Conditions Mistaken for Heart Failure
Pulmonary Artery Aneurysm In Longstanding Idiopathic Pulmonary Arterial Hypertension
Figure
Computed tomography scan of the chest with contrast medium in the soft tissue window in a frontal plane. Red circle: main stem of the left coronary artery and proximal portion of the left anterior descending artery. Black asterisk: ectatic pulmonary trunk
A 54-year-old female patient with a known 20-year history of idiopathic pulmonary arterial hypertension (IPAH) presented as an emergency with typical angina pectoris symptoms as well as increasing dyspnea on exertion 5 years after her last outpatient appointment. In the absence of signs of myocardial ischemia in laboratory tests and on electrocardiography, chest imaging with computed tomography revealed an ectatic pulmonary trunk (Figure) with a maximum diameter >10 cm, prompting the suspicion that this was compressing the main stem of the left coronary artery. Coronary angiography with intravascular ultrasound was performed, ruling out coronary heart disease and main stem compression. Since angina-pectoris symptoms can also be caused by IPAH, we ultimately deemed this to be the cause of our patient's complaints. Following treatment optimization, the patient subsequently presented symptom-free. With an incidence of 2–5/1 000 000, the 5-year survival rate is approximately 50% with vasodilator treatment, depending on concomitant diseases. There are a number of case reports of pulmonary artery aneurysm in pulmonahypertension, which are seen increasingly often given the increasing life expectancy. The most important complications include dissections or ruptures, as well as compression of the bronchial system and/or the main stem of the left coronary artery.
Christopher Alexander Hinze, Dr. Med. Da-Hee Park, Dr. Med. Benjamin-Alexander Bollmann, Klinik für Pneumologie, Medizinische Hochschule Hannover, hinze.Christopher@mh-hannover.De
Conflict of interest statement: The authors state that no conflict of interest exists.
Translated from the original German by Christine Rye.
Cite this as: Hinze CA, Park DH, Bollmann BA: Pulmonary artery aneurysm in longstanding idiopathic pulmonary arterial hypertension.Dtsch Arztebl Int 2023; 120: 890a. DOI: 10.3238/arztebl.M2022.0404
Lung Scarring Symptoms And Causes
Scars on the lung tissue cause it to thicken and lose elasticity. Doctors refer to lung scarring as "pulmonary fibrosis". It can occur due to certain health conditions and exposure to hazardous materials.
Lung scars can result from illness or medical treatment, and they are permanent.
Small scars may not cause noticeable symptoms, but extensive scarring can make it hard for a person to breathe, as it can affect the transfer of oxygen into the bloodstream. As a result, the brain and other organs may not receive the oxygen that they need.
Idiopathic pulmonary fibrosis (IPF) is a long-term condition in which lung scarring becomes gradually worse. It can be life threatening.
In this article, we look at why lung scarring happens and discuss some of the treatment options available.
Several conditions can cause scarring of the lungs.
Interstitial lung diseaseInterstitial lung disease involves inflammation of the air sacs or the web of tissue (interstitium) that surrounds them in the lungs. The inflammation can sometimes cause scar tissue to build up within the lungs, resulting in fibrosis.
IPF is the most common type of interstitial lung disease. If doctors describe a condition as idiopathic, this means that they do not know exactly what causes it.
IPF typically appears between the ages of 50 and 70 years. There are up to 207,000 people affected in the United States, and about 58,000 new cases are diagnosed each year.
Other causes and risk factorsOther risk factors for interstitial lung disease include:
In some cases, there may be a family history of the disease.
Some people experience exacerbations, during which symptoms suddenly worsen. These last for a while and then resolve. Exposure to triggers, such as tobacco smoke, may cause a flare.
The scarring of lung tissue makes it thick and stiff. As the lung tissue thickens, it becomes increasingly difficult for the body to transfer oxygen from the lungs into the bloodstream. As a result, the brain and other organs may not receive enough oxygen.
The symptoms depend on the amount of scarring and how much of the lung it affects, but they may include:
Scarring may also increase the risk of lung cancer.
Idiopathic pulmonary fibrosisThe symptoms of IPF may not be noticeable at first, but they tend to develop and worsen over time, making it increasingly difficult to breathe.
