Overview

People are dying from heart attacks and it is the leading cause of global deaths. The cardiovascular and heart diseases are accused of being the cause of those death rates. Accordingly, it is highly critical to shed light on the list of heart diseases to raise awareness, increase the research in the field and initiate early diagnosis to prevent mortality as well as reducing the numbers, hopefully.

Papillary fibroelastoma is a type of tumour triggering heart attacks and enhancing the pool of death numbers from heart attack. It is a rare condition, therefore, people may not be fully aware of its existence. It can also be confused with cancer disease and dying from the cancer. This article aims to describe the condition and raise awareness of the life saving importance of detecting the tumour earlier.

Introduction

Papillary fibroelastoma is a type of mass that results in tumour formation located in the heart. They are within the group of primary cardiac tumours.¹ They have been reported to be the most prevalent primary cardiac tumours. Papillary fibroelastoma tumours are benign. The benign nature of the tumours can simply be explained by their non-cancerous nature.

They do not metastasize and they grow slowly. However, they are accused of transient ischemic attacks, strokes, myocardial infarction, and sudden death. Therefore, it is crucially life-threatening to be aware of the condition and detect them before they cause potential harm. 

Background Information

Papillary fibroelastoma tumours cover the endocardial cells found on the papillary surface and visually appear as sea anemone.² They can also be found on cardiac valves and in this case they are known as cardiac valve tumours.³ Cardiac valve tumours are the most prevalent papillary fibroelastoma. Also, more than 95% of papillary fibroelastoma appears on the left (the most common) part of the heart.² 

Causes and risk factors

The chance of incidence of PFEs is as rare as %1 and %55 percent of the cases are male.¹ Therefore, it can be concluded that having a male sex assigned at birth can lead to a slightly higher risk of the condition. 60 is the mean age of diagnosis, however, there are cases of reports which clearly inform about the sudden deaths in much younger populations.⁴ The underlying reason for those deaths is the embolism caused by PFEs. 

In the literature it is agreed that the endothelial damage results in the PFEs however, the exact cause is yet to be explored.¹

Clinical presentation

The papillary fibroelastoma is mostly asymptomatic whereas stroke is observed in 30% of the patients as a symptom.¹ Other less common symptoms can be listed as; dyspnea, transient ischemic, angina and syncope.² PFEs do not cause any physical change to the appearance. Therefore, it is highly difficult to diagnose since it makes physical diagnosis highly challenging. There are reports from a published case in Portugal where they have observed neurological abnormalities in very few of the cases with PFEs.⁵ 

There are cases in the literature where the chest-pain has been monitored in patients as a sign of PFE tumour.⁶

How to diagnose papillary fibroelastoma?

Transthoracic echocardiography (TTE) is the primary method for the diagnosis of papillary fibroelastoma.¹ However, the resolution of and definite location can be better visualized by using cardiac magnetic resonance imaging (MRI) and computed tomography (CT).

Nevertheless, those methods are highly expensive and limited access due to places not being able to offer expertise in the use of those technologies or evaluation of the end results. Three-dimensional (3D) TTE can provide better quality of images, however, it has been indicated that during the surgery the situation such as the number of tumours were different than the image.⁷

Papillary fibroelastoma are among the solid tumours, eventually, the best way of diagnosing solid tumours is carrying out a biopsy examination. The specific types of tumours in solid tumours are highly diverse therefore, histologic and heterogenic examination is carried out by biopsy.⁸

Treatment methods

If there is no health risk preventing the surgery, surgical excision of the tumour is the most recommended way of treatment.¹ However, in the case of serious other illnesses, a monitoring method is recommended. In case of valvular damage, valve replacement operation is recommended. On the contrary, anticoagulation can be applied to the cases where the surgery is not possible to prevent embolism. Nevertheless, there is no information or research which has been conducted to confirm the effectiveness of anticoagulation methods yet.¹

When the papillary fibroelastoma tumour is removed fully without the remaining parts during the surgery, there is no risk of recurrence or metastasis because of being a benign tumour. Accordingly, the prognosis is quite good in papillary fibroelastoma as long as it is detected early enough to prevent embolism and heart attack.

Multiple papillary fibroelastoma 

The case where you get multiple tumours with papillary fibroelastoma is rare. However, there is evidence in the literature where the patients get multiple tumours of papillary fibroelastoma.⁷ Transthoracic echocardiography cannot be the most effective way to visualize all the mass presence within heart location.

The reports had declared finding more lesions during the operation which were not present in the images. Consequently, more expensive but higher detailed MRI is a must for those suspected cases of multiple PFE tumours. However, the imaging techniques for PFEs still has a gap to be improved.

Distinguishing papillary fibroelastoma

Probably, you will think about how you can separate the symptoms of papillary fibroelastoma from other heart diseases and risk of heart attacks. This topic is quite confusing because they have the same symptoms. However, your own patient story can help you to determine the underlying reason or at least help you to be prepared.

High blood pressure and high cholesterol levels for example indicate the risk of presence of other heart complications and have high risk for a heart attack. A family history of heart diseases and heart attacks also increase your likelihood of developing heart and cardiovascular diseases. Moreover, smoking also increases your chance of having a heart attack and heart-related diseases.

The reasoning of papillary fibroelastoma is not definite yet. Therefore, those signs and habits can provide you a guide to predict your symptoms. However, as soon as you have your symptoms regardless of the reason, you need to seek urgent help. Detecting heart diseases early saves you from developing a stroke, heart attack and death.

FAQs

What is papillary fibroelastoma?

It is a type of primary cardiac tumour which results in mass formation within the heart.

Is papillary fibroelastoma a kind of cancer? 

No, papillary fibroelastoma is a benign tumour without cancerous features.

What is the difference between papillary fibroelastoma and cancerous tumours?

Papillary fibroelastoma tumours do not spread but cancerous tumours spread.

Is papillary fibroelastoma lethal? 

If it remains untreated, it can be lethal.

What is the current treatment option for papillary fibroelastoma?

Surgery and anticoagulation method for surgically unavailable patients.

Is there any current research for the papillary fibroelastoma?

Unfortunately, there is no ongoing research about papillary fibroelastoma

How can we differentiate symptoms of papillary fibroelastoma from other heart diseases?

Distinguishing the symptoms of papillary fibroelastoma from other heart diseases is very hard. Our history and familial background can help us to determine the likelihood of high cholesterol, high blood pressure related chest-pain or papillary fibroelastoma related chest pain

What causes papillary fibroelastoma? 

The cause of papillary fibroelastoma remains undiscovered but researchers have a common opinion about the possibility of endothelial damage within the heart lining. 

How to protect from lethality of papillary fibroelastoma?

Early diagnosis is life saving as in every kind of disease. Therefore, it is highly critical to go to regular checks and be aware of the symptoms to make early diagnosis.

Summary

Papillary fibroelastoma is among benign groups of cardiac tumours. It does not spread into other tissues, however, it can be lethal because of causing a heart attack. Consequently, it is highly critical to be aware of the symptoms and seek urgent medical help.

Gladly, the surgery grants a definite solution. Nevertheless, patients who are not good candidates for the surgery cannot be provided with a certain cure. They require close monitoring and treatment to reduce the symptoms and prevent stroke by anticoagulation.

There are still gaps in the research for the papillary fibroelastoma and the exact reason remains undiscovered. Also, anticoagulation methods for non-surgical patients require more investigation and there is no evidence of success of this method. Subsequently, it is vital to raise awareness in this topic and strike attention.