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

• Cardiac Fibrous Hamartoma
• Fibroelastic Hamartoma of Heart
• Fibroma of Heart

What is Cardiac Fibroma? (Definition/Background Information)

• Cardiac Fibroma is a very rare, connective tissue tumor of the heart, which is mostly observed in infants and children. This benign tumor is mostly present in the ventricles of the heart (lower heart chambers)
• Some tumors are seen in association with Gorlin syndrome (a genetic disorder), while many are observed to occur sporadically in a random fashion without an established cause
• Cardiac Fibroma can grow to large sizes unobserved or undetected. Some may be asymptomatic, while two-thirds of children with the tumor may show signs and symptoms. This may include cyanosis, arrhythmias, fainting, and even sudden death
• The diagnosis of Cardiac Fibroma may require a thorough physical checkup by the healthcare provider, imaging studies of the heart, and a few specialized tests to evaluate heart functioning. Since, these are congenital tumors, an ultrasound prenatal scan may help detect the tumor during the fetal stage
• The mainstay of treatment of Cardiac Fibroma is surgery to remove the tumor completely. In many cases, the prognosis of the condition is good with surgery

Who gets Cardiac Fibroma? (Age and Sex Distribution)

• The incidence of Cardiac Fibroma is unknown, but the condition occurs very infrequently. Only, 200 cases have been recorded in the medical literature
• Cardiac Fibromas are often diagnosed before age 1 year (a congenital presentation is often seen). The tumor is observed in children and infants. It is not commonly observed in adults
• Both males and females are affected and no gender preference is observed
• All races and ethnic groups are at risk for this tumor type

What are the Risk Factors for Cardiac Fibroma? (Predisposing Factors)

In majority of individuals, the risk factors for Cardiac Fibroma are unidentified.

• Although, about 1 in 30 individuals with Gorlin syndrome (a multisystem genetic disorder that causes the formation of benign and malignant tumors at various locations in the body) are known to be present with Cardiac Fibroma
• Occasionally, the condition is also associated with Beckwith-Wiedemann syndrome (a genetic disorder) and cleft lip and palate

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 Cardiac Fibroma? (Etiology)

The cause of development of Cardiac Fibroma is largely unknown or unexplained.

• Some cases are observed to be linked to Gorlin syndrome; a complex genetic disorder causing the formation of tumors in various parts of the body
• Research is currently being undertaken to identify the relevant causal factors

What are the Sign and Symptoms of Cardiac Fibroma?

Cardiac Fibroma is a slow-growing tumor that can cause heart electrical transmission defects and arrhythmias. The features of Cardiac Fibroma may include:

• It is almost always seen in the ventricle wall separating the right and left lower chambers, or in the ventricle muscle. The tumor is rarely seen at atrial locations (i.e., in the upper chamber)
• It is mostly single and well-circumscribed
• The average size of the tumor is 5 cm; they are usually round in shape

In about 35% of the individuals with Cardiac Fibroma, no signs and symptoms are generally observed.

• In such individuals, the tumor is diagnosed incidentally, while a health checkup is being performed for other medical conditions
• They may be also discovered due to abnormal heart sounds such as a heart murmur

In 65% of the individuals, the following signs and symptoms of Cardiac Fibroma, sometimes due to its large size, may be noted:

• Blood flow obstruction, especially into or out of the valves
• Valve function is affected leading to heart failure
• Bluish skin (cyanosis)
• Severe arrhythmias
• Dizziness and fainting
• Other obstructive symptoms may be present

How is Cardiac Fibroma Diagnosed?

The following tests and exams may be undertaken to diagnose Cardiac Fibroma:

• 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)
• Echocardiography
• Electrocardiogram (EKG): It is used to measure the electrical activity of the heart, to detect arrhythmias
• Electrophysiological studies of the heart to determine where arrhythmia is getting generated in the heart, is often helpful
• MRI scan of heart (with contrast studies) and CT scan of the heart
• Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
• Prenatal ultrasound may help reveal the presence of the tumor
• Tissue biopsy:
  • A biopsy of the tumor 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 additional studies, which may include immunohistochemical stains and molecular studies to assist in the diagnosis

Note: Most tumors are discovered incidentally, while a medical exam is being performed for other health conditions.

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 Cardiac Fibroma?

Some potential complications of Cardiac Fibroma may include:

• Severe arrhythmias
• Large tumors can cause obstructive signs and symptoms include congestive heart failure and cardiac dysfunction
• In about 33% of the cases, the tumor is known to cause sudden death

How is Cardiac Fibroma Treated?

Cardiac Fibroma may be treated through the following measures:

• In case of arrhythmias, anti-arrhythmic medication can be given. If the arrhythmia persists for a long period of time, then surgical treatment measures may be considered
• Cardiac Fibroma is generally treated through a surgical excision procedure. If surgery is difficult, then in some cases, a heart transplantation may be necessary
• Observation and periodic checkups to monitor the condition is recommended

How can Cardiac Fibroma be Prevented?

Presently, there are no specific methods or guidelines to prevent Cardiac Fibroma.

What is the Prognosis of Cardiac Fibroma? (Outcomes/Resolutions)

• The prognosis of Cardiac Fibroma is generally good on its surgical excision and removal
• The tumor is not known to regress spontaneously and surgery is usually required to treat the condition
• Severe complications, such as arrhythmias or large tumor size with obstructive signs and symptoms, can negatively affect the prognosis

Additional and Relevant Useful Information for Cardiac Fibroma:

• Beckwith-Wiedemann syndrome (BWS) is an inherited genetic disorder characterized by an abnormal overgrowth of the body parts

The following link can help you understand Beckwith-Wiedemann syndrome:

http://www.dovemed.com/diseases-conditions/beckwith-wiedemann-syndrome-bws/