Untreated atrial septal defect after intracardiac repair for tetralogy of Fallot

Area of Science:

• Cardiology
• Congenital Heart Disease
• Adult Congenital Heart Surgery

Background:

• Tetralogy of Fallot (TOF) is a complex congenital heart defect often repaired in childhood.
• Surgical repair of TOF typically involves addressing ventricular septal defects and right ventricular outflow tract obstruction.
• Long-term follow-up of TOF repair patients is crucial for identifying residual or new cardiac issues.

Observation:

• A 50-year-old male, previously repaired for Tetralogy of Fallot in early childhood via right ventriculotomy, presented for pulmonary valve replacement.
• During the pulmonary valve replacement procedure, an untreated atrial septal defect (ASD) was incidentally discovered.
• Intraoperative findings suggested the initial TOF repair was performed solely through right ventriculotomy, with the ASD closure potentially coinciding with the pulmonary valve replacement.

Findings:

• The patient had an undiagnosed atrial septal defect (ASD) that persisted despite a prior Tetralogy of Fallot (TOF) repair.
• The surgical approach for the initial TOF repair may have facilitated the later identification and management of the ASD.
• Pulmonary valve replacement in adulthood revealed a previously unrecognized congenital anomaly.

Implications:

• This case underscores the necessity of comprehensive cardiac evaluation in adults with a history of congenital heart disease, even after initial successful repairs.
• The findings suggest that certain surgical techniques for TOF repair might inadvertently mask or complicate the diagnosis of coexisting intracardiac defects like ASDs.
• Long-term surveillance protocols for adult congenital heart disease patients should consider the potential for late-presenting or previously undetected anomalies.