Kauling RM, Ünlütürk S, Cuypers JAAE, van den Bosch AE, Hirsch A, Pelosi C, Bowen DJ, Bogers AJJC, Helbing WA, Kardys I, Roos-Hesselink JW. Int J Cardiol. 2025 Mar 15;423:133005. doi: 10.1016/j.ijcard.2025.133005. Epub 2025 Jan 25.PMID: 39870118 Free article.
Take Home Points:
- While the long-term survival post-tetralogy of Fallot repair was favourable, event-free survival was uncommon.
- Symptomatic arrhythmias and ventricular dysfunction were prevalent, but ventricular tachycardia and sudden cardiac death are relatively rare.
- Despite the significant morbidities, the self-perceived quality of life in the study cohort was comparable to the general Dutch population.
Commentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch:
Tetralogy of Fallot (ToF) is one of the most common cyanotic congenital heart diseases, with surgical repair being the definitive treatment. While many of these patients underwent surgical repair, they are by no means cured. Early mortality has significantly improved over the decades, but long-term outcomes and morbidity remain areas of concern. This study provides a longitudinal follow-up of ToF patients up to 50 years post-repair, offering invaluable insights into survival, complications, and quality of life.
Study Design: This is a single-centre, longitudinal cohort study evaluating 144 patients who underwent ToF repair before the age of 15, between 1968 and 1980. The study examined survival, cardiac reintervention, cardiovascular complications, and quality of life. The study cohort was compared with the general Dutch population.
Key Findings: 66 survivors participated in the latest follow-up. The baseline characteristics are summarised in Table 1. The median follow-up was 45 (range 39-52) years, making this one of the longest longitudinal studies in this patient population.
The study revealed a 71% cumulative survival at 50 years (84% when excluding 30-day mortality), but only 9% of patients were event-free (Figure 1). Reinterventions (40%) and symptomatic arrhythmias (21%) were common, though ventricular tachycardia (VT) was rare (7%). Cardiac magnetic resonance imaging (CMR) revealed right ventricular ejection fraction (RVEF) <45% in 45% of patients and diminished left ventricular function in 42%. Aortic root dimensions significantly increased over time (p < 0.001), with aortic root dilatation (>40 mm) observed in 45% of patients. Exercise capacity (VO₂max) was reduced in 53% of patients but remained stable over time. Quality of life, assessed via the SF-36 questionnaire, was comparable to the general Dutch population, with some domains even scoring higher (Figure 2).
Predictors of mortality included early postoperative arrhythmias, pre-operative low oxygen saturation in the left atrium, VT, and declining exercise capacity. Transannular patch was associated with higher rates of arrhythmias and pulmonary valve replacement, while surgery post-1975 was found to be protective against PVR (supplementary Table 9).
Strengths:
- The study’s 50-year follow-up is unparalleled.
- The use of multimodal evaluations (echocardiography, CMR, exercise testing, and quality-of-life surveys) offers a holistic view of patient outcomes.
Limitations:
- The findings may not be generalisable to patients who underwent repair in the current era, where shunts, if ever needed, are smaller in calibre, and limited transannular patch surgery is very much the norm.
- With only 66 participants in the latest follow-up, the power to detect significant associations, especially for rare events like VT or aortic dissection, may be limited.
Discussion: This study showed that beyond early post-operative mortality, long-term survival in patients with ToF is relatively favorable. Expectedly, reintervention rates were high, primarily for pulmonary valve replacement (PVR) and arrhythmia management. However, the incidence of sustained ventricular tachycardia (VT) remained low (cumulative incidence of 7%); lower than the 14% reported in previously (reference). The estimated annual incidence of sudden cardiac death (SCD) in ToF patients was 0.2%, alleviating concerns about ventricular arrhythmias and SCD, which are major worries following ToF repair. Notably, symptomatic arrhythmias were more common in patients who underwent PVR, likely due to the hemodynamic burden of chronic pulmonary regurgitation before reintervention or more extensive surgical scarring from aggressive transannular patching.
Conclusions: This study reinforces the importance of multidisciplinary, lifelong care for patients with ToF and provides a foundation for counselling and managing this growing ACHD population.
Reference :
P. Khairy, J. Aboulhosn, M.Z. Gurvitz, A.R. Opotowsky, F.-P. Mongeon, J. Kay, et al., Arrhythmia burden in adults with surgically repaired tetralogy of Fallot, Circulation 122 (2010) 868–875
