Papillary Fibroelastoma (Symptoms,Causes and Treatment)
A uncommon benign cardiac tumor called papillary fibroelastoma usually develops on the heart’s valves, especially the aortic or mitral valve. A thin, fibrous stalk that supports a growth that resembles a frond and is covered with elastic tissue makes up the tumor. While the majority of papillary fibroelastosis are asymptomatic, bigger tumors or those that are close to the heart valves can result in cardiac murmurs, blockages, or other issues. Surgery is often used as a form of treatment to remove the tumor and avoid any consequences. To monitor the heart and avoid further issues, it is advised to schedule routine follow-up appointments with a cardiologist.
This article covers the following topics :
Papillary fibroelastoma: What is it?
A rare benign cardiac tumor, papillary fibroelastoma—also known as cardiac papilloma or valvular papilloma—usually develops on the heart’s valves, especially the aortic or mitral valve. A thin, fibrous stalk that supports a growth that resembles a frond and is covered with elastic tissue makes up the tumor. It is typically discovered in patients between the ages of 60 and 70 and is thought to make up fewer than 10% of all cardiac tumors.
While the majority of papillary fibroelastosis are asymptomatic, bigger tumors or those that are close to the heart valves can result in cardiac murmurs, blockages, or other issues. Chest pain, breathing difficulties, lightheadedness, and fainting are possible symptoms. Blood clots brought on by the tumor may potentially result in stroke or other issues if they spread to other parts of the body.
Papillary fibroelastoma’s root cause is unknown, but some research points to aberrant blood flow or turbulence near the valves as a potential contributing factor. The problem has also been linked to a number of illnesses, including Marfan syndrome, a hereditary disorder that affects the connective tissues of the body.
Imaging procedures like echocardiogram, which uses sound waves to make images of the heart and can detect the presence of the tumor and its location, are frequently used in the diagnosis of papillary fibroelastoma. It may also be used to supplement other tests, such a CT scan or an MRI.
When a tumor is causing symptoms or issues, papillary fibroelastoma is normally treated by surgically removing the tumor. The surgeon performs the procedure, removing the tumor and a tiny section of the compromised valve. If the valve has been harmed by the tumor, it may occasionally be repaired or replaced.
Papillary fibroelastoma patients have a typically positive outlook because the tumor is benign and can be surgically safely removed. To monitor the heart and avoid further issues, it is advised to schedule routine follow-up appointments with a cardiologist. In order to lower their risk of developing other cardiac diseases, patients may also be recommended to undertake lifestyle changes like stopping smoking, exercising frequently, and keeping a healthy weight.
Which valve is papillary fibroelastosis most likely to affect?
The most typical location for papillary fibroelastoma, a rare benign cardiac tumor, is the heart valves. After myxomas, which are also typically benign primary heart tumors, it is regarded as the second most prevalent. Small and typically linked to the valve by a stalk-like structure known as a pedicle, papillary fibroelastosis is fibrous in nature.
The aortic valve, which divides the left ventricle of the heart from the aorta, the biggest artery in the body, is most frequently impacted by papillary fibroelastosis. The aortic valve is where approximately 70% of all papillary fibroelastosis develops. However, they can also happen in the tricuspid and mitral valves, two more cardiac valves.
Papillary fibroelastosis is typically asymptomatic and tiny, with a diameter of less than 1 centimeter. However, they may result in symptoms if they disrupt the valve’s operation or if they get loose and migrate through the circulation, obstructing or harming other organs.
Papillary fibroelastosis’ precise origin is uncertain. They may be linked to the aging of the heart valves, which may cause the development of small, roughened regions on the valve surface that may be attracted to tiny blood clots. The valve tissue may then incorporate the clots, creating the papillary fibroelastoma.
The most common method for diagnosing papillary fibroelastosis is echocardiography, a non-invasive diagnostic procedure that uses sound waves to create images of the heart. Options for treatment vary according on the tumor’s size, location, and existence of symptoms. Papillary fibroelastosis frequently doesn’t need to be treated, although it may be advised to undergo routine monitoring. Surgery may be required to remove the tumor if it is causing symptoms or if it is preventing the valve from working properly.
Papillary fibroelastosis has a favorable prognosis overall, with low rates of recurrence and a low chance of developing into malignancy. To avoid issues and provide the greatest results for patients, careful monitoring and management are crucial.
Where can you find papillary fibroelastosis?
Papillary fibroelastosis (PFEs) is a condition that affects the heart’s valves, most frequently the left-sided aortic and mitral valves. They can also be present on the tricuspid and pulmonic valves on the right, as well as in other parts of the heart like the endocardium or the chordae tendineae. PFEs are occasionally discovered in locations other than the heart, such as the major arteries or even other organs. The severity of symptoms and the best course of treatment can be affected by a PFE’s location.
How does my body respond to papillary fibroelastosis?
