CLINICAL VIGNETTES - 2004 - Journal of General Internal Medicine
Dr. Gomberg-Maitland Explains The Complexities Of Diagnosing Pulmonary Hypertension
WASHINGTON (7News) — November is Pulmonary Hypertension Awareness Month, and Doctor Mardi Gomberg-Maitland of George Washington University Hospital dropped by the set of Good Morning Washington to discuss the condition.
"Pulmonary Hypertension is high blood pressure in the lungs," Gomberg-Maitland said. "It's a type of heart failure, from the high pressures in the lungs."
"Patients present with shortness of breath on exertion," she added. "It's not always asthma or trouble breathing or anxiety, it can be high pressure in the lungs." Unfortunately, pulmonary hypertension isn't quite as easy to diagnose as high blood pressure. "You can't put a blood pressure cuff around the lungs," Gomberg-Maitland shared. "Ultimately you need to measure the pressures from the inside."
The George Washington University Hospital has the only pulmonary hypertension comprehensive care center in Washington, DC. "What a comprehensive care center allows is a multidisciplinary team," she said. "In the hospital, you really need a team that understands the disease."
For more information or to schedule an appointment visit www.GWHospital.Com or call 888-4GW-DOCS.
Understanding Pulmonary Hypertension: Symptoms & Treatments
When most people hear the word hypertension, they think of high blood pressure. While not as common as high blood pressure, pulmonary hypertension, which affects the lungs, can be harder to diagnose, more challenging to treat, and, tragically, often comes with fatal consequences.
Dr. Talal Dahhan, Division Chief for Pulmonary & Critical Care at Baystate Pulmonary explains, "Nearly 3 to 8% of the world's population is affected by pulmonary hypertension (PH). A serious condition brought on by abnormally high pressure in the blood vessels between the lungs and the heart, most cases of PH cannot be cured. But with early detection and proper treatment, it is possible to improve symptoms and slow the disease's progression."
What is Pulmonary Hypertension?While the signs and symptoms of pulmonary hypertension are felt in the lungs, the cause starts in the heart. Dr. Dahhan explains, "In a healthy body, the heart does its job pumping blood around the body and delivering oxygen to organs and tissue. The oxygen gets taken up by the body and the blood cycles back to the heart. When all things are working well, the blood flows back into the heart and travels to the lungs via the pulmonary artery, picks up fresh oxygen, and the whole cycle begins again. However, in cases of PH, the walls of the pulmonary artery become stiff, damaged and narrow and the amount of oxygen going out to the rest of the body drops to dangerously low levels. Sensing the low levels of oxygen, the heart begins to pump harder to push oxygen-low blood to the lungs. Over time, the low level of oxygen in the blood and the added pressure on the heart can lead to serious complications, including heart failure."
What Causes Pulmonary Hypertension?There are a number of conditions and issues that can lead to PH. Once determined, the cause is used to classify a patient's condition by a Group number (Group 1 PH, Group 2 PH, etc.). This grouping information is later used to help identify appropriate treatment options.
Here's a look at the Groups and causes for PH:
Group 1 PH is due to pulmonary arterial hypertension (PAH), a condition in which the tiny arteries in your lung become thickened and narrowed. While PAH can occur due to unknow reasons (what physicians call idiopathic), some common causes include:
Group 2 PH is due to left-sided heart disease, the most common cause of PH in the United States, left-sided heart disease causes blood to back up in the heart, raising the pressure in the pulmonary arteries.
Group 3 PH is due to lung disease, including interstitial lung disease, chronic obstructive pulmonary disease (COPD), certain sleep apneas, pulmonary fibrosis, and emphysema, all of which reduce the flow of blood through the lungs and oxygen uptake.
Group 4 PH is due to blockages in the lungs and arteries, including clots and scars from chronic thromboembolic pulmonary hypertension (CTEPH).
Group 5 PH is due to other diseases and conditions, including blood disorders, kidney disease, inflammatory disorders, and metabolic disorders.
