What is Papillary Fibroelastoma?
Primary cardiac tumors, or tumors that originate from the heart, are quite uncommon and mostly non-cancerous. In adults, the most typical types of these tumors are papillary fibroelastomas (PFEs) and cardiac myxomas. PFEs, in particular, are benign or non-cancerous growths.
In a significant study involving 511 patients at the Mayo Clinic, PFEs were found to be the most common type of cardiac tumor. Because advanced imaging techniques, like transesophageal echocardiography (TEE, which involves creating images of the heart through the esophagus) are now being utilized more frequently, doctors are able to detect these types of heart tumors more often.
The first case of PFE was reported in 1975, where it led to complications including an embolism, which is a blockage of blood vessels caused by a blood clot or other foreign material that traveled through the bloodstream and obstructed blood flow, resulting in a heart attack. Since then, PFEs have been noted as a potential cause of vascular embolism, stroke, and cardiac arrest.
However, the debate continues about whether to manage these benign tumors with surgery or medication, especially since they are known to cause further embolic complications. This article aims to give readers the most up-to-date information on PFEs.
What Causes Papillary Fibroelastoma?
The exact cause of papillary fibroelastomas, a type of uncommon tumor, is largely unknown. They are most commonly found originating from the lining of the heart valves, which is believed to be due to damages in these areas.
Risk Factors and Frequency for Papillary Fibroelastoma
Primary cardiac tumors are extremely rare, with studies showing an incidence of less than 0.1%. These types of tumors are most commonly found in males, who make up approximately 55% of the affected patients, and are typically detected at around the age of 60. However, the actual number of people with these tumors may be higher as many are only discovered after the patient has died.
The most common place for these tumors to grow is on the aortic valve, which is found in 35% to 63% of cases. However, they can also appear on other heart valves:
- The mitral valve: 9% to 55% of cases
- The tricuspid valve: 6% to 15% of cases
- The pulmonic valve: 0.5% to 8% of cases
Signs and Symptoms of Papillary Fibroelastoma
Papillary fibroelastoma (PFE) often does not show any symptoms and is usually found by chance. However, in some cases it first shows up through effects such as stroke, neart attack, or heart blockages leading to fainting, heart failure, or sudden death. In fact, stroke is the most common way it first appears, in about 30% of patients. Also, it is often discovered unexpectedly in about a third of all patients. PFE does not change the physical examination findings, but if it is causing complications then the physical exam will show signs of that complication. It’s extremely rare to diagnose PFE based solely on medical history and a physical exam.
Testing for Papillary Fibroelastoma
If your doctor suspects that you have a certain type of heart growth called a papillary fibroelastoma, they might initially use a test called transthoracic echocardiography (TTE). This method is preferred because it is less invasive. TTE can effectively spot papillary fibroelastomas that are larger than 2 mm with an 88.9% accuracy rate for correct detection and an 87.8% accuracy rate for correct ruling out.
However, if there is a strong suspicion that a stroke was caused by a blood clot coming from the heart, which can sometimes be linked to papillary fibroelastomas, another test called a transesophageal echocardiogram (TEE) might be used. This test provides a better view of the inside of the heart. If the papillary fibroelastoma is smaller than 2 mm, the accuracy for detection using TTE is 61.9%, while the accuracy using TEE is 76.6%.
Interestingly, most of the time papillary fibroelastomas are discovered by accident during heart imaging tests, heart surgeries, or even autopsies.
Using these tests, papillary fibroelastomas are typically seen to be anywhere from 2 mm to 40 mm in size and are usually attached by a stalk, which makes them able move independently. They may appear on the aortic side or the ventricular side of heart valves. Still, past studies have shown inconsistent results about where they are more likely to be found.
Alternative imaging tests, such as cardiac magnetic resonance imaging (MRI) and computed tomography (CT), might provide better images. However, these tests are significantly more costly and require specialized resources for performance and evaluation. Just like with any type of solid tumor, a final diagnosis of papillary fibroelastoma needs a pathologic analysis and examination, essentially a biopsy where tissue is examined under a microscope.
Treatment Options for Papillary Fibroelastoma
The two major approaches for treating this condition involve either surgery or close observation. Surgery is commonly advised unless there’s a good reason not to undertake it. The main aim of surgical treatment is to remove all visible signs of the tumor and fix any damage in the heart caused by the tumor. Papillary fibroelastoma, the type of tumors we’re addressing, are typically attached by a stalk and can be accurately removed. The surgeon also checks the other compartments of the heart to ensure no multiple tumors are overlooked. If the tumor has considerably damaged a heart valve, that valve may need to be repaired or replaced during surgery.
In cases where the patient’s health may not effectively handle surgery and they are showing symptoms, blood-thinning medication could be considered as an alternative. The goal here is to prevent complications such as the formation of blood clots, although it’s worth noting that there aren’t any satisfactory clinical trials to fully support this practice.
Those patients not experiencing any symptoms but whose tumor size is over 9 mm, along with highly mobile masses, and independent motion, have higher risks. Therefore, even if they don’t show symptoms, they should still consider opting for surgery.
What else can Papillary Fibroelastoma be?
