Long-Term Management of Right Ventricular Outflow Tract Dysfunction in Repaired Tetralogy of Fallot
Published: November 21, 2024
- Tetralogy of Fallot (TOF) is the fifth most common form of congenital heart disease and the most encountered cyanotic lesion. Contemporary early survival following infant surgery is approximately 98% with 25-year survival of over 90%.
- Surgical reconstruction of the right ventricular outflow tract (RVOT) during TOF repair (rTOF) often results in pulmonary regurgitation, stenosis, or both. These anatomic and functional derangements trigger a complex, slowly progressive pathophysiologic cascade that ultimately results in adverse clinical outcomes, including heart failure, arrhythmias, and premature death.
- Long-term surveillance of patients with rTOF is critical to anticipate and proactively manage complications. Multimodality testing is recommended periodically, and its frequency is tailored to symptoms and severity of illness.
Supporting Materials
Recommended Reading
- 2018 AHA/ACC Guideline for the Management of Adults with Congenital Heart Disease
- 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy
- Evaluation and Management of Chronic Heart Failure in Children and Adolescents With Congenital Heart Disease
- Environmental Exposures and Pediatric Cardiology