What are the other Names for this Condition? (Also known as/Synonyms)

• Cardiac Papillary Fibroelastoma (CPF)
• Fibroelastic Papilloma of Heart
• Giant Lambl Excrescence

What is Papillary Fibroelastoma? (Definition/Background Information)

• Papillary Fibroelastoma is a benign tumor of the heart valves that can occur in children and adults. After myxoma of the heart and lipoma of the heart, Papillary Fibroelastoma is the 3rd most common tumor of the heart
• The cause of Papillary Fibroelastoma is also generally unknown. However, research scientists have proposed various possibilities that include viral infections, heart valve trauma, and thrombus (blood clot)
• Papillary Fibroelastoma typically affect the heart valves; the aortic or mitral valves are affected most times. The signs and symptoms may include breathing difficulties, chest pain, and dizziness. Complications, such as stroke or sudden cardiac death, may occur due to thromboembolism affecting the brain or coronary arteries respectively
• The diagnosis of Papillary Fibroelastoma of Heart might involve a thorough physical evaluation by a healthcare provider, imaging studies of the heart including echocardiography, and a tissue biopsy for a definitive diagnosis
• Individuals without significant signs and symptoms may be placed under observation; however, if symptoms are observed due to the tumor, a surgical excision can be curative. The prognosis is generally good on tumor removal, but may be linked to the associated signs and symptoms of underlying conditions

Who gets Papillary Fibroelastoma? (Age and Sex Distribution)

• Papillary Fibroelastoma is seen in a wide age group that includes children, young and old adults. 10% of all tumors of the heart are Papillary Fibroelastomas
• Studies indicate that these tumors may be found in newborn children and in adults, who are 90 years old. The average age of diagnosis is 60 years
• Both males and females are affected and no gender preference is seen
• All races and ethnic groups are at risk for the condition

What are the Risk Factors for Papillary Fibroelastoma? (Predisposing Factors)

The risk factors for Papillary Fibroelastoma include:

• Rheumatic heart disease
• Infective endocarditis of the heart valves
• Radiation to the chest
• Heart valve surgery

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Papillary Fibroelastoma? (Etiology)

The cause of development of Papillary Fibroelastoma is generally unknown.

• Some researchers believe that it is an organized thrombus (a well-formed blood clot) on the heart valves
• Some believe these tumors are due to abnormal congenital embryological remnants during fetal development
• Some reports indicate that the tumors may be due to viral infections such as Cytomegalovirus (CMV)
• Some researchers believe that Papillary Fibroelastoma is a reaction to repeated trauma to the heart valves

Research is currently being undertaken to identify the relevant causal factors.

What are the Sign and Symptoms of Papillary Fibroelastoma?

In many cases, the condition remains asymptomatic. In others, the signs and symptoms of Papillary Fibroelastoma may include the following:

• The tumor may case difficulty breathing, palpitations, fainting, and chest pain
• Presence of small tumors that range in size from 2 mm to 5 cm in the heart; however, majority tumors are less than 1 cm in size
• In 9 out of 10 cases, only a single tumor is observed; while, in 1 in 10 cases, multiple tumors are present
• Also, 90% of the tumors are sited on the heart valves, while 10% are seen on other areas of the heart. Papillary Fibroelastoma commonly affects the aortic valve or the mitral valve
• Additionally, the signs and symptoms of stroke (due to thromboemboli) may be present

How is Papillary Fibroelastoma Diagnosed?

A diagnosis of Papillary Fibroelastoma may be undertaken using the following tests and exams:

• Complete evaluation of family (medical) history, along with a thorough physical examination; including examination of the heart (with special emphasis to signs such as abnormal heart sounds)
• CT or MRI scan of the heart
• Echocardiography: This procedure uses sound waves to create a motion picture of the heart movement
  • It can help show the size and shape of the heart and how well the chambers and valves are working
  • It can also help visualize the tumors, especially the large tumors; 1 mm and lesser-sized tumors may not be visible though
• Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
• Tissue biopsy of the tumor:
  • A tissue biopsy of the nodule is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Papillary Fibroelastoma?

Some potential complications of Papillary Fibroelastoma include:

• Thromboembolism to the coronary arteries can result in sudden cardiac death
• Thromboembolism to the brain can lead to stroke
• Heart dysfunction due to large tumors

How is Papillary Fibroelastoma Treated?

Papillary Fibroelastoma may be treated through the following measures:

• A ‘wait and watch’ approach may be outlined by the healthcare provider, if the tumor is asymptomatic. However, there is an ongoing controversy whether to treat asymptomatic individuals or not
• Surgical removal of the tumor, if signs and symptoms are observed. In such cases, a complete excision can be curative
• Observation and periodic checkups to monitor the condition is recommended

How can Papillary Fibroelastoma be Prevented?

Presently, there are no specific methods or guidelines to prevent Cardiac Papillary Fibroelastoma (CPF).

What is the Prognosis of Papillary Fibroelastoma? (Outcomes/Resolutions)

• The prognosis of Papillary Fibroelastoma depends on the severity of the underlying signs and symptoms and the associated conditions (if any)
• Nevertheless, the prognosis is good when the tumor can be surgically removed

Additional and Relevant Useful Information for Papillary Fibroelastoma:

The following article link will help you understand other heart conditions:

http://www.dovemed.com/healthy-living/heart-center/