Nephron

Cystic Tumor of Atrioventricular Node

Article
Heart & Vascular Health
Diseases & Conditions
+1
Contributed byKrish Tangella MD, MBAJan 09, 2019

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

  • Congenital Polycystic Tumor of AV Node
  • Cystic Tumour of the Atrioventricular Nodal Region
  • Mesothelioma of AV Node

What is Cystic Tumor of Atrioventricular Node? (Definition/Background Information)

  • Cystic Tumor of Atrioventricular Node (CTAVN) is a benign congenital tumor that is located in the heart atrium, near the atrioventricular node. The tumor has cystic configuration as the name suggests (i.e., it is present as a cyst)
  • The atrioventricular node (AV node) is an important component of the heart electrical conduction system. It allows electrical signals initiated by the sinoatrial node, to be distributed to the heart ventricles (lower chambers). Any disruption in the heart’s electrical system can be serious and life-threatening
  • The tumor may be diagnosed in childhood or when the individual is well into adulthood. Cystic Tumors of Atrioventricular Node are known to result in sudden death due to complete heart block (that occurs in a majority of individuals) and/or severe arrhythmias
  • Often small, multicystic tumors are present that are less than a few cm in size. The tumors may cause signs and symptoms that include palpitations, chest pain, shortness of breath, and fainting
  • A complete surgical excision and removal of Cystic Tumor of Atrioventricular Node (if possible) can result in a good prognosis. In a minority of individuals, severe symptoms causing sudden cardiac deaths are known to occur

Who gets Cystic Tumor of Atrioventricular Node? (Age and Sex Distribution)

  • Cystic Tumor of Atrioventricular Node is a rare tumor that has a congenital origin. However, it may not be diagnosed at birth or in infancy
  • A wide age range is observed - from between birth to 78 years; mean age of diagnosis is 38 years. Hence, both children and adults are diagnosed with the tumor. Some tumors are discovered during an autopsy
  • Both males and females are affected, though a strong predilection for females is observed (female-male ratio is 3:1)
  • No racial or ethnic preference is noted

What are the Risk Factors for Cystic Tumor of Atrioventricular Node? (Predisposing Factors)

Currently, no clearly established risk factors are noted for the development of Cystic Tumor of Atrioventricular Node.

  • Studies have indicated that about 10% of the individuals may have midline field defects (defects of congenital origin that occur along the central vertical body axis)
  • 75% of the cases are noted in females, and hence, they are at a higher risk for Cystic Atrioventricular Node Tumor

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 Cystic Tumor of Atrioventricular Node? (Etiology)

The exact cause and mechanism of development of Cystic Tumor of Atrioventricular Node is unknown. The tumor is believed to have a congenital origin.

What are the Signs and Symptoms of Cystic Tumor of Atrioventricular Node?

Cystic Tumor of Atrioventricular Node is a unique type of tumor; such tumor types are not seen elsewhere in the body. The signs and symptoms of the tumor may include:

  • Heart block:
    • Obstruction in the heart’s electrical system leading to abnormal, especially reduced heart beats (due to disrupted electrical signal); or, it may be described as an electrical conduction defect
    • Over 65% of the individuals may have a total heart block; 15% may present with partial AV block
  • Conduction abnormalities can cause arrhythmias leading to:
    • Palpitations
    • Chest pain
    • Shortness of breath
    • Dizziness and fainting; lightheadedness
  • The tumors may be small in size; size ranging from 2 mm to 2 cm
  • Generally, multicystic tumors are observed

In many cases, the tumor is diagnosed in later years (adulthood); thus, the congenital origin is missed. The diagnosis may also occur during an autopsy.

How is Cystic Tumor of Atrioventricular Node Diagnosed?

The following tools may be used towards establishing a diagnosis of Cystic Tumor of Atrioventricular Node:

  • 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 or ECG): 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
  • Blood tests to look for any trigger factors that causes arrhythmias:
    • Electrolyte levels
    • Complete blood count
    • Thyroid hormone levels
    • Use of certain medication or drug
  • MRI scan and CT scan of the heart
  • Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
  • Tissue biopsy of the tumor:
    • 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, molecular studies, and electron microscopy 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 Cystic Tumor of Atrioventricular Node?

Complications due to Cystic Tumor of Atrioventricular Node could include:

  • Total heart block causing severe electrical abnormalities in the heart
  • Severe cardiac arrhythmias
  • Associated heart attack and stroke
  • Sudden cardiac death, which is complication of severe arrhythmia

How is Cystic Tumor of Atrioventricular Node Treated?

The treatment measures for Cystic Tumor of Atrioventricular Node may include the following:

  • Complete surgical excision of Cystic Tumor of AV Node, where possible, is the treatment of choice
  • Additionally, anti-arrhythmic medications are used to treat cardiac arrhythmias
  • Pacemaker implantation may be necessary in some cases

How can Cystic Tumor of Atrioventricular Node be Prevented?

  • Current medical research has not established a way of preventing the formation of Cystic Tumor of Atrioventricular Node
  • The possibility of cystic tumor should be eliminated in individuals, who have a history of heart block and/or arrhythmias, through imaging techniques

What is the Prognosis of Cystic Tumor of Atrioventricular Node? (Outcomes/Resolutions)

  • The prognosis of Cystic Tumor of Atrioventricular Node is good with early diagnosis and prompt medical treatment (surgical removal of the tumor), in a majority of individuals
  • However, most of the individuals are known to experience from partial-to-complete heart block and severe arrhythmias. The condition is known to cause sudden death, even though the tumor is benign
  • 10% of the individuals are known to die without a previous history of heart block

Additional and Relevant Useful Information for Cystic Tumor of Atrioventricular Node:

Please visit our Heart & Vascular Health Center for more physician-approved health information:

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

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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