Coronary artery disease in adults with tetralogy of Fallot
Alexander C Egbe1, Sindhura Ananthaneni1, Raja Jadav1
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
Insights
Coronary artery disease (CAD) affects 19% mild and 15% significant cases in adults with repaired Tetralogy of Fallot (TOF). Significant CAD independently increases mortality risk in these patients.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease
- Interventional Cardiology
Background:
- Limited data exists on coronary artery disease (CAD) outcomes in adults with repaired Tetralogy of Fallot (TOF).
- Understanding CAD prevalence and impact in this population is crucial for long-term management.
Purpose of the Study:
- To determine the prevalence of CAD in adults with repaired TOF.
- To describe CAD treatment strategies in this cohort.
- To evaluate the impact of CAD on long-term survival.
Main Methods:
- Retrospective review of the MACHD database (1990-2017).
- Inclusion of adults with repaired TOF who underwent aortic root/coronary angiography.
- Categorization into No CAD, Mild CAD (≤50% stenosis), and Significant CAD (>50% stenosis).
Main Results:
- 105 of 465 TOF patients (23%) had angiograms; mean age 47 years.
- Prevalence of mild CAD was 19% and significant CAD was 15%.
- Significant CAD was an independent risk factor for mortality (HR: 2.03, P=.022).
Conclusions:
- Mild and significant CAD prevalence in adults with repaired TOF are 19% and 15%, respectively.
- Significant CAD is an independent predictor of mortality in this population.
- Further research is needed for optimal diagnostic and revascularization strategies.
Background:
There are limited data about outcomes of coronary artery disease (CAD) in adults with repaired tetralogy of Fallot (TOF). The purpose of this study was to describe the prevalence and treatment of CAD in adults with TOF, and the impact of CAD on long-term survival.
Methods:
Retrospective review of MACHD database for adults with repaired TOF who underwent aortic root/selective coronary angiogram, 1990-2017. Patients were categorized into three groups: (1) No CAD defined as normal coronary angiogram; (2) Mild CAD defined as ≤50% stenosis in all vessels; and, (3) Significant CAD defined as >50% stenosis in any vessel.
Results:
We identified 105 (23%) of 465 TOF patients that had angiograms; mean age 47 ± 12 years. The prevalence of mild CAD and significant CAD was 19% (20 patients) and 15% (16 patients), respectively. Of these 16 patient with significant CAD, 9 (56%), 3 (19%), and 4 (24%) patients received guideline directed medical therapy, percutaneous coronary intervention, and coronary artery bypass grafting, respectively. Significant CAD was an independent risk factor for mortality (HR: 2.03, 95% CI 1.64-4.22, P = .022) after adjustment for differences in age, and prevalence of atrial fibrillation and renal dysfunction.
Conclusions:
Based on a review of a selected cohort of 105 TOF patients, the prevalence of mild CAD and significant CAD was 19% and 15%, respectively. Significant CAD was an independent risk factor for mortality. There is need for more research to determine optimal noninvasive diagnostic strategies and optimal patient selections and methods for revascularization.
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