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Predicting Survival in Repaired Tetralogy of Fallot
Quick Takes
- A risk score for mortality was developed using RV LGE extent, presence of LV LGE, RVEF, LVEF, BNP, peak exercise oxygen uptake, prior sustained atrial arrhythmia, and age ≥50 years. This score identified patients with a 4.4% annual risk of mortality.
- A risk score for ventricular arrhythmia was developed using RV LGE extent, presence of LV LGE, RVEF, LVEF, peak exercise oxygen update, BNP, akinetic RV outflow length, and RV systolic pressure. This score identified patients with a 3.7% annual risk of ventricular arrhythmia.
Study Questions:
What are risk factors for death and malignant ventricular arrhythmia in patients with repaired tetralogy of Fallot (rTOF)?
Methods:
Patients were prospectively enrolled at a single referral center. Consecutive patients were recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right ventricular (RV) and left ventricular (LV) fibrosis in addition to previously established risk factors. The primary endpoint was all-cause mortality.
Results:
A total of 550 patients (median age 32 years, 56% male) were enrolled, of which 27 died over a mean follow-up of 6.4 ± 5.8 years. Mortality was independently predicted by RV LGE extent, presence of LV LGE, RV ejection fraction (RVEF) ≤47%, LVEF ≤55%, B-type natriuretic peptide (BNP) ≥127 ng/L, peak exercise oxygen uptake ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the above factors differentiated a high-risk subgroup with a 4.4% annual risk of mortality. The secondary endpoint was a composite endpoint of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death, which occurred in 29 patients. A different weighted score was calculated with this group, using some of the above predictors as well as RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg. This score identified high-risk patients with a 3.7% annualized risk of ventricular arrhythmia. RV LGE was heavily weighted in both risk scores due to its strong relative prognostic value.
Conclusions:
The authors present a score integrating multiple weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death and may benefit from targeted therapy.
Perspective:
Multiple risk factors for adverse outcomes in adults with tetralogy have been proposed over the years, including RV and LV dysfunction, prolonged QRS duration, previous ventriculotomy, or palliative shunt procedure, among others. Our ability to noninvasively predict patients at highest risk for serious arrhythmias and sudden death remains limited. This manuscript proposes two risk scores, one predicting the primary endpoint of all-cause mortality, and the other predicting the risk of ventricular arrhythmia. Quantification of LGE on CMR imaging was an important component of both scores. These scores provide an additional means of risk prediction for adults with rTOF but require additional study with external validation.
Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Prevention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Quality Improvement, Heart Failure and Cardiac Biomarkers, Magnetic Resonance Imaging
Keywords: Arrhythmias, Cardiac, Death, Sudden, Cardiac, Diagnostic Imaging, Fibrosis, Gadolinium, Heart Defects, Congenital, Magnetic Resonance Imaging, Natriuretic Peptide, Brain, Risk Factors, Secondary Prevention, Stroke Volume, Tachycardia, Ventricular, Tetralogy of Fallot, Ventricular Dysfunction, Left, Ventricular Dysfunction, Right, Ventricular Fibrillation
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