Small, benign tumors called papillary fibroelastosis (PFEs) commonly develop on the heart’s valves or in other places. PFEs are frequently found by accident during autopsies or medical imaging procedures and do not typically produce any symptoms or consequences.
PFEs can, however, occasionally cause issues depending on where they are located and how big they are. Larger PFEs have the potential to restrict blood flow via heart valves and impair the heart’s regular operation. A PFE can result in significant consequences including a stroke, heart attack, or pulmonary embolism if it separates from its original site and spreads to other parts of the body.
PFE symptoms can change depending on where they are and how much obstruction they produce. Some typical signs include:
*Exhalation difficulty
*Chest discomfort or pain
*Heart palpitations or erratic heartbeat
*Weakness or fatigue
*Quiver or feeling faint
Working closely with your healthcare practitioner will help you monitor the PFE’s progress and location and choose the best course of therapy if you suffer any of these symptoms or have been diagnosed with a PFE.
Papillary fibroelastosis affects who?
Rare cardiac tumors known as papillary fibroelastosis can affect people of any age or gender. Although they have also been noted in infants, kids, and younger people, they are most frequently found in adults between the ages of 60 and 70. Men experience papillary fibroelastosis more frequently than women. They are frequently found by chance during imaging tests carried out for other purposes or during heart surgery. Many people with papillary fibroelastosis are asymptomatic, while some may suffer symptoms like chest pain, breathlessness, palpitations, or symptoms resembling a stroke.
Papillary fibroelastosis is how common?
Less than 10% of primary cardiac tumors are papillary fibroelastosis (PFE), which are uncommon cardiac tumors. The incidence of primary cardiac tumors in the general population ranges from 0.001% to 0.3%. PFEs have been observed in people of various ages, including infants and children, but they are more frequently found in adults over the age of 60. The median age of patients with PFEs was 67 years old in a 2017 study that was published in the Journal of the American College of Cardiology, with a range of 18-94 years.
Men are somewhat more likely than women to get PFEs. Nevertheless, there are inconsistent findings about gender distribution in the medical literature, with some research revealing no appreciable differences between males and girls. Further research is necessary to determine the cause of any potential gender disparities in the occurrence of PFEs.
Despite their rarity, PFEs contribute significantly to cardiovascular morbidity and mortality because they can result in life-threatening consequences such stroke, embolism, and valvular dysfunction. Therefore, to avoid these potentially fatal consequences, timely diagnosis and therapy of PFEs are essential.
What signs might indicate a papillary fibroelastoma?
Papillary fibroelastosis (PFEs) is frequently asymptomatic and unintentionally found during imaging tests that are carried out for other purposes. However, according on where they are and how big they are, they may result in symptoms like:
1-Chest discomfort or pain
2-Palpitations or an unsteady heartbeat
3-Exhalation difficulty
4-Fatigue
5-Lightheadedness or dizziness
6-Stuttering or syncope
7-Rarely, a transient ischemic attack (TIA) or a stroke may occur.
A tumor’s ability to embolize a portion of itself or to produce arrhythmias can all cause symptoms to appear. The location of the tumor and the degree of blood flow restriction or embolization determine the intensity and presentation of the symptoms.
Why do papillary fibroelastomas develop?
Although the precise cause of papillary fibroelastoma is unknown, there are a number of risk factors connected to it. These consist of:
1-Age: People over 60 are more likely to have papillary fibroelastosis.
2-Men are more prone than women to acquire papillary fibroelastosis.
3-Heart issues: Papillary fibroelastosis is more likely to develop in people who have specific heart disorders, such as mitral valve prolapse, aortic stenosis, and atrial fibrillation.
4-Inherited diseases: Conditions like Marfan syndrome, which is an inherited disease, can raise the risk of papillary fibroelastosis.
5-Trauma: Papillary fibroelastosis has been documented to appear in reaction to heart trauma or injury, albeit this is uncommon.
It is significant to remember that papillary fibroelastosis occasionally manifests itself without any identified risk factors.
Exactly how is papillary fibroelastosis identified?
Imaging studies such as an echocardiography, transesophageal echocardiogram (TEE), or cardiac MRI are frequently used to identify papillary fibroelastosis. A tiny transducer is positioned on the chest during an echocardiography to transmit sound waves that produce an image of the heart. In a TEE, a tiny probe is inserted into the esophagus through the mouth to get sharper pictures of the heart. A strong magnetic field and radio waves are used in a cardiac MRI to provide precise images of the heart.
A doctor may request blood tests in addition to imaging studies to look for inflammation or other heart disease signs. To check for irregular cardiac rhythms, an electrocardiogram (ECG) may also be performed.
A biopsy or tumor removal may be required if a papillary fibroelastoma is detected in order to confirm the diagnosis and avoid complications.
What kinds of tests are used to identify papillary fibroelastoma?
A combination of the medical history, physical examination, and diagnostic testing may be utilized to identify papillary fibroelastoma. Papillary fibroelastoma can be diagnosed using the following tests:
1-The first test performed to identify papillary fibroelastoma is an echocardiogram. Images of the heart and its architecture are produced using sound waves. It can aid in determining the papillary fibroelastoma’s size, location, and features.
