Absent right superior vena cava with persistent left superior vena cava: implications and management

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Congenital absence of the right superior vena cava (ARSCVC) is a rare anomaly.
• Persistent left superior vena cava (PLSVC) can occur with ARSCVC.
• These anomalies are often associated with other cardiac defects.

Purpose of the Study:

• To report on seven cases of ARSCVC with PLSVC and normal situs in pediatric patients.
• To describe the clinical presentation, associated cardiac defects, and outcomes.
• To highlight necessary precautions for potential interventions.

Main Methods:

• Retrospective review of seven pediatric cases diagnosed at Children's Hospital of Pittsburgh.
• Diagnosis confirmed via autopsy in two cases and cardiac catheterization in five cases.
• Analysis of associated cardiac defects, surgical interventions, and electrocardiogram findings.

Main Results:

• All seven patients had associated cardiac defects, including ventricular septal defect, atrial septal defect, subaortic stenosis, pulmonary atresia, and patent ductus arteriosus.
• Five children diagnosed since 1966 via cardiac catheterization are living and well.
• Electrocardiograms showed a short P-R interval and leftward P wave axis in four patients, suggesting a low atrial focus, with no significant conduction issues.

Conclusions:

• ARSCVC with PLSVC in pediatric patients is consistently associated with cardiac malformations.
• Despite associated defects, patients can have favorable outcomes with appropriate management.
• Careful consideration of cardiac anatomy and potential conduction abnormalities is crucial for surgical and interventional procedures.