Eventually, pulmonary hypertension or respiratory failure can develop, both of which can be life threatening because they prevent oxygen from reaching the body's organs.
Many factors can lead to a cough or breathlessness, and many conditions can result in lung scarring.
Anyone who has concerns about breathing symptoms should seek medical help. A doctor will carry out a physical examination.
They will ask the person about:
After this, the doctor may carry out some tests, such as:
They may also carry out specific tests to rule out other conditions, such as TB.
As with scars on the skin, scars on the lung are permanent. It is not usually possible to remove them. The lungs are resilient, however, and small scars often do not cause any adverse effects.
Proper diagnosis and monitoring of the scars are key to treatment.
Scars that remain unchanged fortwo years or more are not usually a cause for concern. However, if they spread, they could indicate pulmonary fibrosis.
Idiopathic pulmonary fibrosisCurrently, a lung transplant is the only cure for IPF. However, a doctor can prescribe medications to help slow the progression of the disease and prevent flares. Pirfenidone (Esbriet) and nintedanib (Ofev) are two drugs that have proven effective.
As the disease progresses, the symptoms can become more severe. They can also worsen during a flare. In these cases, a person may need to spend time in the hospital. Treatment will include supplemental oxygen to support the person's breathing.
Occasionally, a doctor may recommend a transplant. This procedure can cure the condition, but the person will need to take medications for the rest of their life.
Lifestyle tips that may help prevent or relieve symptoms include:
It is important to talk to a doctor before making any significant lifestyle or dietary changes. A doctor can advise on a suitable exercise regimen, for example.
The outlook for pulmonary fibrosis varies among individuals and depends partly on a person's age and overall health. The person will need regular assessments.
In the past,most people diagnosed with IPF had a life expectancy of 3–5 years.
However, new medications may slow the progression of the condition and reduce the risk of death in the first few years after diagnosis. Doctors hope that the outlook will continue to improve.
Below are some commonly asked questions about lung scarring.
Is scarring of the lungs serious?Yes, scarring of the lungs, also known as pulmonary fibrosis, may be a serious condition.
When lung tissue becomes damaged and scarred, it leads to stiffness in the lungs and makes it difficult for them to function properly.
This can result in symptoms such as shortness of breath, persistent dry cough, fatigue, and, in severe cases, respiratory failure.
How are scarred lungs treated?Treatment options for scarred lungs, particularly in the case of IPF, often involve a combination of medication, lifestyle changes, and supportive therapies.
Two medications that have shown effectiveness in slowing down the progression of scarring of the lungs are pirfenidone and nintedanib.
Can a person live 20 years with pulmonary fibrosis?People with pulmonary fibrosis experience disease progression at different rates. Some people progress slowly and live with the condition for many years, while others decline more quickly.
Ultimately, it is a serious disease but research advancements are being made all the time that allow people to live longer and have a better quality of life.
What is the life expectancy of someone with ILD?Interstitial lung disease (ILD) is an umbrella term used for a large group of diseases that cause scarring of the lungs.
Therefore, a person's life expectancy can vary widely depending on several factors, including the specific type and severity of the ILD, their overall health, whether they smoke, and how well they respond to treatment.
Pulmonary fibrosis, or lung scarring, causes thickened and less elastic lung tissue.
Risk factors include having a previous viral infection or pneumonia, gastroesophageal reflux disease (GERD), smoking, and aging. Alternatively, the condition can be idiopathic – meaning the exact cause is unknown.
Diagnosis entails tests like X-rays and CT scans, with treatments encompassing medication to decelerate progression. Lifestyle modifications, such as stopping smoking and weight management, may also aid symptom management. In more severe cases, a lung transplant may be necessary.
Despite a traditionally restricted life expectancy, these advancements in medications give hope for better outcomes.
Pulmonary Hypertension: A Dangerous Condition
High blood pressure in the lungs, known as pulmonary hypertension (PH), is a dangerous condition that disproportionately affects women. While experts have long recognized this disparity, very little is known about why it exists. Physician scientists at National Jewish Health are uncovering the mysteries behind PH in women and finding solutions from every angle, from prompt and accurate diagnoses to targeted treatments and disease management to the development of new therapies that may ultimately halt disease progression and save lives.