2-Transesophageal echocardiography (TEE): This is a customized echocardiogram that uses a tube put into the esophagus to provide more precise images of the heart’s chambers and valves.
3-Cardiac magnetic resonance imaging (MRI): This procedure produces precise images of the heart’s internal components using a magnetic field and radio waves. It can reveal details regarding the papillary fibroelastoma’s size, location, and features.
4-Computerized tomography (CT) scan: This imaging procedure employs X-rays to produce fine-grained pictures of the heart’s internal architecture. It can aid in determining the papillary fibroelastoma’s size, location, and features.
5-Coronary angiography: This examination makes use of X-rays and a dye to show the heart’s blood arteries. It can assist in locating any artery blockages that might be generating symptoms resembling those of papillary fibroelastoma.
6-Biopsy: A papillary fibroelastoma biopsy can assist confirm the diagnosis and obviate the presence of additional diseases.
To accurately diagnose papillary fibroelastoma, several tests could occasionally be required.
What does an echo reveal about a papillary fibroelastoma?
A papillary fibroelastoma often shows up as a tiny, moveable lump adhering to a valve leaflet or chordae tendineae on echocardiography. With several finger-like projections spreading from a central stalk, it has a distinctive “sea anemone” or “frond-like” appearance. Depending on its size and position, the mass, which is typically homogeneous and well-circumscribed, may result in valvular dysfunction or blockage. In some circumstances, the mass may also be unintentionally found in other imaging tests like a CT scan or an MRI.
Exactly how is papillary fibroelastosis treated?
The course of treatment for papillary fibroelastosis is determined by the size, location, and symptoms of the tumor. The patient may just need to be monitored through routine echocardiograms and imaging examinations if the tumor is tiny and not causing any symptoms.
However, surgical excision can be required if the tumor is huge or results in symptoms like a heart attack or stroke. Open-heart surgery is typically used for the procedure, while minimally invasive methods might be employed in rare circumstances. The tumor is taken out of the heart chamber or valve during surgery, and if necessary, the region is repaired.
Other therapies might be considered in some situations, especially if the tumor is too difficult to remove or the patient is not a suitable candidate for surgery. These could include drugs to treat symptoms or lower the chance of problems like blood clots.
The patient can typically anticipate a favourable long-term prognosis because papillary fibroelastosis have a low chance of recurrence once they have been excised. However, it’s crucial to follow up with a doctor on a regular basis to check for any potential issues.
What kind of chances do those who have papillary fibroelastosis have?
Given that papillary fibroelastosis is primarily caused by benign tumors and has a minimal risk of leading to major consequences, the prognosis for affected individuals is normally favorable. However, there is a chance that the tumor could fragment and obstruct the heart or another blood channel, resulting in a stroke or other catastrophic issues if neglected. It’s critical for people with papillary fibroelastoma to follow up with their doctor on a frequent basis to monitor the tumor’s size and position as well as to make sure that any potential complications are quickly diagnosed and addressed.
When should I schedule a consultation with my doctor about Papillary Fibroelastoma?
If you have been diagnosed with a heart issue or are exhibiting any symptoms associated with heart function, you should consult your doctor. Have regular check-ups with your healthcare provider to monitor your heart health if you have a history of heart disease or risk factors for heart disease, such as high blood pressure or high cholesterol. You should speak with your doctor if you have any worries about papillary fibroelastosis or any other cardiac disease.
What inquiries concerning papillary fibroelastosis should I make to the doctor?
The following are some queries you can think about posing to your doctor regarding papillary fibroelastosis:
1-How did I develop a papillary fibroelastoma, and what is it?
2-What are the signs and how may I identify a papillary fibroelastoma?
3-What tests are necessary to determine whether I have a papillary fibroelastoma?
4-Are there any dangers or drawbacks to having a papillary fibroelastoma?
5-What are my papillary fibroelastoma therapy options, and what is the best option for me?
6-How do persons with papillary fibroelastosis fare over the long term?
7-Should I alter my lifestyle in any way to stop a papillary fibroelastoma from recurring?
8-When should I contact you for follow-up appointments?
9-Do I need to see any additional doctors for my papillary fibroelastoma?
10-How can I treat my papillary fibroelastoma-related symptoms or complications?
What does papillary fibroelastosis illness signify by the mass in your heart?
A tiny, benign (noncancerous) tumor that grows on a heart valve or the endocardium (inner lining) of the heart is referred to as a mass in the heart in papillary fibroelastoma illness. These tumors include tiny projections, known as papillae, that resemble fingers and are made of elastic and fibrous tissue. Even though they are usually small (less than 1 cm), if one breaks off and travels to the brain or other organs, it might result in symptoms like a heart attack or stroke. In order to avoid problems, timely diagnosis and treatment are crucial.