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Pulmonary Hypertension (PH)

Online Health Chat with Dr. Omar A. Minai

May 24, 2011


Introduction

Cleveland_Clinic_Host: Pulmonary hypertension is a rare and serious lung disorder. It occurs in individuals of all ages, races, and ethnic backgrounds -- although it is 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. As the disease worsens, it may become difficult to carry out any activities. 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.

Omar A. Minai, MD, FCCP, DABSM, is a Staff Physician in the Department of Pulmonary, Allergy, and Critical Care Medicine and in the Sleep Center and the Lung Transplant Center at Cleveland Clinic.

Dr. Minai’s main clinical and research interests include pulmonary hypertension, sleep disorders, advanced COPD, and lung transplantation. He has authored or co-authored more than 70 publications primarily related to advanced lung diseases such as pulmonary arterial hypertension, emphysema, and lung transplantation.

Dr. Minai is board-certified in the fields of Pulmonary medicine, Critical care medicine, and Sleep medicine. He is a Fellow of the American College of Chest Physicians, and a member of the American Thoracic Society and the American Academy of Sleep Medicine.

To make an appointment with Omar Minai, MD, or any of the specialists in the Respiratory Institute at Cleveland Clinic, please call 216.445.5763 or call toll-free at 800.223.2273, ext. 55763. You can also visit us online at my.clevelandclinic.org/lungs-breathing-allergy/default.aspx.

Cleveland_Clinic_Host: Welcome to our Online Health Chat with Dr. Omar Minai. We are thrilled to have him here today for this chat. Let’s begin with some of your questions.


morrison: What are the symptoms of pulmonary hypertension (PH)? Through a health study I was participating in for something else, I was told that I have borderline pulmonary hypertension. What does this mean and should I now be seen by a specialist?

Dr__Omar_Minai: Symptoms of pulmonary hypertension are somewhat non-specific and include shortness of breath, fatigue, dizziness, etc. However, there are several other diseases that cause these same symptoms that are more common in the general population than PH. This is where your physician has to perform a thorough evaluation to determine what is causing your symptoms. Borderline PH is not a standard term, but your physician is most likely saying that your pulmonary pressures are elevated. You should speak to your physician to find out what he meant by this; and if you have PH, you should be seen by a specialist to determine if treatment is needed or not.

Melonlady17: What is today's projection of life expectancy for those diagnosed with PH? I know it used to be three to four years, but I know so many PHers are living much longer, so I wonder if this had changed.

Dr__Omar_Minai: This is a great question. Survival projections depend on the underlying cause of PH and the degree of severity at presentation. It is different for all forms of PH and varies even within disease sub-type. It is clear that advances in medications and overall medical care has resulted in improved survival in patients with PH, however, there is not enough survival data on the available treatment options to make precise predictions in individual patients.


Diagnosis

jxk335: Is a diagnosis made by an echocardiogram accurate?

Dr__Omar_Minai: No it is not. Echocardiography (echo) is the best non-invasive screening test we have; however, it is not a good diagnostic test. Over the years I have seen many patients for whom the echo was incorrect either in making the diagnosis of PH or in determining its causative factors. Current PH guidelines recommend that all patients being considered for treatment for pulmonary hypertension absolutely have to have a right heart catheterization for accurate measurement of their pressures. Right heart catheterization also helps us make diagnoses that echocardiography can miss, and helps the physicians determine long-term prognosis, as well as to select appropriate treatment.

jxk335: Why is early diagnosis a good thing if there are no treatments and if you don't have symptoms?

Dr__Omar_Minai: Most patients who see their physicians do so because they have some early symptoms. So, it is very uncommon to find patients who have absolutely no symptoms. It has been shown that early treatment slows down the rate of disease progression and can improve hemodynamics and functional capacity in patients with PH. In a disease that is progressive, early treatment is better because it has a chance to be more effective.

jxk335: Should everyone with a diagnosis of pulmonary hypertension on echo, even if there are no symptoms, have a cardiac catheterization?

Dr__Omar_Minai: This is a very complex questions because the need for right heart catheterization will depend on several factors and is very individualized. The echo was likely done in response to symptoms and is the best non-invasive screening test we have; however, it is not a good diagnostic test. All patients with suspected PH should have right heart catheterization if medical treatment for PH is being considered. This is because right heart catheterization allows us to make an accurate diagnosis of PH, to make an accurate assessment of the type of PH, and helps the physicians determine severity of PH which impact long-term prognosis and selection of appropriate treatment. In patients with minimal symptoms, right heart catheterization should be done if the echo is abnormal and the patient has risk factors for PH such as scleroderma, etc. The need for right heart catheterization will also depend on the presence of co-morbid illnesses such as cardiac or pulmonary diseases.

harold: My sister is 40 years old and was recently diagnosed with PH. She was not able to keep her oxygen saturations in the 90s and is now on oxygen. She told me that her PH is considered mild, and she is not currently on any medications. What is considered mild PH? I don’t understand why it is mild if she is on oxygen.

