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Cor Triatriatum

 Cor Triatriatum


DISEASE OVERVIEW

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.

SIGNS & SYMPTOMS

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).

CAUSES

Cor triatriatum results from the incomplete absorption of the common pulmonary vein, which is normally reabsorbed during the development of a fetus and becomes a part of the left atrium.

DISORDERS WITH SIMILAR SYMPTOMS

Symptoms of the following disorders can be similar to those of cor triatriatum. Comparisons may be useful for a differential diagnosis:

Atrial Septal Defects are common congenital heart defects characterized by the presence of a small opening between the two atria of the heart. This defect leads to an increase in the workload on the right side of the heart, and excessive blood flow to the lungs. The symptoms, which may occur during infancy, childhood, or adulthood, can vary greatly and depend on the severity of the defect. The symptoms tend to be mild at first and may include difficulty breathing (dyspnea), increased susceptibility to respiratory infections, abnormal bluish discoloration of the skin and/or mucous membranes (cyanosis). Some people with Atrial Septal Defects may be at increased risk for the formation of blood clots that can travel to the major arteries of the blood system (embolism). (For more information on this disorder, choose “Atrial Septal Defects” as your search term in the Rare Disease Database.)

Ventricular Septal Defects (Cor Triloculare Biventricularis) are a group of common congenital heart defects characterized by the absence of one ventricle. Infants with this defect have 2 atria and 1 large ventricle. Symptoms of these defects which are similar to Atrioventricular Septal Defect may include an abnormal rapid rate of breathing (tachypnea), wheezing, a rapid heartbeat (tachycardia), and an abnormally enlarged liver (hepatomegaly). Ventricular Septal Defects can also cause the excessive accumulation of fluid around the heart leading to congestive heart failure. (For more information on this disorder, choose “Ventricular Septal Defects” as your search term in the Rare Disease Database.)

Atrioventricular Septal Defect is a rare congenital heart defect characterized by the improper development of the heart’s valves and septa. Symptoms may include difficulty breathing (dyspnea), a bluish discoloration of the skin and mucous membranes (cyanosis), excessive accumulation of fluid in the lungs (pulmonary edema), and/or congestive heart failure. Other symptoms may include poor feeding habits, abnormally rapid breathing (tachypnea), excessive sweating (hyperhidrosis), and/or an abnormally rapid heartbeat (tachycardia). (For more information on this disorder, choose “Atrioventricular Septal Defect” as your search term in the Rare Disease Database.)

Cor Triloculare Biatriatum is an extremely rare congenital heart defect characterized by the absence of one ventricle. Infants with this defect have two atria and one large ventricle. The symptoms are similar to those of Atrioventricular Septal Defect and include breathing difficulties (dyspnea), excessive accumulation of fluid in the lungs and around the heart (pulmonary edema), and/or a bluish discoloration of the skin and mucous membranes (cyanosis). Other symptoms may include poor feeding habits, abnormally rapid breathing (tachypnea), and/or an abnormally rapid heartbeat (tachycardia).

Mitral Valve Stenosis is a rare heart defect that may be present at birth (congenital) or acquired. In the congenital form, the symptoms vary greatly and may include coughing, difficulty breathing, heart palpitations, and/or frequent respiratory infections. In acquired Mitral Valve Stenosis, the symptoms may also include weakness, abdominal discomfort, chest pain (angina), and periodic loss of consciousness.

DIAGNOSIS

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.

STANDARD THERAPIES

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.

TYPE OF DOCTOR AND DEPARTMENT: Heart Specialist CAN DIAGNOSE THIS DISEASE.

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