Management of Fallot's Uncorrected Tetralogy in Adulthood: A Narrative Review

The majority of cyanotic congenital cardiac defects are caused by the tetralogy of Fallot. Some symptoms include a biventricular connection of the aortic root, right ventricular hypertrophy, blockage of the right ventricular outflow tract, and a ventricular septal defect. Our understanding of tetralogy of Fallot (TOF) has significantly advanced since it was first described in 1888, and early diagnosis has led to improved surgical management and increased life expectancy. Adults with unrepaired and repaired TOF present with a range of late complications, including heart failure, the need for re-interventions, and late arrhythmias. Right ventricular (RV) failure, often caused by chronic pulmonary regurgitation, is a significant cause of heart failure in patients with TOF. Current treatment options are limited, and mainstay surgical procedures such as pulmonary-valve replacement (PVR), trans-annular repair (TAR), or infundibular widening repair have not shown a significant reduction in preventing right ventricular (RV) failure or death. Here, we explain the mechanisms of RV failure in ToF, chronic pulmonary regurgitation, heart failure, and secondary polycythemia. HF management in untreated adults is discussed. The progression of the disease, as well as complications, are also discussed. The treatment plan and the need to investigate the best management approach for this unsolved problem are included. This review aims to fill the knowledge gaps and supply valuable information regarding mechanisms of RV failure, chronic pulmonary regurgitation, and secondary polycythemia. To summarize, a new combat strategy must be found to battle RVF, and a more profound vision of these mechanisms is required. If it is not corrected, it will be one of the future research lines that will contribute to designing more efficacious treatment techniques for adults with TOF.