Cor triatriatum masked by primary pulmonary hypertension

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Pulmonary Hypertension

Background:

• Right heart failure in infants can be challenging to diagnose.
• Primary pulmonary hypertension is a rare but serious condition.
• Congenital heart anomalies can present with varied clinical signs.

Observation:

• A 17-month-old boy presented with signs of right heart failure.
• Cardiac catheterization suggested primary pulmonary hypertension.
• Echocardiography revealed a left atrial membrane (cor triatriatum).

Findings:

• Postmortem examination confirmed cor triatriatum and primary pulmonary hypertension.
• Pulmonary venous obstruction was masked by reduced pulmonary blood flow.
• Standard pulmonary wedge pressure measurements were insufficient for diagnosis.

Implications:

• Cor triatriatum can mimic or coexist with primary pulmonary hypertension.
• Accurate diagnosis requires a comprehensive evaluation, including detailed imaging and potentially postmortem analysis.
• Understanding this diagnostic challenge is crucial for managing pediatric heart failure.