Cor triatriatum dexter, atrial septal defects, and pulmonary stenosis-a rare association

Area of Science:

• Cardiology
• Congenital Heart Disease
• Pediatric Cardiology

Background:

• Cor triatriatum dexter (CTD) is an exceptionally rare congenital cardiac malformation.
• It involves the division of the right atrium into two distinct chambers by an anomalous membrane.
• The reported incidence is approximately 0.1% of all congenital cardiac malformations.

Observation:

• The septation in CTD arises from the incomplete resorption of the right valve of the sinus venosus.
• This developmental anomaly leads to the formation of anterolateral and posteromedial portions within the right atrium.
• CTD can manifest and be diagnosed at any life stage, often discovered incidentally.

Findings:

• The primary finding in CTD is the presence of a dividing membrane within the right atrium.
• This anatomical variation results from a specific embryological failure during cardiac development.
• The condition's presentation varies, with incidental discovery being common.

Implications:

• Accurate diagnosis of CTD is crucial for appropriate clinical management.
• Understanding the embryological basis aids in diagnosing and managing this rare anomaly.
• Early or incidental detection facilitates timely intervention and monitoring of associated cardiac issues.