Cor triatriatum is an extremely rare congenital (present at birth) heart defect. Normally, the human heart has four chambers of which two are the atria. These two are separated from each other by a partition (septum) called the atrial septum. The other two chambers, known as ventricles, are also separated by a septum. In cor triatriatum there is a small extra chamber above the left atrium of the heart. The pulmonary veins, returning blood from the lungs, drain into this extra "third atrium." The passage of blood from the lungs into the heart (left atrium and ventricle) is slowed by this extra chamber. Cor triatriatum may eventually lead to features of congestive heart failure and obstruction over time.
The symptoms of cor triatriatum vary greatly, depending on the size of the opening between the extra chamber and the left atrium. If the opening is small, symptoms usually develop early in infancy and probably will include abnormally rapid breathing (tachypnea), wheezing, coughing, and/or abnormal accumulation of fluid in the lungs (pulmonary congestion). Progressive enlargement of the heart (cardiomegaly) occurs and often results in congestive heart failure along with abnormally high pressure within the artery that leads to the heart from the lungs (pulmonary artery). Some newborns with cor triatriatum may also have abnormal heart sounds or heart murmurs.
In older people (after childhood), the symptoms of cor triatriatum may include abnormal swelling of areas of the body (generalized edema), pain and discomfort while breathing (dyspnea), an abnormally rapid heartbeat (tachycardia), and the excessive accumulation of fluid in the lungs (pulmonary congestion). Acute inflammation of the lungs (pneumonia) and bronchial tubes (bronchitis) may occur frequently and may lead to congestive heart failure. Individuals with cor triatriatum are also at increased risk for bacterial infection of the delicate membranes surrounding the heart (endocarditis).
Cor triatriatum is an extremely rare congenital heart condition that affects males and females in equal numbers. In the USA, this disorder accounts for an extremely small percentage (0.1% to 0.4%) of all infants with congenital heart disease.
The diagnosis of cor triatriatum is usually made by using imaging techniques such as magnetic resonance imaging (MRI) and echocardiography (EC). In another procedure known as a cardiac catheterization, a long fine tube (catheter) is inserted into a large vein and then channeled directly into the heart. This allows the physician to further identify heart defects and to determine the rates of blood flow through the heart. Angiography is also a very useful diagnostic procedure and allows the physician to view an enhanced x-ray of the heart. Children with cor triatriatum also have abnormal EKG patterns.
Infants with cor triatriatum should be referred to a hospital that can perform sophisticated diagnostic procedures and cardiovascular surgery. Most patients with cor triatriatum will require surgery at a young age, usually before the age of one year.
Prior to surgery, congestive heart failure associated with cor triatriatum may be managed by reducing fluid volume with diuretic drugs and, if necessary, the dietary restriction of fluids and salt. The drug digitalis may also be administered to decrease the heart rate and increase the strength of the heart's contractions. Oxygen therapy may also prove beneficial.
Because people with cor triatriatum are susceptible to bacterial infection of the membranes that surround the heart (endocarditis), any respiratory infection should be treated vigorously and early. Affected individuals should also be given antibiotics before invasive dental procedures (i.e., root canal or extractions) or other surgical procedures to help prevent potentially life-threatening infections.
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