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

• Cor Triatriatum Sinistrum
• CTS (Cor Triatriatum Sinister)
• Divided Left Atrium

What is Cor Triatriatum Sinister? (Definition/Background Information)

• Cor Triatriatum Sinister (CTS) results when the left atrium is divided into two compartments by a membrane. The membrane can vary in size and shape and may have one or more openings
• Severe cases of Cor Triatriatum Sinister usually present in infancy and are often associated with other heart defects. In less severe cases, the diagnosis may not be made until later in life
• The specific symptoms depend on the degree to which the membrane obstructs the flow of blood and whether or not there are additional heart defects
• Symptoms may range from mild shortness of breath during exercise to signs of heart failure and lung congestion. Some people with CTS may not have symptoms
• The treatment of Cor Triatriatum Sinister varies according to the severity of the symptoms. For symptomatic patients, surgery is generally considered the definitive course of therapy

(Source: Cor Triatriatum Sinister; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Cor Triatriatum Sinister? (Age and Sex Distribution)

• Cor Triatriatum Sinister is a rare congenital disorder. The presentation of symptoms may occur in infancy, if the abnormality is severe
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Cor Triatriatum Sinister? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Cor Triatriatum Sinister

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s 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 Cor Triatriatum Sinister? (Etiology)

The exact cause of Cor Triatriatum Sinister is not known at the present time.

What are the Signs and Symptoms of Cor Triatriatum Sinister?

The signs and symptoms of Cor Triatriatum Sinister are determined by the extent of obstruction in the left atrium. While those with mild obstruction may be asymptomatic, individuals with moderate to severe obstruction may exhibit the following signs and symptoms (during infancy):

• Shallow and rapid breathing
• Wheezing
• Coughing
• Chest congestion
• Enlarged heart
• Heart murmurs
• Pulmonary hypertension

Signs and symptoms in individuals diagnosed later in life (beyond infancy)

• Labored breathing
• Rapid heartbeats
• Fluid accumulation in the lungs
• Edema
• Recurrent respiratory tract infections

How is Cor Triatriatum Sinister Diagnosed?

Cor Triatriatum Sinister is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

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 Cor Triatriatum Sinister?

The complications of Cor Triatriatum Sinister may include:

• Pneumonia
• Susceptibility to endocarditis
• Congestive heart failure

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Cor Triatriatum Sinister Treated?

Treatment for Cor Triatriatum Sinister varies depending on the symptoms. The options may include:

• Diuretics
• Medications to change the force of the heart's contractions (inotropic medications)
• Medications to prevent clots from forming (anticoagulant therapy)
• Surgery

(Source: Cor Triatriatum Sinister; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How can Cor Triatriatum Sinister be Prevented?

• The cause and specific risk factors for Cor Triatriatum Sinister are not known. Hence, no guidelines or methods are presently available for its prevention
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Cor Triatriatum Sinister
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Cor Triatriatum Sinister? (Outcomes/Resolutions)

• The prognosis of Cor Triatriatum Sinister is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Cor Triatriatum Sinister:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/