Understanding Cor Triatriatum: Causes, Symptoms, Diagnosis, Treatment, and Prevention
There is a membrane or septum in the atrium that divides it into two chambers in cor triatriatum, a rare congenital heart defect. There is no clear cause for cor triatriatum, but it is believed to be caused by abnormal embryonic development during the early stages of fetal development. Occasionally, cor triatriatum may occur as part of a congenital heart disorder or as part of a genetic syndrome.
Cor triatriatum can cause a variety of symptoms depending on the size and location of the membrane within the atrium, and the degree of obstruction to blood flow. Common symptoms include:
Exertion-induced shortness of breath (dyspnea)
Fatigue and weakness
Palpitations (rapid or irregular heartbeat)
Difficulty feeding (in infants)
Poor weight gain (in infants)
Cyanosis (bluish discoloration of the skin and mucous membranes) in severe cases
Signs of heart failure, such as fluid retention (edema) and difficulty breathing when lying flat (orthopnea)
A combination of clinical evaluations, imaging tests, and diagnostic procedures is used to diagnose cor triatriatum:
A thorough physical examination may reveal signs of cor triatriatum, including heart murmurs, abnormal heart sounds, and other clinical findings.
In order to diagnose cor triatriatum, echocardiography (ultrasound of the heart) is used. This allows visualization of the abnormal membrane within the atrium and assessment of its size, location, and impact on blood flow.
Additional imaging tests may be performed to further evaluate the anatomy of the heart and confirm a diagnosis of cor triatriatum, such as cardiac magnetic resonance imaging (MRI) or computed tomography (CT).
Cor triatriatum is treated based on the severity of symptoms and the degree of blood flow obstruction caused by the abnormal membrane:
Patients with mild or moderate obstruction may be closely monitored with regular follow-up appointments to determine if their symptoms or cardiac function have changed.
In order to manage symptoms of heart failure and improve cardiac function, medications such as diuretics, angiotensin-converting enzyme inhibitors, and beta-blockers may be prescribed.
Symptomatic individuals or those with severe obstructions to blood flow may be advised to undergo surgical correction of cor triatriatum. In surgery, the abnormal membrane is removed or fenestrated (opened) to improve blood flow and relieve symptoms.
Because cor triatriatum is congenital, it cannot always be prevented. As a result, early detection and management of other congenital heart defects and genetic syndromes associated with cor triatriatum can reduce complications and improve outcomes. To assess the risk of cor triatriatum and other congenital abnormalities in future pregnancies, individuals with a family history of congenital heart defects or genetic syndromes may benefit from prenatal screening and genetic counseling.
The cor triatriatum is a rare congenital heart defect characterized by the presence of a membrane dividing the atrium into two chambers. In order to relieve symptoms, improve cardiac function, and prevent complications associated with cor triatriatum, early diagnosis, appropriate management, and, in some cases, surgical intervention are essential. A healthcare provider should be consulted if you or your child experience symptoms suggestive of cor triatriatum or are concerned about your heart health.
