2022 Western Medical Research Conference
Lung Scarring Symptoms And Causes
Scars on the lung tissue cause it to thicken and lose elasticity. Doctors call this pulmonary fibrosis and 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 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.
Learn more here about interstitial lung disease.
Idiopathic pulmonary fibrosis 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.
Idiopathic pulmonary fibrosis typically appears between the ages of 50 and 70 years. It affects 13–20 people in every 100,000 worldwide, according to the National Library of Medicine.
Other causes and risk factorsOther risk factors 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.
What do the lungs do?
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:
shortness of breath, especially during or after physical activity
Scarring may also increase the risk of lung cancer.
Idiopathic pulmonary fibrosisThe symptoms of idiopathic pulmonary fibrosis 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 is key to treatment.
Scars that remain unchanged for 2 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 idiopathic pulmonary fibrosis. 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, only about 50% of people who received a diagnosis of idiopathic pulmonary fibrosis lived another 3 years, while close to 20% lived another 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.
What Is Tetralogy Of Fallot?
Tetralogy of Fallot (TOF) is a birth defect of the heart in which four conditions are happening. One, there is a hole between the heart's pumping chambers, or the ventricles. This is called a ventricular septal defect. Two, there is some obstruction or blockage of the pulmonary valve and the pulmonary artery, which connects the heart to the lungs. This is called pulmonary stenosis. Three, the aortic valve is larger than usual, and opens from both of the heart's ventricles instead of the left ventricle only. Four, the muscle surrounding the lower right ventricle thickens more than usual. This is called ventricular hypertrophy.
In someone without TOF, the right side of the heart pumps oxygen-poor blood to the lungs, where the lungs absorb oxygen and remove carbon dioxide. This creates oxygen-rich blood that can be sent to the left side of the heart, which sends it to arteries and the rest of the body.
In TOF, blood moves across the hole in the ventricles. The blood can then move through the aorta, sending oxygen-poor blood through the body.
The most common symptom for people with TOF is that they often turn blue, which is called cyanosis. The areas of the body most likely to turn blue are the fingers, toes, and lips. This is such a prominent symptom that tetralogy of Fallot was referred to as Blue Baby Syndrome many years ago.
Some parts of the body may not get as much oxygen as they should. This can result in shortness of breath.
For some people with TOF, symptoms do not occur right after birth. In some cases, cyanosis can occur months after the baby is born. Research suggests the severity of cyanosis depends on how obstructed the pulmonary artery is.
Other symptoms can include clubbing of the fingers, difficulty feeding, inability to gain weight, passing out, poor development, and squatting during episodes of shortness of breath. Squatting can lead to an increase in blood flow to the lungs, allowing the body to get more oxygen.
People with TOF can live with no symptoms, but some may have problems that occur over and over. This can include a leaky heart valve, an obstruction (blockage of blood leaving the right ventricle), and heart rhythm problems.
The cause of TOF in most children is unknown. It is the most common heart defect in children that is present at birth, and is more commonly seen in children with Down syndrome and DiGeorge syndrome. DiGeorge syndrome is a condition where a piece of a specific chromosome (chromosome 22) is deleted. This condition causes heart defects, low calcium levels, and poor immune function.
Some heart defects may be related to a person's genes or chromosomes, while others could be related to the environment. Factors which may increase risk of TOF include excessive alcohol consumption in the birthing parent, untreated diabetes of the birthing parent, a person who gives birth over the age of 40, poor nutrition during pregnancy, and Rubella or other viral illnesses during pregnancy.
Diagnosis of TOF can occur either during pregnancy at a prenatal visit or right after a baby is born.
During PregnancyTetralogy of Fallot may be diagnosed during a routine prenatal ultrasound (a tool used to view the developing embryo or fetus in utero). If the health care provider suspects TOF from the images of the ultrasound, they may order an echocardiogram to confirm the results from the ultrasound. The echocardiogram will help the healthcare provider examine blood flow and the heart's structure.
After BirthTetralogy of Fallot can be diagnosed after the baby is born if no tests are done during pregnancy. This is usually done if the baby turns blue when crying or feeding. This is called a "tet spell." The healthcare provider may also suspect TOF during a physical exam if they notice the skin has a bluish color or if they hear a heart murmur.