While PH can be caused by a wide range of conditions it can also manifest without any known cause, and its symptoms are vague and broad, such as fatigue and shortness of breath, making diagnosis a challenge. Many women go years with a misdiagnosis of things like asthma or anxiety, while pulmonary hypertension is only causing more damage to blood vessels and becoming more difficult to treat.
"It's a disease that requires input from multiple specialties, so we have built a comprehensive program where pulmonologists work together with cardiologists, rheumatologists and radiologists to thoroughly screen each patient, provide an accurate diagnosis and offer every available treatment," said Patricia George, MD, director of the Pulmonary Hypertension Program at National Jewish Health.
Through this comprehensive program, clinicians are able to give patients concrete answers and effective treatment, but there are still many lingering questions about exactly why PH develops so often in women and how to target its biological causes.
"We can ask specific questions based on what we see in the clinic, and then take these questions to the lab and try to design experiments to answer them," said Tim Lahm, MD, director of pulmonary vascular research at National Jewish Health. "At the same time, we can take the discoveries we make in the lab to the clinic to offer patients cutting-edge knowledge and therapies."
It's this bench-to-bedside approach that helps patients like Kira Cronk, who suffer from the most dangerous form of PH, pulmonary arterial hypertension (PAH).
"I was experiencing symptoms that prevented me from living a normal life for a couple of years before I had a diagnosis. Even just trying to climb a flight of stairs, I'd have to stop halfway up to catch my breath and my lips would turn purple," Cronk said. "Finally, I had a blood clot that caused my arm to swell up to twice its size and I knew it was time to figure out what was really going on."
At this point, it was determined that Cronk had congestive heart failure, but the root cause was still unknown. Finally, she was referred to pulmonary specialists at National Jewish Health, where comprehensive testing confirmed she was suffering from PAH, and while there is currently no cure, there are treatments to ease symptoms and help her manage the disease.
"Finally receiving effective medication and implementing some lifestyle changes to manage PAH has made an incredible difference in my symptoms," Cronk said. "Before my diagnosis, my body had essentially started shutting down — my heart was failing, my kidneys were affected and I was unable to even get up to shower on my own — but now, I'm able to drive myself to appointments, I can go shopping with my daughter, even take my dogs for a walk around the neighborhood. It's like night and day, and I feel like I got my life back."
"We truly value educating patients about their disease. Oftentimes, patients are their greatest advocates and we try to set them up for success," said Jordin Rice, RN, a pulmonary nurse practitioner at National Jewish Health. "Being able to teach patients like Kira about their medications and why their symptoms vary is a huge part of the comprehensive care we provide."
While current treatments work to relax blood vessels to treat PH, researchers are also developing and testing new treatments that address the biological cause of unexplained PH involving the thickening of blood vessel walls, which make it harder for the lungs to perform the critical tasks of taking in oxygen and releasing carbon dioxide.
"There are several drugs currently in clinical development that we are very excited about and feel will have a profound impact for patients suffering with pulmonary hypertension," said Dr. Lahm. "These novel therapies actively slow down the uncontrolled growth of the blood cells of the vessel wall, which is a whole new way to treat this disease."
There is additional research in the works that is providing more insight into cases of PAH without a clear cause, including a study recently published in CHEST exploring genetics, patients' knowledge of how their family history contributes to their disease and the role of genetic testing in assessing risk and customizing treatment.
"Genetic testing is a necessary component of comprehensive care and risk stratification for patients and family and can influence the treatment of pulmonary hypertension," said Rice, the lead author of the study. "Patients who undergo PAH evaluation should be given the opportunity to speak with a genetic counselor, but our study found that less than half of PAH patients were offered this resource."
Researchers continue to find clues about how the disease behaves uniquely in women and the specific biological pathways that hold the key to customizing treatment for women and men to one day even prevent the disease from developing. As that research evolves, it's important for anyone with unexplained symptoms to talk to their doctor about screening for PH. Early diagnosis is key to limiting damage to the heart and lungs and improving quality of life.
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