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The following is a summary of “Long term outcome after surgical tetralogy of Fallot repair at young age: Longitudinal follow-up up to 50 years after surgery.,” published in the March 2025 issue of International Journal of Cardiology by Kauling et al.

This study aimed to prospectively evaluate the long-term clinical outcomes and quality of life in patients with Tetralogy of Fallot (ToF) following surgical repair. Despite advancements in treatment, the very long-term prognosis for these patients remains inadequately understood. A single-center, longitudinal cohort study was conducted, assessing 144 patients with ToF who underwent surgical repair before the age of 15 between 1968 and 1980, with follow-up evaluations every decade. A total of 66 patients (92% of the 72 eligible survivors) participated, with a median age of 48.5 years at the time of the study, and a median follow-up duration of 45 years (range 39-52 years). Cumulative survival at 50 years was 71%, excluding 30-day mortality, with event-free survival at a mere 9%. The most common complications were reinterventions (40%) and symptomatic arrhythmias (21%), although ventricular tachycardia (VT) was observed in only 7% of patients. 

Cardiac magnetic resonance imaging revealed a right ventricular ejection fraction of less than 45% in 45% of the cohort, and 42% exhibited diminished left ventricular function. The aortic root diameter increased over time, exceeding 40 mm in 45% of the patients. Additionally, 53% of patients showed reduced VO2max, though this decline remained stable over time. Notably, self-perceived quality of life remained stable throughout the follow-up period and was comparable to that of the general Dutch population. Predictive factors for mortality included early postoperative arrhythmias, preoperative low oxygen saturation in the left atrium, VT, and a decline in exercise capacity over time. 

Furthermore, a transannular patch was identified as a risk factor for both arrhythmias and the need for pulmonary valve replacement. Conversely, surgery performed after 1975 was protective against the need for pulmonary valve replacement. In conclusion, only 9% of patients with ToF are alive at 50 years post-surgery without experiencing a major event. Reintervention and symptomatic arrhythmias remain prevalent, though symptomatic VT is rare. Despite these challenges, exercise capacity and self-perceived quality of life appear to stabilize over time, offering valuable insights into the long-term care survivors of ToF.

Source: internationaljournalofcardiology.com/article/S0167-5273(25)00048-8/fulltext