Online Health Chat with Raed A. Dweik, MD
Monday, September 15, 2014
Pulmonary hypertension (PH) 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 sensations), 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 eventually become difficult to carry out any activities.
Appropriate diagnosis and analysis of the problem are 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.
About the Speaker
Raed A. Dweik, MD, is Professor of Medicine and Director of the Pulmonary Vascular Program in the Department of Pulmonary and Critical Care Medicine in the Respiratory Institute at Cleveland Clinic. He is board certified in internal medicine, pulmonary disease and critical care medicine. Dr. Dweik's clinical interests are pulmonary hypertension, asthma, critical care and chronic beryllium disease. He has a joint appointment in the Department of Pathobiology in the Lerner Research Institute (LRI) and is Professor of Medicine at the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University.
Dr. Dweik's research interests are in exhaled breath analysis and the role of nitric oxide in cardiopulmonary physiology and disease, especially pulmonary hypertension and asthma. He served on several related national and international panels and committees including: the American Thoracic Society (ATS) Chronic Beryllium Disease Diagnosis and Management Committee, the ATS Taskforce to Standardize Measurement of Nitric Oxide (NO) in Exhaled Breath and the Joint ATS-ERS Taskforce on Exhaled Breath Condensate (EBC). He also chaired the ATS committee writing the guidelines for the use and interpretation of exhaled NO (FENO) in clinical practice and the ATS committee on pulmonary hypertension phenotypes.
Let’s Chat About Pulmonary Hypertension
Moderator: Welcome to our chat today with Cleveland Clinic pulmonologist Raed Dweik, MD. We are thrilled to have Dr. Dweik available to share his knowledge and expertise about Pulmonary Hypertension.
Tree: I have been diagnosed with idiopathic PH. My echo was 63 and cath was 36. I have been taking Revatio® for three weeks. My breathing is worse. I have been coughing up green phlegm for over two years. I do not have a chronic cough and never smoked. Do you believe I have an underlying condition and could possibly have secondary PH?
Raed_Dweik,_MD: While your echo and cath numbers do suggest PH, coughing up green phlegm is not part of pulmonary hypertension. I encourage you to see your physician for an x-ray to determine if anything else is going on.
lilred67: With a sustained pulmonary pressure of 160, how much higher can this pressure get without being fatal?
Raed_Dweik,_MD: I am not sure to which number you are referring, but I assume it is derived from an echo. 160 is certainly very high, but echo numbers are usually estimates and need to be confirmed by actual measurements on a right heart catheterization.
Ed95: My 85-year-old mother was recently diagnosed with pulmonary hypertension. She has always been an extremely active person and still teaches line dancing classes several times a week. She refuses to believe that age means you slow down. However, her shortness of breath from the pulmonary hypertension is giving her a run for her money. She has recently had an echo/stress test, as well as a right heart catheterization, which showed elevated pressure on both sides of her heart. She is currently taking 40mg of Lasix and 20mg of Revatio (which doesn't seem to be helping.) My question: She complains of a pressure above her stomach (below her lungs) that she swears is the cause of her shortness of breath. She feels like something is in the way and causing her the breathing difficulty. Is this a symptom of pulmonary hypertension or somehow otherwise related? Also, what else might her doctor try to alleviate her symptoms?
Raed_Dweik,_MD: The symptoms you describe are non-specific but they can certainly be due to PH. Once patients are started on treatment, it would be useful to monitor for a response. That can be done simply by a six-minute walk or echo, but sometime requires invasive testing with a right heart cath. This will help determine if additional therapy is needed.
AnnieD: I have been told that I have "mixed" PH. Is there a way to determine the main cause of my PH? I have PAH (pulmonary arterial hypertension), left diastolic dysfunction and pulmonary fibrosis, all secondary to scleroderma? Does knowing the main cause matter in the treatment?
Raed_Dweik,_MD: Our approach is usually to address all underlying and associated causes of PH. Each one is treated differently. A key question to answer is whether you have pulmonary 'arterial' hypertension. In patients with multiple etiologies, we try to determine if the pulmonary hypertension is out of proportion to left heart disease or lung disease. This will help determine if you will benefit from specific PAH therapy.
ckilpatr: What specific type of testing should siblings/cousins have when a child in the family has been diagnosed or died of pulmonary hypertension? In my situation, my 4-year-old nephew died of pulmonary hypertension in 2000 and was not diagnosed until after death. Thank you so much for your consideration.
Raed_Dweik,_MD: If any family member has symptoms suggestive of PH such as shortness of breath, leg swelling, chest pain or passing out, they should certainly seek medical attention immediately. Familial and heritable pulmonary hypertension is a well-recognized entity. There are genetic tests available. Genetic counseling would be the way to start to determine if any family members would benefit from genetic testing.
anniep: I was wondering if you would go over the diagnostic process, what tests, etc.; as you know, symptoms overlap. I have been told I have mild PH. I am currently taking fludrocort and doing flex exercises before getting out of bed and wearing compression stockings during the day, prescribed by an electrophysiologist. My heartbeat continues to be bothersome along with breathlessness. I am dizzy or lightheaded, too. I have COPD, a repaired atrial septal defect and have gained weight with the steroids. My blood pressure is not real high or low, a mixture. I'd be interested in knowing about nutrition and other self-help things, exercise, supplements. Thank you for offering this program!
Raed_Dweik,_MD: Having cardiac defects in general is a risk factor for PH, even after they are repaired. There are many tests we do in the process of evaluating for PH, but we usually start with an echocardiogram. If that suggests PH, further testing is done to confirm the diagnosis and its severity and to find out about any associated or contributing conditions.
annco: Is a treatment available for pulmonary hypertension if you also have idiopathic pulmonary fibrosis? My doctor is doing nothing at this time.
