research continues with that goal in mind. Ideally the sooner you identify and treat the condition, the better. The disease is usually suspected when patients have shortness of breath and an abnormal echocardiogram.
Batyah: What are the initial signs and symptoms of pulmonary hypertension (PH)? Can someone have PH without symptoms?
Adriano_Tonelli,_MD: Shortness of breath with activity, passing out or dizziness, and swelling of the lower extremities.
hotoldlady: Does this swelling of the lower extremities include the abdomen?
Adriano_Tonelli,_MD: Some patients with advanced form of pulmonary hypertension (PH) develop ascites (fluid inside the abdomen) that on occasion requires drainage. In general, swelling in PH occurs in the dependent areas like the lower extremities.
witter123: Can left side heart failure eventually cause PH?
Adriano_Tonelli,_MD: Yes. A significant number of patients with left heart disease develop pulmonary hypertension at some point. The usual management for this type of patient is the treatment of the left heart disease. As this improves, the PH also gets better.
walter: I have been diagnosed with pulmonary hypertension (PH), but a later diagnosis indicted no PH. I do have times that I am worn out at the end of the work day.
Adriano_Tonelli,_MD: Right heart catheterization is the gold standard. If the right heart catheterization is performed in a center with experience in the diagnosis of the disease, and it is normal—you do not have PH. There are several other causes of shortness of breath or fatigue that need to be investigated. Few patients can have PH induced by exercise or fluid challenge. This is usually rare, and requires further investigation of this diagnosis in patients.
Batyah: What is the connection between sleep apnea and pulmonary hypertension (PH)?
Adriano_Tonelli,_MD: Some patients with uncontrolled sleep apnea can develop PH. This is likely in relation with the low oxygenation that occurs during the night. Adequate treatment of obstructive sleep apnea (OSA) should help in the treatment of this condition. If PH is severe and does not improve with the treatment of the sleep disorder, then other reasons for PH need to be investigated.
Batyah: Can pulmonary hypertension (PH) cause a reduction in oxygen during sleep?
Adriano_Tonelli,_MD: This is a very good question. Yes, patients with PH can have low oxygenation during sleep. Interestingly, patients with sleep apnea—a condition associated with low oxygenation during sleep—can also be a cause of mild PH.
hotoldlady: At what point do I begin therapy for pulmonary hypertension (PH)?
Adriano_Tonelli,_MD: Treatments for PH are approved for patients with some degree of shortness of breath with activity. If you have no symptoms, the echocardiogram is normal, and the blood work is normal, then you can be followed closely by a physician with experience treating the disease.
lizziel: Can having pulmonary ablations for atrial fibrillation cause pulmonary hypertension? Can having sarcoid of the lungs in childhood that has been in remission for 30 years cause pulmonary hypertension?
Adriano_Tonelli,_MD: Rarely ablation for atrial fibrillation can narrow the pulmonary veins and give an uncommon type of pulmonary hypertension. If sarcoid is in remission, it is not likely the cause of PH.
Sarcoidosis and Pulmonary Hypertension
Batyah: What is the connection between sarcoidosis and pulmonary hypertension (PH)? As a sarcoidosis patient, what can I do to prevent or minimize the risk of having PH? As a sarcoidosis patient, what are the treatments for PH?
Adriano_Tonelli,_MD: A limited number of patients with sarcoidosis develop PH. It is hard to predict which patients with sarcoidosis will develop the disease. A regular follow up would be able to determine if you develop this. There are no FDA approved treatments for PH in sarcoidosis at this point. A recent article suggested that Tracleer® (bosentan) may be a useful medication.
SarcoidLady: For a person who already has pulmonary sarcoidosis, what are the symptoms of developing pulmonary hypertension (PH)?
Adriano_Tonelli,_MD: Symptoms of PH include worsening of the respiratory symptoms, edema, and dizziness or passing out. A higher need for oxygen is another presentation.
Batyah: Do you have any statistics on the prevalence of pulmonary hypertension in sarcoidosis?
Adriano_Tonelli,_MD: I do not have a statistic on its prevalence, but it is rare. It is hard to tell as there are many patients with sarcoidosis that have limited symptoms and remain undiagnosed.
Batya: What are the initial tests to confirm or exclude pulmonary hypertension (PH)?
Adriano_Tonelli,_MD: The initial test is a patient history and physical examination performed by a physician. If suspicion exists, the next step is an echocardiogram. If the echocardiogram is suggestive of the diagnosis, then PH is confirmed with a right heart catheterization.
Za4ck13: When you state "echo", do you mean "stress echo" or regular ”echo”?
Adriano_Tonelli,_MD: Echocardiogram Doppler. Stress echo is usually not regularly done in pulmonary hypertension (PH) patients unless there is suspicion of ischemic (coronary) artery disease. Rarely echocardiograms are done during exercise to see if the estimated right ventricular systolic pressure increases with activity (exercise-induced PH).