Dr__Omar_Minai: The need for oxygen may not necessarily have a direct correlation with the severity of PH. This is because the need for oxygen depends on several other factors such as underlying concomitant cardiac or pulmonary diseases. So patients with ‘mild’ PH may need supplemental oxygen if they have concomitant cardiac or pulmonary problems. I would recommend talking to her physician, since this requires an answer based on your sister's specific issues.


PH Progression

donald: How long does it take for pulmonary hypertension to progress from an echo reading of 48 mmhg to the point where you can no longer walk for 10 minutes?

Dr__Omar_Minai: This is a question that requires further research. The rate of progression of PH depends mainly on the underlying etiology of PH. PH in a patient with idiopathic pulmonary arterial hypertension (PAH) is likely to advance faster than a patient with PAH due to congenital heart disease. However, even within these types, the rate of disease progression can be variable.

In addition, functional limitation and survival are not always directly linked to pulmonary pressures. So, patients with scleroderma may have shorter survival than patients with idiopathic PAH despite having lower pulmonary pressures. My suggestion would be to see someone who will do a thorough evaluation of the underlying disease and PH severity, and give a good idea of where one is based on individual evaluation.

BuffaloStach: How important is keeping ones oxygen saturation level at 95 or greater while fighting PH? Does a continued 95+ reading with O2 reflect some level of stabilizing the progression of the disease?

Dr__Omar_Minai: Several patients with PH may have low oxygen levels at rest, with exertion, or while sleeping. All patients with PH should be evaluated for hypoxia with exertion and with sleep, and current guidelines recommend keeping oxygen saturation at or above 90 percent. If this requires supplemental oxygen, then that should be given.


Treatment

jxk335: What is the treatment for class one idiopathic pulmonary hypertension (IPH)?

Dr__Omar_Minai: I am not sure what you mean by "class one pulmonary hypertension." The current guidelines divide pulmonary hypertension into five categories, and class one in these guidelines is “pulmonary arterial hypertension (PAH).” This includes patients with idiopathic PAH, hereditary PAH, and PAH associated with scleroderma, lupus, congenital heart diseases, and many other types. All these patients should be thoroughly evaluated; and if found to have PAH, should be treated with appropriate medications depending on the underlying cause and disease severity.

Severity of patients with PAH is assessed by the World Health Organization’s functional class, and there "class one" refers to patients who do not have any symptoms and have no limitation of their activity even with more than usual activity. There are currently no approved treatment recommendations for these patients in view of their being asymptomatic. Since PH is a progressive disease, these patients should be followed closely.

linda1717: What are the current treatments for idiopathic pulmonary fibrosis (IPF), and is this disease related to idiopathic pulmonary hypertension?

Dr__Omar_Minai: IPF can sometimes be associated with PH; however, this form of PH is very different than PAH, and management recommendations are also different. I would recommend asking your physician on how to proceed.


Medications

toohard: I have been taking Revatio® (sildenafil), but lately it does not seem to be having the same effect. Do these medications lose their effectiveness after taking them for a period of time? Should I be asking the doctor about considering other medications or treatments?

Dr__Omar_Minai: PH does progress over time despite medical therapy, so the medications that were working initially may not be as effective as time goes on. It would be reasonable to ask your physician what the other available options are. These will depend on the cause and severity of PH.

donald: I take Avapro® (irbesartan) for high blood pressure. Would switching to a calcium channel blocker reduce the hypertension in my lungs?

Dr__Omar_Minai: This is a great question. Pulmonary hypertension does refer to increased pressure in the lung blood vessels, but it is very different from "hypertension" that is increased blood pressure in the vessels of the rest of the body. So, while calcium channel blockers are effective in most patients with "hypertension" (high blood pressure), they are not effective in most patients with "pulmonary hypertension” (high pressure in the lung blood vessels). In fact, calcium channel blockers are effective in only approximately 5 percent of patients with "pulmonary hypertension" and are actually harmful in the rest. There is a specific test (called a ‘vasoreactivity test’) that should be performed during right heart catheterization that tells us whether or not calcium channel blockers will be beneficial. If that test was not performed or was performed and was negative, calcium blockers are more likely to worsen pulmonary hypertension symptoms and should not be used.


