Tetralogy of Fallot: Late outcome after repair and surgical implications

Cardiac surgery has transformed the outcome for patients with tetralogy of Fallot. Repair has conveyed excellent long-term results with most patients remaining well and leading normal lives. However, there are problems with late morbidity and mortality primarily due to right ventricular dysfunction, exercise intolerance, arrythmia, and sudden cardiac death. There has been a dynamic shift in our surgical approach to managing patients with tetralogy over the past 5 decades. This in part accounts for persisting difficulties in predicting late outcome for evry single patient with repaired tetralogy of Fallot. There are, however, several confounding variables, influencing long-term outcome for these patients, namely the underlying anatomical substrate, age at repair, surgical approach to repair, and residual hemodynamic abnormalities. It is gratifying to see that recent knowledge accumulated from long-term follow-up studies is influencing contemporary surgical practice. Individualized strategies aiming to minimize the potential for free pulmonary regurgitation, and the long-term detrimental effects associated with it, need to continue to develop. Preservation of right ventricular and pulmonary valve function combined with early restoration of normal pulmonary blood flow are likely to convey an even better long-term outlook for these patients. Further follow-up studies with assessment of bi-venticular function, however, are needed in both our older and contemporary cohorts with repaired tetralogy of Fallot. Copyright 2000 by W.B. Saunders Company