When a doctor detects a mass on a patient’s heart valve, there are many potential diagnoses they might consider. The best guess often depends on the patient’s symptoms and health history. For example:
- If the patient shows signs of infection, the mass might be due to endocarditis, a serious heart infection. This can often be confirmed with specific types of ultrasound scans.
- If the patient has a condition like lupus or other inflammatory diseases, the mass could be a rare form of endocarditis that’s not caused by bacteria.
- The mass could be a blood clot, a harmless overgrowth of normal heart tissue (Lambl’s excrescence), or a buildup of calcium on the heart valves.
- It is not uncommon for masses on the heart to actually be cancer that started somewhere else in the body, like the breast, liver, kidney, or the tissue that connects the body’s muscles and skin.
- Rarely, the mass could be a specific type of cancer that started in the heart itself. These are typically either angiosarcoma or leiomyosarcoma, and they usually don’t affect the aortic valve (the main valve that controls blood flow from the heart).
In all these scenarios, the correct diagnosis is crucial for effective treatment. Therefore, it’s essential for healthcare providers to consider all these possibilities.
Surgical Treatment of Papillary Fibroelastoma
Papillary fibroelastoma is a type of heart tumor that requires surgery for its removal. This surgery needs a procedure called cardiopulmonary bypass, which temporarily takes over the function of the heart and lungs during the operation. Depending on the patient’s risk factors, such as personal and family medical history, a heart evaluation with a special imaging technique called catheterization might be necessary before the surgery. However, if the tumor is on the aortic side of the heart valve, there may be risks related to the possibility of the tumor breaking off due to the catheter.
The surgery starts out by making a cut down the middle of the patient’s chest to access the heart. Tubes are then inserted into the aorta (the main artery carrying blood out of the heart) and the right atrium (one of the heart’s chambers) to allow blood to be diverted to the cardiopulmonary machine. This process is called cannulation. It gives the surgeons flexibility to work on the heart without it having to pump blood at the same time.
Then, a solution called cardioplegia can be given to protect the heart muscle during the surgery. The heart is stopped (arrested), and an incision is made in the aorta (aortotomy) to reach and inspect the aortic valve, where the tumor is located.
The tumor is then carefully cut away, trying to avoid as much damage to the valve leaflets (the parts of the valve that open and close to control blood flow) as possible. If necessary, part of the valve leaflets may also need to be removed. After this, the heart’s lower chamber, or ventricle, and the ventricular side of the other valve (the mitral valve) are thoroughly inspected.
If the aortic valve is found to still be functioning properly, the incision in the aorta is closed. However, if the valve is damaged, it may need to be repaired or replaced. After this, the air is removed from the heart and the patient is gradually taken off the cardiopulmonary bypass machine.
Before the surgery is concluded, an imaging test called a transesophageal echocardiogram (TEE) is performed to ensure that there are no other problems. Lastly, the removed tumor is sent to the lab for further testing. The use of frozen sections – a procedure where the sample is quick-frozen and sliced thin for immediate examination – is not usually necessary.
What to expect with Papillary Fibroelastoma
The outlook after removing a papillary fibroelastoma, a type of non-cancerous heart tumor, is usually positive. As these tumors are benign (not harmful in effect), the chances of them spreading to other parts of the body or coming back are low. However, if the tumor wasn’t completely removed during the initial surgery, there’s a possibility of it returning.
Possible Complications When Diagnosed with Papillary Fibroelastoma
Some of the complications associated with heart procedures come from the risk of embolization, which is a medical term for when a blood vessel gets blocked. Research has indicated the possibility of sudden heart failure, irregular heart rhythms, heart attacks, and strokes due to this risk. Following surgery, additional risks are present; these are common to any heart surgery performed on heart-lung bypass. These include the aforementioned blockage of blood vessels, excessive bleeding, irregular heart rhythms, and infection.
Possible Complications:
- Emboli or blood vessel blockage
- Sudden cardiac death
- Ventricular fibrillation or irregular heart rhythms
- Myocardial infarction or heart attack
- Stroke
- Bleeding
- Arrhythmias or more irregular heart rhythms
- Infections
Recovery from Papillary Fibroelastoma
The care provided after open-heart surgery is similar to other such operations. This care typically involves an incision along the center of the chest, known as a median sternotomy. It’s encouraged that patients begin to walk around as soon as the day after the operation. Drainage tubes, used to remove excess fluid from the operation site, are taken out according to the hospital’s standard procedures.
Whether or not patients should participate in post-surgery cardiac rehabilitation or physical therapy depends on their ability to move around. These methods can be especially beneficial for patients who are older or have been inactive for a period of time before their surgery.
Preventing Papillary Fibroelastoma
It’s worth noting that the most common type of heart tumor is still quite rare, occurring in less than 0.1% of cases. These tumors, known as papillary fibroelastomas (PFEs), are usually discovered by chance during a heart scan called a transthoracic echocardiogram. When PFEs are identified, they should be assessed first by a heart specialist (cardiologist) and a surgeon who specializes in heart and lung surgery (cardiothoracic surgeon).
PFEs generally don’t cause any symptoms. However, if they break off and block a blood vessel (a process known as embolization), they can cause serious problems depending on where they end up. If a PFE blocks a vessel in the heart, it can lead to a heart attack. If it blocks a blood vessel in the brain, it can cause a stroke. Despite this, there are no well-established risk factors for developing PFEs.
