Pulmonary HypertensionSymptoms, Doctors, Treatments, Advances & More
Learn About Pulmonary Hypertension
Pulmonary hypertension is high blood pressure in the pulmonary arteries of the lungs. It makes the right side of the heart work harder than normal.
Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension; Cor pulmonale - pulmonary hypertension
The right side of the heart pumps blood into the pulmonary artery and then through the lungs, where it picks up oxygen. Blood returns to the left side of the heart, where it is pumped to the rest of the body.
When the small arteries (blood vessels) of the lungs become narrowed, they cannot carry as much blood. When this happens, pressure builds up. This is called pulmonary hypertension.
The heart needs to work harder to force the blood through the vessels against this pressure. Over time, this causes the right side of the heart to become larger and malfunction. This condition is called right-sided heart failure, or cor pulmonale.
Pulmonary hypertension may be caused by:
- Autoimmune diseases that damage the lungs, such as scleroderma and rheumatoid arthritis
- Birth defects of the heart
- Blood clots in the lung (pulmonary embolism)
- Heart failure (of the left side of the heart)
- Heart valve disease
- HIV infection
- Low oxygen levels in the blood for a long time (chronic)
- Lung disease, such as COPD or pulmonary fibrosis or any other severe chronic lung condition
- Medicines (for example, certain diet medicines)
- Obstructive sleep apnea
In rare cases, the cause of pulmonary hypertension is unknown. In this case, the condition is called idiopathic pulmonary arterial hypertension (IPAH). Idiopathic means the cause of a disease is not known. IPAH affects more women than men.
If pulmonary hypertension is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension.
Shortness of breath or lightheadedness during activity is often the first symptom. Fast heart rate (palpitations) may be present. Over time, symptoms occur with lighter activity or even while at rest.
Other symptoms include:
- Ankle and leg swelling
- Bluish color of the lips or skin (cyanosis)
- Chest pain or pressure, most often in the front of the chest
- Dizziness or fainting spells
- Fatigue
- Increased abdominal size due to fluid buildup
- Weakness
People with pulmonary hypertension often have symptoms that come and go. They report good days and bad days.
There is no cure for pulmonary hypertension. The goal of treatment is to control symptoms and prevent more lung damage. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve problems.
Many treatment options for pulmonary arterial hypertension are available. If you are prescribed medicines, they may be taken by mouth (oral), received through the vein (intravenous, or IV), or breathed in (inhaled).
Your provider will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well you are responding to the medicine. Do not stop taking your medicines without talking to your provider.
Other treatments may include:
- Blood thinners to reduce the risk of blood clots, especially for some forms of pulmonary hypertension
- Oxygen therapy at home
- Lung transplant, or in some cases, heart-lung transplant, if medicines do not work
Other important tips to follow:
- Avoid pregnancy.
- Avoid heavy physical activities and lifting.
- Avoid traveling to high altitudes.
- Get a yearly flu vaccine, as well as other vaccines such as the pneumococcal (pneumonia vaccine), and the COVID-19 vaccine.
- Stop smoking.
Wakemed Specialists Group LLC
Kishan Parikh is an Advanced Heart Failure and Transplant Cardiologist and a Cardiologist in Raleigh, North Carolina. Dr. Parikh is rated as an Elite provider by MediFind in the treatment of Pulmonary Hypertension. His top areas of expertise are Pulmonary Hypertension, Pulmonary Veno-Occlusive Disease, Heart Failure, Hypertension, and Heart Transplant. Dr. Parikh is currently accepting new patients.
Brigham And Women's Hospital, Heart And Vascular Center
Dr. Aaron B. Waxman is the director of the Pulmonary Vascular Disease Program at Brigham and Women’s Hospital (BWH). He is also an associate professor of medicine at Harvard Medical School.After receiving his medical degree from Yale University School of Medicine, Dr. Waxman completed a residency in internal medicine at Yale New Haven Hospital. He then completed two fellowships: the first at Howard Hughes Medical Institute, and the second in pulmonary and critical care at Yale New Haven Hospital. He is board certified in critical care medicine, internal medicine and pulmonary disease.Dr. Waxman’s clinical interests include pulmonary arterial hypertension (PAH), right heart failure, thromboembolic disease and pulmonary vascular disease. His research focuses on the inflammatory mechanisms of pulmonary vascular remodeling that lead to right heart failure. His research into the regulation of cell death proteins by IL-6 cytokines in mice led to the discovery of a relationship between dyspnea and mitochondrial dysfunction, and resulted in additional clinical studies examining the abnormalities of inflammatory mediators and oxygen extraction in patients with PAH. He has authored over 110 peer-reviewed publications and has received funding from the National Institutes of Health and the National Heart, Lung, and Blood Institute. Dr. Waxman is rated as an Elite provider by MediFind in the treatment of Pulmonary Hypertension. His top areas of expertise are Pulmonary Hypertension, Pulmonary Veno-Occlusive Disease, Interstitial Lung Disease, Lung Transplant, and Thrombectomy.
