Pediatric Cor Triatriatum Clinical Presentation

Updated: Dec 18, 2020
  • Author: M Silvana Horenstein, MD; Chief Editor: Stuart Berger, MD  more...
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Cor triatriatum is essentially a form of left atrial inflow obstruction and presents with signs and symptoms of pulmonary venous obstruction.

Most patients present during infancy with a restrictive opening in the membrane. These infants usually present with evidence of low cardiac output, including pallor, diminished peripheral pulses, and tachypnea.

Feeding difficulties, poor weight gain, and respiratory distress are common.

Presentation later in life is less classic; however, when the patient becomes symptomatic, evidence of pulmonary venous obstruction predominates. In these patients, the membrane may become calcified with its orifice becoming smaller or the patient may develop mitral valve insufficiency. These patients may also present with arrhythmias secondary to an enlarged, hypertensive atrium.


Physical Examination

Despite the intracardiac defect, no characteristic murmur or pathognomonic physical characteristics are present in patients with cor triatriatum. This often leads to incorrect diagnosis of primary pulmonary vascular or parenchymal disease.

Signs of pulmonary venous obstruction and pulmonary hypertension are present. A right ventricular lift and accentuation of the pulmonary second sound are frequent and may be accompanied by an early diastolic murmur of pulmonary insufficiency. Rales may be present in the lung bases.

A soft, continuous murmur may be present due flow across the membrane. A murmur at the left sternal border is heard in patients with an atrial septal defect and a left-to-right shunt. A diastolic rumble of mitral stenosis at the apex is generally not heard with cor triatriatum.

Low cardiac output manifests as pallor, tachypnea, and diminished peripheral pulses.

Children are typically small, suffering from poor weight gain.

Patients presenting later in life may be dyspneic with a history of frequent pulmonary infections. They may have signs of right-sided heart failure, including distended peripheral veins and hepatomegaly. Signs and symptoms of pulmonary hypertension may be severe.