Tetralogy of Fallot in AdultsMost people are diagnosed with TOF as an infant or young child. Tetralogy of Fallot that is left untreated can be seen in adults, as they may experience certain areas of the body turning blue. Treatment options can look similar for children and adults.
In TOF, the goal for treatment is to minimize symptoms and live as normal a life as possible. The most important part about living with a heart defect is to see a cardiologist (a medical doctor who specializes in the heart) regularly and seek treatment options as necessary.
Treatment options for TOF include two types of surgery: a shunt operation or a complete repair.
Because there is no proven research explaining what causes TOF, there is no known way to prevent the condition. Some risk factors for TOF are related to the birthing parent's dietary habits. It is important to see a healthcare provider during pregnancy to discuss any potential risk factors.
For those who do have heart surgery, physical activity exercise should be limited if shortness of breath is a recurring issue. Check with a cardiologist about physical limitations and recommendations for strenuous activities.
Children diagnosed with TOF are at higher risk for endocarditis (inflammation of the lining around the heart's chamber and valves). Some children may also need to take antibiotics before certain dental procedures. This can help to prevent endocarditis.
Surgery to open up the pulmonary valve can lead to complications, including a leaky pulmonary valve. Your healthcare provider may suggest another surgery to resolve this problem. Obstructed pathways can also be a problem as the person with TOF grows. Another surgery to replace the pulmonary valve may be needed later if these conditions occur.
In addition to these conditions, people with TOF may experience weakened function of the right-side of the heart or an abnormal heart rhythm. Pregnant people who have had a repair for TOF should be monitored by a cardiologist for treatment options in case there are complications during pregnancy or while giving birth.
Tetralogy of Fallot is rare, but it is also the most common type of heart disease at birth. If you or your child have been diagnosed with TOF you will need to consult with your doctor following surgical repair for guidelines.
Even if surgical repair of the heart goes well, regular follow up with a cardiologist is recommended. The cardiologist should be someone who specializes in congenital heart defects. Even after your child becomes an adult, they will still need to follow up with a cardiologist regularly. In addition to regular follow up with a cardiologist, you may need medications and more surgeries if any complications from TOF occur.
Faulty Tricuspid Valve Linked To Less Success For Mitral Valve Therapy
PHOENIX -- Tricuspid regurgitation (TR) at baseline was linked to poorer outcomes after percutaneous mitral valve repair and didn't improve for most patients afterward, two registry studies showed.
The proportion alive and well based on quality-of-life scores at 1 year was significantly lower for patients with baseline severe TR compared with none, trace, or mild TR (adjusted OR 0.53, 95% CI 0.40-0.71).
The same stair step across mild to moderate to severe TR was seen for all-cause mortality at 1 year (adjusted HR 1.17, 95% CI 1.04-1.32 and aHR 1.49, 95% CI 1.29-1.72), with a nonsignificant trend in all-cause readmission as well, reported researchers led by Yasser Sammour, MD, MSc, of Houston Methodist, during the Society for Cardiovascular Angiography and Interventions (SCAI) meeting here.
And in another study of transcatheter mitral valve repair patients with concomitant severe TR, TR improved in only 42%, noted Craig Basman, MD, of Northwell Health and Lenox Hill Hospital in New York City, reporting the results at SCAI and online in JSCAI.
Impact of Baseline TR
Sammour and team's study included all 19,593 consecutive adults enrolled in the Society of Thoracic Surgeons/American College of Cardiology TVT Registry who had no prior mitral valve procedures and underwent isolated mitral valve transcatheter edge-to-edge repair (M-TEER) from January 2019 through June 2021. Baseline TR was none, trace, or mild for 52% of the patients, while 33% had moderate TR and 15% had severe TR.
Alive and well at 1 year was defined by a Kansas City Cardiomyopathy Questionnaire (KCCQ) score of at least 60 on the 100-point scale without a drop of 10 or more points compared with baseline.
Baseline KCCQ was poorer with higher grades of TR (45.4 with mild or less TR, 41.9 with moderate TR, and 38.1 with severe TR, P<0.0001). M-TEER procedural success showed the same pattern (76.3%, 73.2%, and 69.4%, P<0.0001).