Johns Hopkins Outpatient Center
Stephen C. Mathai, MD, MHS is an Associate Professor of Medicine and part of the Johns Hopkins Pulmonary Hypertension Program. Dr. Mathai received his undergraduate degree from Rutgers College and medical degree from Jefferson Medical College. He pursued residency training in Internal Medicine at Thomas Jefferson University Hospital, where he also served as Chief Medical Resident. Dr. Mathai came to Johns Hopkins for fellowship training in Pulmonary and Critical Care Medicine. During his 5-year fellowship, he obtained a Masters of Health Science degree from the Johns Hopkins Bloomberg School of Public Health and successfully competed for an individual Ruth L. Kirschstein National Research Service Award. Subsequently, he received a K23 award for his research in pulmonary vascular disease in scleroderma. He currently serves as Co-Principal Investigator for the Johns Hopkins PVDOMICS program supported by the NIH and is principal investigator for a clinical trial of therapy for scleroderma associated pulmonary hypertension supported by the Department of Defense Peer Reviewed Medical Research Program. He is also the Co-Director of the Ann Dana Kusch Multidisciplinary Program for Interstitial Lung Disease and Pulmonary Hypertension. Dr. Mathai has been active in numerous committees and holds several leadership positions in academic societies. He is the current chair of the Pulmonary Vascular Disease Network for the American College of Chest Physicians and has served on the steering committee for this organization for the past 4 years. He has served on several committees for the Pulmonary Circulation Assembly of the American Thoracic Society, including the Program Committee and Nominations Committee. He has organized symposia and delivered presentations related to pulmonary hypertension at numerous international meetings. He is the author of more than 100 publications and has given invited research lectures nationally and internationally. Dr. Mathai is also an active member of patient advocacy organizations. Currently, he is a member of the Scientific Leadership Council of the Pulmonary Hypertension Association where he serves as national chairperson of the Preceptorship Program Planning Committee. He is also a member of the Rare Disease Advisory Panel for the Patient Centered Outcomes Research Institute. Dr. Mathai also serves as the faculty leader of the inpatient pulmonary service, the Carol Johns Service, where he is responsible for the education of the internal medicine housestaff and pulmonary fellows. He has also received the Frederick L. Brancati Award for mentorship from the Department of Medicine at Johns Hopkins. Dr. Mathai is rated as an Elite provider by MediFind in the treatment of Pulmonary Hypertension. His top areas of expertise are Pulmonary Hypertension, Systemic Sclerosis (SSc), Pulmonary Veno-Occlusive Disease, Sarcoidosis, and Lung Transplant.
How well you do depends on what caused the condition. Medicines for IPAH may help slow the disease.
As the illness gets worse, you will need to make changes in your home to help you get around the house.
For people with severe disease, lung transplant, or in some cases, heart-lung transplant can be considered.
Contact your provider if:
- You begin to develop shortness of breath when you are active
- Shortness of breath gets worse
- You develop chest pain
- You develop other symptoms
- You feel lightheaded or dizzy
- You have persistent palpitations
Summary: Researchers are looking for new ways to treat people with a type of pulmonary hypertension called combined postcapillary and precapillary pulmonary hypertension (Cpc-PH). This study focuses on Cpc-PH that is caused by heart failure with preserved ejection fraction (HFpEF). Researchers want to know if the study treatment, sotatercept, can treat people with Cpc-PH caused by HFpEF. This is an extensi...
Summary: The investigators will study whether the drug tadalafil improves shortness of breath in 126 Veterans with Chronic Obstructive Pulmonary Disease (COPD) and high blood pressure in the lungs. The investigators will also assess whether tadalafil improves quality of life, home daily physical activity, exercise endurance, the frequency of acute flares of COPD, blood pressure in the lungs, and lung funct...
Published Date: May 03, 2024
Published By: Allen J. Blaivas, DO, Division of Pulmonary, Critical Care, and Sleep Medicine, VA New Jersey Health Care System, Clinical Assistant Professor, Rutgers New Jersey Medical School, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Lammi MR, Mathai SC. Pulmonary hypertension: general approach. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 83.
Maron BA. Pulmonary hypertension. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 88.