Batyah: Are there any blood tests that can point out that pulmonary hypertension (PH) may be occurring or help diagnose in the event that it’s unclear whether a patient has PH?
Adriano_Tonelli,_MD: There are limited studies that can be done at this point to help in the diagnosis. We order several blood studies to help determine the cause of pulmonary hypertension. The test that is frequently used to follow patients with PH is brain natriuretic peptide (BNP) or NT-pro BNP. This is a biomarker that helps determine if you have volume overload (excessive fluid).
gigiw7: Can a CAT scan offer any help with diagnosis?
Adriano_Tonelli,_MD: CT scan of the chest can reveal a dilated pulmonary artery that can suggest the diagnosis. However, this alone is not enough and further evaluation is needed. Some patients have PH related to lung diseases and these most of the time can be seen in a CT of the chest. In addition, patients with unresolved clots in the lung can be identified during a CT with contrast.
ssbdw: My heart catheterization in 2012 showed right ventricular systolic pressure of 41, and the echocardiogram (echo) was normal. Now the echo shows a pressure of 51. Should the right heart catheterization be repeated?
Adriano_Tonelli,_MD: The right ventricular systolic pressure estimated by echocardiogram is not very precise. It uses tricuspid flow to estimate a pressure and variations of more than and less than 20 mmHg are not uncommon. Thus, if everything else is OK, I would not worry very much about this increase. However, it is important to follow this with another echo in three months or so to see if there is a trend. Right ventricular function is more important that the actual pressure estimated by the echo.
ssbdw: How significant is a rise in right ventricular systolic pressure from 41 to 51 in one year?
Adriano_Tonelli,_MD: Echocardiogram estimated pressure varies significantly—by as much as more than or less than 20 mmHg or even more than that when this pressure is actually determined during a right heart catheterization. A variation of 41 to 51 may be within the error of the measurement. I would not consider this significant unless there is worsening in the size and function of the right ventricle. I agree that if the new echo shows a pressure of 61, then it is likely that there is an increase in pressure overtime.
donrobi: If you have higher pressure than the normal pressure of 25 in your right ventricle, how do you know if you have pulmonary hypertension (PH)?
Adriano_Tonelli,_MD: If your mean pulmonary artery pressure during a right heart catheterization is 25 mmHg or above, you do have pulmonary hypertension. If your systolic right ventricular pressure on an echocardiogram is 25 mmHg—this is in normal range. However, echocardiogram is not the best tool to exclude or include PH. Further investigation with a right heart catheterization is decided on a case-by-case basis.
TOMMYBOY: I have just had a right heart catheterization that confirmed pulmonary arterial hypertension (PAH). What can I expect my life expectancy to be?
Adriano_Tonelli,_MD: It would depend on a variety of factors. There is an online calculator based on the REVEAL registry that will be able to tell you life expectancy—the REVEAL calculator. However, none of these tools are perfect. Fortunately, there are 10 pulmonary hypertension medications available and most patients improve.
TOMMYBOY: How do I find the REVEAL registry?
Adriano_Tonelli,_MD: You can Google REVEAL calculator and pulmonary hypertension.
Joan: Can you go over the medications and classes for pulmonary hypertension (PH)—especially Tracleer® (bosentan), Viagra® (sildenafil citrate) and when to progress to the next medication?
Adriano_Tonelli,_MD: The classes of PH include:
- 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).
At Cleveland Clinic, our patients are on various advanced pulmonary hypertension therapies, such as intravenous epoprostenol (Flolan® and Veletri®), intravenous and inhaled treprostinil sodium (Remodulin® and Tyvaso®), oral bosentan and ambrisentan (Tracleer® and Letairis®), and oral sildenafil and tadalafil (Revatio® and Adcirca®).
There are two new medications in the market—Adempas® (riociguat) and Opsumit® (macitentan) that we also are starting to use. Progression of treatment depends on different factors, such as symptoms, distance walked, right ventricular function on echocardiogram, and so on.
Shalom: Can you tell us something about the new medications that were approved by the FDA and how they related to the present medications? Are they to be used instead of the present medications or to be added to the "cocktail"?
Adriano_Tonelli,_MD: Instead would be the answer. Opsumit® (macitentan) is an endothelin receptor blocker like Tracleer® (bosentan) and Letairis® (ambrisentan). Adempas® (riociguat) is a new type of medication that works on the nitric oxide pathway, like Revatio® (sildenafil) and Adcirca® (tadalafil). Therefore, they cannot be used together.
Shalom: I have idiopathic pulmonary arterial hypertension. I'm on Revatio® (sildenafil) and Letairis® (ambrisentan). How do the new medications recently approved by the FDA relate to the present medications that are available?
Adriano_Tonelli,_MD: Adempas® (riociguat) would have to be used instead of Revatio®. Opsumit® (macitentan) would have to be used instead of Letairis® (ambrisentan). It is unclear whether riociguat is equally effective, better, or worse than Revatio® (sildenafil) as no comparative studies have been done at this point. Similarly, it is unknown whether Letairis® is better than Opsumit®.
sybyla: What do you think about the new FDA approved pulmonary hypertension drug Adempas® (riociguat)?