Raed_Dweik,_MD: There are certainly treatments for pulmonary hypertension. If you have underlying lung disease, however, we only treat if it is out of proportion to the underlying disease, meaning not fully explained by the lung disease. There are no specific medications approved for PH due to lung disease.
swamimami: Could you discuss specific treatments for pulmonary venous hypertension, including medications. Thank you.
Raed_Dweik,_MD: Pulmonary venous hypertension usually refers to a cause from the left side of the heart, and treatments are usually focused on that. This usually involves diuretics, ACE inhibitors, beta blockers, etc. PAH-specific therapy is reserved to times when the PH is deemed out of proportion to the left heart disease.
Tree: I have been diagnosed with PH and the pulmonologist gave me meds that are not helping. What are my next steps? I've had all the tests. Do I just continue the meds and the oxygen?
Raed_Dweik,_MD: You may want to consider a second opinion from a PH center. We would be happy to see you here at Cleveland Clinic.
LucyJ: Since Viagra and Cialis are used as a therapy, and I noticed a commercial on TV about NO pills being used to "enhance", how helpful are they or is it with PAH patients?
Raed_Dweik,_MD: Nitric oxide deficiency is a main cause for pulmonary hypertension. Several medications used to treat PH are based as you mentioned. Inhaled NO as a gas is used in the catheterization lab to determine response to certain therapies. The pills you describe, however, have not been studied in PH and are not regulated. It may be dangerous to take them with or instead of other PH therapies.
OUengr: Can pulmonary hypertension be caused by the heart's tricuspid or mitral valve? I have malfunctions (leakage) in both. Ref: I had a pericardiectomy at Cleveland Clinic in October 2013, but a valve replacement was not recommended at that time.
Raed_Dweik,_MD: Valvular heart disease (stenosis or leakage) is a common cause of pulmonary hypertension. The best treatment is usually to fix the valve, if possible. If replacement was not recommended, you may need close follow up.
Sam78: Can mitral valve prolapse develop over a long time with pulmonary hypertension?
Raed_Dweik,_MD: PH is one of the serious but rare complications of MVP.
lilred67: I have PH with a PDA (patent ductus arteriosus). My pulmonary pressure is 160. What risks are there with general surgery?
Raed_Dweik,_MD: Patients with PH are at high risk when they undergo anesthesia and surgery. A serious discussion has to occur about the risks and benefits of the procedure. If the procedure is deemed absolutely necessary, the patient needs to be adequately treated and be closely monitored before, during and after surgery. We recommend that this be done at a specialized PH center.
mothersheart: I have pulmonary fibrosis secondary to NSIP (nonspecific interstitial pneumonia). Now my heart is enlarged and I have mild PH. My sats are dropping into the 60s and 70s when I ambulate. I'm on O2 at 12L oxy mask or 6L oxymizer high flow nasal cannula. My question is: Is this a part of the fibrosis, and how much organ damage am I doing by allowing the sats to drop so low? I do recover into the 90 percents within about three minutes of sitting or lying down. Thank you.
Raed_Dweik,_MD: What you are describing can certainly be explained by having pulmonary fibrosis. It is possible that you also have PH on top of that. You would need a right heart catheterization to confirm or rule out this possibility.
AnnieD: Have there been any advances in the treatment of PH secondary to scleroderma?
Raed_Dweik,_MD: Excellent question. There are new guidelines for PH treatment in general, which include scleroderma, although nothing specific to PH associated with scleroderma is new.
AnnieD: Are there any new understandings of PH, treatments or discoveries that you are excited about or you think hold great promise?
Raed_Dweik,_MD: There are many exciting things happening in the PH field. Several new medications have recently been approved. We have an annual meeting at the Clinic to update doctors about recent advances. PH patients and their families are always welcome to attend free of charge.
LucyJ: When a woman has a child and typically the second child, and then gets PAH, is there a "name" for that other than IPAH? Is it caused by the birthing process?
Raed_Dweik,_MD: While pregnancy does not usually cause pulmonary hypertension, it can certainly exacerbate the disease. We strongly recommend that women who have PH not get pregnant. To determine if a disease is idiopathic, further testing into possible underlying or associated causes needs to be done first.
kasperek: I have not been diagnosed with pulmonary hypertension but have had an x-ray of my chest because of PVC's that were occurring. He said the x-ray showed a small nodule on my right lung 2 mm. Several months later, I had another x ray that showed nothing. Should I have a CT scan done? I do not have shortness of breath, but have always had irregular heartbeats. Why would one show a nodule and another show nothing? Is there a reason to be concerned? I am 66 years old. Thank you for your time.
Raed_Dweik,_MD: Nodules can be caused by multiple reasons including transient infection. They could also be due to a confluence of shadows on the film. If you are still worried about having something there, and especially if you smoke or smoked in the past, a CT scan give a more detailed view and can resolve some of these questions.
Moderator: I am sorry to say that our time with Cleveland Clinic pulmonologist, Raed Dweik, MD, is now over. Thank you for sharing your expertise and time to answer questions today.
Raed_Dweik,_MD: Thank you for joining us today. We will now be concluding today's Health Chat on Pulmonary Hypertension.
To make an appointment with Dr. Dweik, or any of the other specialists in Cleveland Clinic’s Respiratory Institute, please call 216.445.5763 toll-free at 800.223.2273 (extension 55763) or visit us at clevelandclinic.org/pulmonaryhypertension for more information.
For More Information
On Cleveland Clinic
The pulmonary program at Cleveland Clinic Respiratory Institute is ranked third in the United States by U.S .News & World Report. 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 find it important to 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. This enables us to provide the most comprehensive care for our patients and the best treatment options available for pulmonary hypertension.
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