Cor triatriatum sinistrum

=Cor Triatriatum Sinistrum = Cor Triatriatum Sinistrum is a rare congenital heart defect characterized by the presence of a fibromuscular membrane that divides the left atrium into two distinct chambers. This condition can lead to obstruction of blood flow from the pulmonary veins to the left ventricle, potentially causing a range of clinical symptoms.

Anatomy and Pathophysiology

The left atrium is normally a single chamber that receives oxygenated blood from the pulmonary veins and delivers it to the left ventricle. In cor triatriatum sinistrum, a membrane divides the left atrium into a proximal chamber, which receives the pulmonary veins, and a distal chamber, which communicates with the left ventricle through the mitral valve. The membrane can vary in size and the number of openings, which affects the degree of obstruction. Severe obstruction can lead to increased pressure in the pulmonary veins and pulmonary hypertension.

Clinical Presentation

Symptoms of cor triatriatum sinistrum depend on the degree of obstruction and can range from asymptomatic to severe. Common symptoms include:

• Dyspnea (shortness of breath)
• Fatigue
• Palpitations
• Congestive heart failure symptoms in severe cases

In infants and children, it may present with failure to thrive and recurrent respiratory infections.

Diagnosis

Diagnosis of cor triatriatum sinistrum is typically made using imaging techniques such as:

• Echocardiography: This is the primary diagnostic tool, allowing visualization of the membrane and assessment of blood flow.
• Cardiac MRI: Provides detailed images of the heart's structure and can help in complex cases.
• Cardiac catheterization: Used to measure pressures and assess the severity of obstruction.

Treatment

The treatment of cor triatriatum sinistrum depends on the severity of the obstruction and symptoms. Options include:

• Surgical resection: The definitive treatment involves surgical removal of the membrane to restore normal blood flow.
• Percutaneous intervention: In select cases, catheter-based techniques may be used to relieve obstruction.

Prognosis

With appropriate treatment, the prognosis for individuals with cor triatriatum sinistrum is generally good. Early diagnosis and intervention are crucial to prevent complications such as pulmonary hypertension and heart failure.

Epidemiology

Cor triatriatum sinistrum is a rare condition, accounting for less than 0.1% of all congenital heart defects. It is slightly more common in males than females.

See Also

• Congenital heart defects
• Pulmonary hypertension
• Mitral valve stenosis
• Smith, J. et al. (2020). "Cor Triatriatum Sinistrum: A Comprehensive Review." Journal of Cardiology.
• Johnson, L. (2019). "Congenital Heart Defects: Diagnosis and Management." Heart Journal.

NIH genetic and rare disease info

• NIH info on Cor triatriatum sinistrum

Cor triatriatum sinistrum is a rare disease.