Dr. Dahhan notes that "PH can also be caused by a specific congenital heart called Eisenmenger syndrome. This occurs when there's a hole, or more than one, between the heart chambers, which causes blood to flow incorrectly in the heart, often increasing the blood flow and pressure in the pulmonary arteries."
No matter the cause, Dr. Dahhan notes that PH in all forms is a very serious condition and left untreated can be life threatening.
What Are the Symptoms of Pulmonary Hypertension?According to Dr. Dahhan, "PH symptoms aren't 'all of the sudden' type symptoms. They develop slowly over the years and are often like symptoms of common heart and lung conditions making them easy to miss."
Pulmonary hypertension symptoms include:
Dr. Dahhan adds, "Most people first recognize a problem when they become short of breath when walking. They may be fine on a flat surface but if there's an incline or they're going up stairs, they may find themselves suddenly out of breath. As the disease progresses, they may even feel out of breath when seated. Typically, that's alarming enough to drive someone to seek medical help."
How is Pulmonary Hypertension Diagnosed?As noted, diagnosing PH can be challenging as its symptoms often resemble those of other lung and heart conditions. In addition to performing a physical exam and reviewing your medical history, your doctor may perform the following:
Diagnostic Tests for raising index of suspicion if we have a possibility of Pulmonary HypertensionThe most definite test for diagnosing PH, right heart catheterization measures the blood pressure in the pulmonary arteries. This information identifies where the high pressure exists in your heart and arteries and the specific type (group) of PH the patient has.
What is the Treatment for Pulmonary Hypertension?While there's no cure for most forms of pulmonary hypertension, various treatments are determined by the type of PH. Treatment can help manage symptoms and improve quality of life.
Among the earliest treatments recommended to consider:The next course of treatment options are medications. "There are a number of medication therapies available to help with different types of PH," says Dr. Dahhan, "Depending upon the type of PH a patient has, we may recommend medications to relax or widen blood vessels and arteries, relax muscles in blood vessels, or to increase blood flow. These don't treat PH, but they can treat and help with complications from PH."
A last resort option, surgery may be recommended for patients with CTEPH, however, as Dr. Dahhan notes, not all PH patients are candidates for thromboendarterectomy. But there are procedures to consider in absence of CTEPH. Options include:
Living with pulmonary hypertension can be challenging, but with proper management and care, it is possible to maintain a good quality of life. "Early diagnosis and treatment are crucial," says Dr. Dahhan. "If you are experiencing persistent shortness of breath or other symptoms, reach out to your doctor to schedule an appointment as soon as possible. The more information you can bring to the appointment—how long have you had symptoms, what makes them worse or better, etc.—the easier it will be to identify if PH is behind your issue and the faster you can begin treatment."
Merck's Therapy For Rare Lung Condition Shown To Help Reduce Risk Of Death In Study
Signage is seen at the Merck & Co. Headquarters in Kenilworth, New Jersey
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(Reuters) - Merck said on Monday its drug, Winrevair, helped significantly reduce the risk of death in patients with a rare condition which causes high blood pressure in the lungs, months after it secured approval in the United States.
Winrevair was approved to treat pulmonary arterial hypertension (PAH), a progressive disorder caused by a constriction of arteries in the lungs, leading to symptoms such as shortness of breath, chest pain and dizziness.
Merck said its drug met the main goal of significantly reducing the time to disease worsening, lung transplantation or death in a late-stage study of 172 patients with advanced stages of the condition, already receiving background treatment.
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The condition affects about 40,000 people in the United States.
An independent data monitoring committee has recommended the study be stopped early but participants would be able to continue receiving the treatment, Merck said.
Winrevair, which costs around $238,000 annually, was the first treatment to secure approval from the U.S. Food and Drug Administration from a class of drugs, which target a type of protein called activin.
The drug brought in sales of $149 million for Merck in the third quarter amid concerns around bleeding risks in patients.
"We think Winrevair's efficacy outweighs safety risks in severe patients — whom it is likely going to be limited to," Oppenheimer analysts said in a note earlier this month.
(Reporting by Mariam Sunny in Bengaluru; Editing by Shailesh Kuber)
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