Adriano_Tonelli,_MD: Adempas® is a promising medication especially for the group of patients with inoperable or residual chronic thromboembolic hypertension—a condition due to residual clots. For these conditions there were no FDA approved therapies.
Surgery and Pulmonary Hypertension
nutzy: What is your recommendation for a 60-year-old female who needs mitral valve surgery, but the doctor also discovered severe pulmonary hypertension (PH) of 80-85? Should I start medical treatment before the surgery?
Adriano_Tonelli,_MD: A patient with mitral valve disease commonly will have pulmonary hypertension. Usually the risks of surgery increase in the presence of advanced PH. There are no FDA-approved PH therapies for this indication. Some physicians will consider sildenafil. However, this needs to be done very carefully as some patients could get worse.
Exercise and Weight Loss for Pulmonary Hypertension
Za4ck13: Can losing weight and doing cardio (aerobic) exercise help with mild pulmonary hypertension.
Adriano_Tonelli,_MD: Yes. Weight loss and exercise increase your capacity to do more, almost as much as some oral pulmonary hypertension therapies.
Shalom: How do you know when you should "push" during exercise or if "pushing" might be dangerous?
Adriano_Tonelli,_MD: Exercise in progressive amounts and without excess is the best approach. Most patients join a rehabilitation program, so they can increase their activity in a supervised setting. The level of exercise would depend on how well controlled your pulmonary hypertension is.
Batyah: You mentioned weight loss and exercise to improve pulmonary hypertension (PH) or for PH symptoms. Can exercise and weight loss reverse pulmonary hypertension (PH) in someone who is 10 to 15 percent over the recommended weight?
Adriano_Tonelli,_MD: It depends on the type of PH. If the pulmonary hypertension is due to left ventricular diastolic dysfunction, then yes, it can be reversed by a significant weight loss and exercise. Other types of PH such as pulmonary arterial hypertension are not reversed by these interventions. Exercise and weight loss will help you to have less symptoms and feel better, but will not reverse it.
gigiw7: What type of exercise equipment would you recommend to use at home?
Adriano_Tonelli,_MD: Usually patients use a treadmill or stationary bike. Ideally, and depending on the type and severity of pulmonary hypertension (PH), patients start activities as part of a rehabilitation program and then they buy a machine that they use and like. I would not suggest starting exercise activities at home without the approval of your PH physician. Activities should be increased progressively and stopped if symptoms develop.
Shalom: Are medications being tested or developed that would eventually replace the intravenous and subcutaneous meds?
Adriano_Tonelli,_MD: Yes, there is a promising medication called selexipag by Actelion that is in phase 3 (large clinical trials). If approved by the FDA, it may potentially replace the intravenous and subcutaneous medications.
Finding a Specialist
TOMMYBOY: How do you find an expert?
Adriano_Tonelli,_MD: In general big institutions such as Cleveland Clinic have pulmonary hypertension (PH) teams that can certainly help you. The Pulmonary Hypertension Association has a website with physicians interested in PH. You can search by state and find physician treating the disease in your area.
Batyah: If pulmonary hypertension is suspected or confirmed, is it better to have it diagnosed, monitored, and treated by a pulmonologist or a cardiologist?
Adriano_Tonelli,_MD: Either a pulmonologist or a cardiologist can treat the disease. However, the disease is rare and ideally you need someone with experience in its treatment. The short answer is to find an expert in the treatment of the disease (cardiologist or pulmonologist).
Moderator: I am sorry to say that we are at the end of our chat. We appreciate your participation and hope you will join us for other chat topics in the future. Thank you, Dr. Tonelli, for sharing your expertise and answering questions today about pulmonary hypertension.
Adriano_Tonelli,_MD: Thank you for questions and interest in the disease. Fortunately research brought 10 medications to the market and likely more therapies will be available in the future. I wish you all the best.
To make an appointment with Dr. Tonelli or another Cleveland Clinic pulmonary hypertension specialist, call 216.445-5763 or toll-free at 1.800.223.2273 (ext. 55763), or email medical secretary Jessica Klinko at email@example.com.
For More Information
About Pulmonary Hypertension
Learn more about symptoms, causes, diagnostic tests and treatments for pulmonary hypertension and other pulmonary vascular diseases at:
On Cleveland Clinic
At 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.
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. At Cleveland Clinic, we offer patients various advanced pulmonary hypertension medication therapies and surgery for chronic thromboembolic pulmonary hypertension (CTEPH) with pulmonary thromboendarterectomy. Cleveland Clinic is one of the few centers in the country with expertise in this procedure.
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.
The pulmonary program at Cleveland Clinic Respiratory Institute is ranked third in the United States by US News & World Report.
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