Cor triatriatum is a rare congenital heart disease in which the left atrium or right atrium is divided into two parts by a fold of tissue, a membrane, or a fibromuscular band. It may be associated with major congenital cardiac lesions such as common atrioventricular canal. The cause is not clear. Usual symptoms are exertional dyspnea, cough, hemoptysis, orthopnea and palpitation. Echocardiography, transesophageal echocardiography (TEE) and cardiac MRI can tell the diagnosis of cor triatriatum. Surgery is the main treatment opinion. Prognosis of cor triatriatum depends on whether the surgery has been done.
What are the symptoms of cor triatriatum?
Signs and symptoms depend upon the size of the opening in the septum and the manifestations of associated congenital cardiac defects. In most patients, the opening is severely restrictive and produces symptoms similar to those of severe mitral stenosis. Usual symptoms are the following:
- Exertional dyspnea
- Effort intolerance and easy fatigability
- Signs of systemic embolism
Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.
Who is at highest risk?
Like many congenital heart disease, the cause of cor triatriatum is not clear. Clinical studies suggest that both genetic and environmental factors may play a role during the pregnancy.
- Viral infection
- Poor nutrition
- Genetic disorder
- Echocardiography and transesophageal echocardiography (TEE): Echocardiography is the most commonly used imaging technique for the diagnosis of cor triatriatum. It can assess atrial morphology and characteristic anatomic relations, determinate resultant flow disturbances, and detect associated anomalies. Transesophageal echocardiography (TEE) is frequently needed to precisely define the anatomy of the membrane, its relation to other structures, and the pulmonary venous drainage pattern.
- Chest x-ray: An x-ray image of chest allows the doctor to check the size and shape of your heart to determine whether the atrium is enlarged. And it also helps the doctor check the condition of your lungs. Patients with cor triatriatum may show enlarged atrium and pulmonary congestion on an x-ray.
- Cardiac MRI and CT: Cardiac MRI and CT can create pictures of the ventricles, atrium, valves and major blood vessels. It can help doctors analyse the structure and function of the heart and decide the treatment protocols for the patient.
- Cardiac catheterization: In a catheter room, the doctor threads a thin tube through a blood vessel in the patient’s arm or groin to an artery in the heart and injects dye to see the heart and the arteries on an x-ray. It can supply detailed information about the severity of obstruction to left ventricular filling and assess magnitude of pulmonary hypertension.
When to seek urgent medical care?
Call your health care provider if symptoms of cor triatriatum develop. If you experience either of the following symptoms, seeking urgent medical care as soon as possible:
The main treatments for cor triatriatum are medicines and surgery.
- Medicines: Medicines are used to control ventricular rate in patients with atrial fibrillation, or prevent from deep vein thrombosis and pulmonary embolism in those with right-sided heart failure, or treat arrhythmia, or prepare for the surgery.
- Surgery: A common approach is to resect the membrane completely and close the atrial septum with a pericardial patch. Surgical resection of the accessory membrane has been successful in symptomatic patients with cor triatriatum. At the same time, associated congenital defects need to be corrected.
Diseases with similar symptoms
- Pulmonary vein stenosis
- Pulmonary veno-occlusive disease
- Supravalvular mitral ring
- Atrial tumors, such as atrial myxoma
- Inferior vena cava syndrome
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What to expect (Outlook/Prognosis)?
The prognosis of cor triatriatum depends on whether the surgery has been done. The outcomes of infants or children, who are untreated with symptomatic cor triatriatum sinistrum associated with complex congenital cardiac defects, are very poor. On the contrary, adults with isolated cor triatriatum after surgery have good outcomes.
Although the cause is unknown, doctors found the following measure may desrease the risk for pregnant woman to have a baby with cor triatriatum.
- Avoidance of viral infection
- Good nutrition in pregnancy
- Screening test and regular check for the people with genetic disorder