Transplantation

BuffaloStach: What are the critical factors patients must look at to know if they are ready for a lung transplant: six-minute walk? pulmonary pressures? ECG? A 2-lung transplant has been suggested by my physician, who will be recommending me to Cleveland Clinic for an evaluation. My current medications are Letairis® (ambrisentan) and sildenafil. A recent 6-minute walk revealed, in their opinion, that I am not ready for Ventavis® (iloprost) at this time. It is my understanding that Ventavis® is probably the last medication available to prolong my time. My December 10 catheterization pressures were 52. Is it too early to be placed on the transplant list? I heard of situations where Ventavis® has reduced the pressure considerably. Although I am committed to the process and the eventuality of a transplant, I am nervous that I do not jump into it prematurely. To that end, when does one make that choice?

Dr__Omar_Minai: This is a great question, although a complex one. I cannot make specific recommendations unless we have access to more detailed medical records and your history. However, I will try and address some of your concerns. When making a decision about lung transplant candidacy in patients with pulmonary hypertension, we take several factors into consideration. These include age, overall health status, co-morbidities, how advanced the disease is, and which medications have been used to treat the PH. In pulmonary hypertension, all factors, including hemodynamics -- as measured by right heart catheterization and the six-minute walk test -- are considered. From a patient perspective, I don’t think that you need to focus on individual numbers or test results. As a rule of thumb, if you are experiencing progressive symptoms and functional limitation despite escalating therapy, then it is time to be evaluated. If your physician feels that you should be evaluated for lung transplantation, then it is reasonable to do so. Lung transplant is a significant step; therefore, it is perfectly natural to be anxious about it. When you come to see us, we can discuss your specific issues and make recommendations.

BuffaloStach: What is the largest cause for post-transplant rejection?

Dr__Omar_Minai: Post-transplant rejection has several reasons. However, pulmonary hypertension patients have long-term outcomes post-transplant very similar to patients undergoing lung transplant for other diseases.

BuffaloStach: Do you perform initial preliminary reviews based on patients’ medical records without a visit, or is a visit mandatory?

Dr__Omar_Minai: We do perform initial reviews. However, specific recommendations regarding management usually require not just a review of the records but a face-to-face evaluation by the physician.

BuffaloStach: How can I arrange for a preliminary assessment based on medical records, a precursor to coming to Cleveland?

Dr__Omar_Minai: For Pulmonary Hypertension you can call 216.445.5763 to initiate a consultation for evaluation. For evaluation for lung transplantation, you would need to contact the Lung Transplant Center at Cleveland Clinic by calling 1.216.444.8282 or 1.800.223.2273, ext. 48282.


General Questions

TMitchellFMW: Is there a cause/effect relationship between pulmonary sarcoidosis and pulmonary hypertension?

Dr__Omar_Minai: Patients with pulmonary sarcoidosis may develop PH. However, all sarcoid patients with pulmonary involvement don't develop pulmonary hypertension. There are several reasons why this might occur. This may be the result of advanced lung fibrosis and the accompanying vascular damage. It may be due to hypoxia, or it may be due to direct vascular involvement in sarcoidosis. Sarcoid patients may also have cardiac involvement, which may also produce pulmonary hypertension.

fanjean4: Does pulmonary hypertension lead to congestive heart failure?

Dr__Omar_Minai: Congestive heart failure usually implies left heart failure with pulmonary congestion. While this may also be associated with PH, the usual recommendation is to evaluate the left-sided disease and optimize its management before considering PH-specific issues.

PH can produce right-sided heart failure as it progresses. Treatment of this should be based on the cause and severity of the PH.

Closing

Cleveland_Clinic_Host: I'm sorry to say that our time with Dr. Omar Minai is now over. Thank you again, Dr. Minai, for taking the time to answer our questions today about pulmonary hypertension.


More Information

To make an appointment with Omar Minai, MD, or any of the specialists in the Respiratory Institute at Cleveland Clinic, please call 216.445.5763 or call toll-free at 800.223.2273, ext. 55763. You can also visit us online at my.clevelandclinic.org/lungs-breathing-allergy/default.aspx .

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

If you need more information, click here to contact us, chat onlineor call the Center for Consumer Health Information at 216.444.3771 or toll-free at 800.223.2272 ext. 43771 to speak with a Health Educator. We would be happy to help you. Let us know if you want us to let you know about future web chat events!

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This chat occurred on May 24, 2011

This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. ©Copyright 1995-2011 The Cleveland Clinic Foundation. All rights reserved.