Edema formation in congestive heart failure and the underlying mechanisms
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."
When Faced With A Trying Situation, My Son Tries Harder
My 25-year-old son Cullen has been trying to deal with trying situations since he was a child.
For years he explained to both his parents and medical professionals that something more than asthma or anxiety was making it difficult for him to physically keep up with his friends. A correct diagnosis of long QT syndrome, a rare heart condition that affects the electrical system that controls the heartbeat, confirmed that he should not be participating in certain activities such as sports.
But trying times weren't anywhere close to over for Cullen. Even though he was taking beta blockers, which make the heart beat slowly and less forcefully, and not overexerting himself, he wasn't feeling any better.
Trying to survive as a PAH patientThanks to self-advocating and having parents and doctors who listened and were proactive, Cullen's concerns were eventually validated. At 8 years old, he was diagnosed with pulmonary arterial hypertension (PAH), and with it, life became more trying.
He was placed on triple therapy, including continuous intravenous Flolan (epoprostenol GM). PAH symptoms, medication side effects, and lugging around a Flolan pump connected to a central venous catheter (a thin, flexible tube that's inserted into a large vein to provide access to the heart) challenged Cullen throughout his childhood.
Surviving wasn't his only goal; maintaining a good quality of life was equally important. Cullen attended class as often as he could and earned good grades, whether from home or school. He had fun with friends by partaking in activities that wouldn't overexert him. He graduated from eighth grade on time and looked forward to his future. Life was altered but still livable.
As hard as he tried, Cullen couldn't win his fight against PAH without having a heart and double-lung transplant, which finally happened in 2014. The surgery was a great success, and with a lot of trial and error, his life since then has been a success, too.
He tried to attend high school, but his lungs developed bronchiolitis obliterans (scarring and inflammation), which can lead to chronic organ rejection. His battle with this and extracorporeal photopheresis treatments kept him home and taking classes online.
Cullen graduated from high school thanks to determination and a never-stop-trying attitude. His future looked bright.
Cullen Steele enjoys life on campus. (Photo by Colleen Steele)
Giving it the old college tryDue to ongoing health struggles post-transplant, Cullen decided to attend community college close to home in Washington state.
When the COVID-19 pandemic hit, he was likely more prepared to deal with it than others were. He transferred to online classes at Southern New Hampshire University so that he could continue his education. He excelled, and when he felt it was safe for him to physically return to class, he applied and was accepted to the University of Washington (UW).
But the most trying times were yet to come. Cullen's dad, Brian Steele, passed away in April 2023. Cullen made the difficult decision to decline his acceptance to UW and move to New Jersey with me where I would be caring for my aging parents.
"If I were a 'normal' person, I would have been more crushed about giving up the UW, but I'm used to trauma and making adjustments," Cullen admitted. He applied and was accepted to Immaculata University in Pennsylvania, with a scholarship.
He decided to give residence life a try and was accommodated with a private dorm room and bath to limit exposure to airborne illnesses. Professors provide more one-on-one education and are understanding when Cullen misses classes due to medical appointments or health challenges. Attending a smaller university has its benefits.
A trying life leads to a career in social workCullen's focus is on medical social work, preferably for transplant patients, with a minor in psychology and a double major in social work and sociology. He is a unique student because he's faced difficult life experiences and learned many lessons from each trying situation.
He is used to working hard and making sacrifices. Medical terminology has been in his vocabulary since he was a child. He's lived through trauma and the reality that not all patients survive. As dark as that lesson has been, it will have a compassionate effect on how he handles his responsibilities as a social worker. Cullen places importance on treating the whole person — mind and body — but also on supporting family members who might be struggling as their loved one navigates a life-threatening disease or life post-transplant.
"My approach won't be doom and gloom, but I am prepared to be there for patients and their loved ones if something goes wrong," Cullen said.
Are you or a loved one living with PH, dealing with complications post-transplant, or facing the decision to have one? Don't stop trying because of a trying life. Use these experiences as lessons to help you move forward and accomplish your goals.
Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to pulmonary hypertension.
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