Cor triatriatum dexter with right ventricular hypoplasia: Role of multimodality imaging in decision making

Area of Science:

• Cardiology
• Congenital Heart Disease
• Pediatric Cardiology

Background:

• Cor Triatriatum Dexter (CTD) is a rare congenital anomaly arising from the failure of regression of the right-sided sinus venosus valve.
• This anomaly results in abnormal septation of the right atrium, leading to obstructive lesions affecting the right atrial and tricuspid valves.

Observation:

• A 10-month-old male presented with persistent hypoxia, a sign often associated with congenital heart defects.
• The patient had previously undergone balloon valvuloplasty for mild pulmonic valve stenosis, had a large secundum atrial septal defect, and a mildly hypoplastic right ventricle.

Findings:

• This case describes a late diagnosis of Cor Triatriatum Dexter (CTD) in a pediatric patient.
• The persistent hypoxia despite previous interventions suggests complex underlying cardiac pathology.

Implications:

• Late diagnosis of CTD can occur even in patients with known congenital heart defects and prior interventions.
• Understanding the varied presentations of CTD is crucial for timely diagnosis and management in pediatric cardiology.
• This case underscores the importance of considering rare anomalies in persistent or unexplained symptoms in children.