Evaluation and Management of Right-Sided Heart Failure: A Scientific Statement From the American Heart Association
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."
Patients With PAH Who Get Sepsis Experience Care Differences
New data suggests clinicians may be slower to recognize sepsis in people with pulmonary arterial hypertension.
Patients with pulmonary arterial hypertension (PAH) who develop sepsis are treated differently from patients without PAH, in part because it takes physicians longer to identify sepsis, according to a new review.
The findings are based on a decade's worth of data from two academic hospitals. The study was published in BMC Pulmonary Medicine.
Alexander Sherman, MD, of the University of California Los Angeles David Geffen School of Medicine, explained along with colleagues that sepsis is the second most common cause of in-hospital death among people with PAH.
A 2020 study found that while mortality among people with PAH who were admitted to the hospital for a non-cardiac primary cause, the overall mortality rate was 6.9% (P < 0.001). However, among patients with PAH hospitalized due to sepsis, the mortality rate was 25.0%.
Sherman and colleagues said several cardiac and pulmonary implications of sepsis, including decreased right ventricular preload, increased right ventricular afterload, and reduced contractility, are already exacerbated in people with PAH. Yet, they said current management guidelines for PAH are based on expert opinion rather than clinical data. That lack of data can be particularly challenging with regard to fluid management, they said. Fluid resuscitation is typically a first step toward restoring preload in patients with sepsis. Yet, doing so risks worsening a patient's cardiac function.
"[R]ight ventricular volume overload can lead to impaired cardiac output, with catastrophic implications for the pulmonary and systemic circulations," they said.
In an effort to build the database of available information, Sherman and colleagues decided to look back at the cases of 30 patients with pre-existing PAH who were admitted to either of two academic hospitals for sepsis between the years 2013 and 2022. They constructed a cohort of 96 matched controls with which to compare the PAH group.
They found that concerns about fluid management appeared to impact patient care. The control group received significantly more fluids during the first 24 hours of care compared to people with PAH (median 0 mL v. 1216 mL, P < 0.001). People with PAH, on the other hand, were more likely to receive vasoactive medications (23.3% vs. 8.3%; P = 0.037).
"The 2021 Surviving Sepsis Guidelines recommend fluid resuscitation as first-line in patients with sepsis and organ hypoperfusion or septic shock, yet PAH patients with hypotension in this study frequently received pressors as the first treatment rather than IV fluid," Sherman and colleagues noted.
They said their results, along with other recent evidence, suggest that systematic fluid restriction may be harmful, though they said it is also important that hemodynamic evaluations be made on an individualized basis.
When patients with PAH waited longer to receive fluids, they also waited longer to receive antibiotics, the investigators said, suggesting clinicians may have been slow to identify sepsis. People with PAH also tended to stay in the hospital longer (mean ± SD, 31.6 ± 48.5 days vs. 17.5 ± 30.7 days, P = 0.127).
Moreover, 30-day mortality was notably higher among people with PAH (23.3% v. 13.5%, P = 0.376). Patients with PAH who died tended to be older and have higher Charlson comorbidity indices compared to those who served, the investigators found. Those who died were also more likely to need high flow nasal cannula within 24 hours, and to have lower heart rates and temperatures upon admission.
Sherman and colleagues said they believe theirs is the first study to look at PAH and sepsis alongside right heart catheterization data that could validate PAH diagnosis. Prior research, they said, relied on large administrative databases that lacked objective confirmation of PAH. They said their findings make clear that the existing research gap is leading to differential care.
"Further research is needed to help guide decision-making around early fluid management in patients with PAH presenting with sepsis," they concluded.
After 25 Years, I'm Still Absorbing Lessons From Chronic Illness
When I was in college and my health was in decline with pulmonary hypertension, I had a month when my symptoms became exacerbated. That led to a week of things becoming noticeably worse, which led to a day of increasing worry.
By 9 at night, I felt I had to tell my mom, mainly because I was afraid of what might happen if I went to sleep. My symptoms had been building for a while, and they'd hit the point where I genuinely felt scared for my well-being. Half an hour later, we were in the car and headed to the emergency department (ED).
Throughout my years of chronic illness, I've had countless times when I've attempted to ignore my body's warning signs — despite being a typically rational person. I can think of many reasons why. Sometimes it's been fear of what answers I'll get. Sometimes it's because I had too much happening in my life to give those warnings the attention they deserved. And sometimes it was because my symptoms had been worse before, making it hard to worry about anything less severe.
Unfortunately, just as I experienced in college, ignoring my body's messages can lead to a frightening turning point. That can happen quickly for those of us living within a fragile body. As a result, I'm constantly learning that it's better to address a problem before it gets out of control.
Back to the emergency departmentJust this past week, I ran myself into the same scenario. A symptom that'd been lingering for about a month took a sudden concerning shift, and after a difficult day, I was on that same track back to the ED.
I know it's an issue I could've addressed sooner. But in this case, despite feeling somewhere outside of my baseline, I wasn't experiencing enough impact on my day-to-day life for me to put forth the proper concern. In my head, this specific issue would work itself out.
This reasoning might sound foolish to some, but I don't think it's hard to understand how exhausting it can be to investigate an unfamiliar symptom. Most people in the chronic illness community can attest to being worn down by the repetition of similar scenarios throughout a lifetime.
This situation was no exception. My 32-hour stay in the ED was exhausting, but it led to an answer that didn't worry me and a reasonably straightforward treatment plan. From my experience, that seems to be how most health issues are resolved. Of course, a symptom in some cases is a sign of a bigger problem than expected. But for the most part, answers can be found, and solutions exist.
It's frustrating to have to learn this lesson over and over. I sometimes feel it's unfair that I have to deal with health setbacks at all, which is perhaps another reason why I tend to push myself to the limit. But sitting in the ED the other day, feeling very unwell, the typical thoughts came to mind. Had I waited too long? Would I be in the hospital for the holiday? Did my negligence contribute to the problem?
Fortunately, I'm home now and feeling confident that I'll improve with the care plan my doctors have put into place. But it's a sharp reminder that this task of living in a chronically ill body takes a great deal of balance. I try to avoid panic and excess worry, but the lesson I'm constantly relearning is that it's best not to push things to their breaking point. After all, if I take the initiative to deal with an issue, time has proven that, more often than not, things will be all right.
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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