Cor triatriatum dextrum
Cor Triatriatum Dextrum[edit | edit source]
Cor Triatriatum Dextrum is a rare congenital heart defect characterized by the presence of a fibromuscular membrane that divides the right atrium into two distinct chambers. This condition is the right-sided counterpart to Cor Triatriatum Sinistrum, which affects the left atrium.
Anatomy and Pathophysiology[edit | edit source]
In cor triatriatum dextrum, the right atrium is divided by an abnormal membrane, which can lead to obstruction of blood flow from the superior vena cava (SVC) and inferior vena cava (IVC) into the right ventricle. This membrane is thought to be a remnant of the right valve of the sinus venosus, which normally regresses during fetal development.
Clinical Presentation[edit | edit source]
The clinical presentation of cor triatriatum dextrum can vary widely depending on the degree of obstruction caused by the membrane. Some patients may be asymptomatic, while others may present with symptoms of right-sided heart failure, such as:
- Fatigue
- Peripheral edema
- Ascites
- Jugular venous distension
In severe cases, the condition can lead to cyanosis and hypoxia due to impaired blood flow through the heart.
Diagnosis[edit | edit source]
Diagnosis of cor triatriatum dextrum is typically made using imaging techniques such as:
- Echocardiography: This is the most common and non-invasive method to visualize the membrane and assess its impact on blood flow.
- Cardiac MRI: Provides detailed images of the heart's structure and can help in assessing the severity of the obstruction.
- Cardiac catheterization: May be used to measure pressures within the heart chambers and confirm the diagnosis.
Treatment[edit | edit source]
The treatment of cor triatriatum dextrum depends on the severity of the symptoms and the degree of obstruction. Options include:
- Surgical resection: The definitive treatment involves surgical removal of the membrane to restore normal blood flow.
- Percutaneous intervention: In some cases, a catheter-based approach may be used to perforate or remove the membrane.
Prognosis[edit | edit source]
The prognosis for individuals with cor triatriatum dextrum varies. Surgical intervention often results in good outcomes, especially if performed before significant cardiac damage occurs. However, untreated severe cases can lead to complications such as right heart failure and arrhythmias.
Epidemiology[edit | edit source]
Cor triatriatum dextrum is an extremely rare condition, with only a few cases reported in the medical literature. It is often diagnosed incidentally during imaging for other conditions.
See Also[edit | edit source]
NIH genetic and rare disease info[edit source]
Cor triatriatum dextrum is a rare disease.
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Contributors: Prab R. Tumpati, MD
