The Unnatural History of Tetralogy of Fallot: Prospective Follow-Up of 40 Years After Surgical Correction

Study Questions:

What are the long-term survival and outcomes beyond 30 years after surgical correction of tetralogy of Fallot (TOF)?

Methods:

A single-center longitudinal cohort study was performed of 144 patients with TOF who underwent surgical repair at <15 years of age between 1968 and 1980. Patients returned every 10 years for investigation. At the third in-hospital investigation, patients underwent extensive testing including electrocardiography, Holter, echocardiography, cardiopulmonary exercise testing, N-terminal pro–B-type natriuretic peptide (NT-proBNP) measurement, cardiac magnetic resonance imaging with dobutamine stress testing, and the Short Form-36 questionnaire.

Results:

Cumulative survival was 72% for the 136 patients with available follow-up data, including 23 patients who died within 30 days of surgery. For those who survived their initial operative hospitalization, cumulative survival was 98% after 10 years, 96% after 20 years, 92% after 30 years, and 86% after 40 years. Event-free survival was 25%. Late mortality was due to heart failure and ventricular fibrillation. Seventy-two of 80 (90%) eligible survivors took part in the extensive late testing protocol. Exercise performance and aortic dimensions remained stable over time, while a decline in ventricular function was seen. Predictors of late mortality included a prior shunt operation (hazard ratio [HR], 2.9), low temperature during surgery (HR, 1.1), and early postoperative arrhythmias (HR, 2.5). Increase in QRS duration, decline in exercise tolerance, and ventricular dysfunction did not predict mortality. Placement of a transannular patch during the initial surgery was a predictor of late arrhythmias (HR, 4.0).

Conclusions:

While many patients develop arrhythmias or need for repeat operation, long-term survival is quite good after repair of TOF early in life.

Perspective:

This study describes not only long-term survival after repair of tetralogy, but detailed follow-up information on a cohort of patients undergoing repair between 1968 and 1980. Consistent with previous studies, the study showed previous shunt procedures to be a risk factor for late mortality. Interestingly, this study did not show increasing QRS duration as a risk factor for death or arrhythmia, although the sample size became somewhat small for long-term follow-up. Atrial arrhythmias appeared to be associated with increased risk for sudden death. This will require further investigation in larger cohorts. Overall, the long-term results for hospital survivors are excellent and only slightly lower than that seen in the general population. This is in the setting of the authors describing their center as not particularly aggressive in pulmonary valve replacement.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, 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, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Echocardiography/Ultrasound, Magnetic Resonance Imaging

Keywords: Exercise Tolerance, Tetralogy of Fallot, Follow-Up Studies, Ventricular Dysfunction, Ventricular Fibrillation, Death, Sudden, Disease-Free Survival, Risk Factors, Electrocardiography, Magnetic Resonance Imaging, Survivors, Dobutamine, Heart Failure, Peptide Fragments, Ventricular Function, Questionnaires, Arrhythmias, Cardiac, Natriuretic Peptide, Brain, Echocardiography, Exercise Test


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