"It's important to understand the impact of TR on those patient outcomes, especially in the emerging era of transcatheter interventions for tricuspid regurgitation," Sammour said. Such findings might help determine which patients would benefit the most from those therapies, he added.
Session moderator George Hanzel, MD, of Emory University in Atlanta, noted that "when we see people who have severe tricuspid and mitral regurgitation, I don't think we necessarily have to exclude them from treatment, but I think that we have to look at what the next treatment options are for the tricuspid valve."
"We know these patients ultimately do poorly," he continued. "I'm not sure if a tricuspid clip is the best way to treat them, but potentially, would they be good candidates for clinical trials to help hopefully improve their clinical status and hopefully long-term outcomes?"
Predictors of Success
Basman's study included the 107 patients with severe regurgitation of the the tricuspid and mitral valves among the 479 who underwent M-TEER with the MitraClip as recorded in the Northwell TEER registry, a "prospectively maintained mandatory database" of four high-volume valve centers in a single health system.
Of these patients, 84% had mitral regurgitation (MR) drop to moderate (grade 2+) or less, and 42% dropped at least one TR grade to moderate (2+) or less TR at 1 month.
In the unadjusted analysis, predictors of severe residual TR were right atrial area and lack of success in reducing MR. On the multivariate logistic regression model, the only predictor of a reduction in TR was MR reduction of 3+ with MitraClip.
"Other clinical and echocardiographic variables (including pulmonary hypertension, right ventricular function, tricuspid annular dilation, atrial fibrillation, and presence of a cardiac implantable electrical device) were not associated with residual TR," Basman and team noted. "Inability to predict TR reduction after M-TEER highlights the importance of establishing transcatheter tricuspid valve therapies and should factor in heart-team discussions."
An accompanying editorial by Vikrant Jagadeesan, MD, of West Virginia University School of Medicine in Morgantown, and John Blair, MD, of the University of Chicago Pritzker School of Medicine, pointed out that this study was the "largest analysis to date on significant predictors of severe TR after m-TEER," but the many limitations of the study hurt its ability to be practice-changing.
Along with the small sample size in a single hospital system, "the number of operators and their experience is not known, which may affect procedural success and outcomes," they added.
In addition, all baseline covariates were determined by transthoracic rather than transesophageal echocardiography, which left out a number of potential important analyzable predictors; for example, baseline tricuspid coaptation gap, which could be "very relevant" given that more than 90% of the TR pathology was functional. Moreover, degree of left ventricular systolic or right ventricular dysfunction were not quantified and procedural variables -- such as average number of clips per case or mitral mean gradients at the end of procedure or on follow-up -- were not described.
Furthermore, 12-month outcomes would have had more significance, the editorialists added.
"Future studies in larger cohorts using hemodynamic parameters and multimodality imaging will help elucidate in which patients residual TR after m-TEER is a bystander responsive to reduction in PA [pulmonary arterial] pressure or an accomplice to severe MR that will continue to portend a poor prognosis even after successful m-TEER," they concluded.
Disclosures
Sammour disclosed no relevant conflicts of interest.
Basman disclosed no relevant conflicts of interest. Co-authors disclosed relationships with Abbott, Edwards Lifesciences, and Medtronic.
Jagadeesan and Blair disclosed no relevant conflicts of interest.
Primary Source
Society for Cardiovascular Angiography and Interventions
Source Reference: Sammour Y "Impact of baseline tricuspid regurgitation on health status and clinical outcomes after transcatheter edge-to-edge repair of the mitral valve: insights from the STS/ACC TVT Registry" SCAI 2023.
Secondary Source
JSCAI
Source Reference: Basman C, et al "Predictors of residual severe tricuspid regurgitation after transcatheter mitral valve repair" JSCAI 2023; DOI: 10.1016/j.Jscai.2023.100612.
Additional Source
JSCAI
Source Reference: Jagadeesan V, Blair J "Residual tricuspid regurgitation after mitral transcatheter edge-to-edge repair: accomplice or bystander?" JSCAI 2023; DOI: 10.1016/j.Jscai.2023.100999.
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