Long-Term Survival in Repaired TOF is Excellent

A new study shows that the 25-year survival rate of surgical tetralogy of Fallot (TOF) patients is primarily influenced by the coexistence of an associated genetic condition.

Researchers at Emory University (Atlanta, GA, USA), the University of Minnesota (UMN; Minneapolis, USA), and other institutions conducted a retrospective cohort study involving 3,283 patients (56.4% men) enrolled in the Pediatric Cardiac Care Consortium (PCCC) between 1982 and 2003 and whom survived surgical repair of simple TOF. Main outcomes and measures were transplant-free survival during the early (less than six years) and late phase following surgical repair, adjusted for era and patient characteristics.

The results revealed that one-year survival rate following TOF repair was 98.6%, decreasing to 97.8% at five years; 97.1% at 10 years; 95.5% at 20 years; and 94.5% at 25 years, with an early steep mortality hazard increase peaking shortly after repair, followed by a late, gradual hazard increase. Increased mortality risks were found in non-valve-sparing operations and staged repair. Genetic abnormalities were associated with increased mortality risk in both early and late post-surgical phases. The study was published on December 19, 2018, in JAMA Cardiology.

“Long-term transplant-free survival beyond the first six years is influenced primarily by the coexistence of an associated genetic condition. Within the 25-year follow-up period, most deaths were associated with the underlying diagnosis of TOF and mediated by arrhythmias and congestive heart failure,” concluded lead author Clayton Smith, MD, of Emory University, and colleagues. “Staged repair and non–valve-sparing operations were negatively associated with survival in the early post-repair phase. These data are important for patients with repaired TOF and their caretakers, and may guide surgical strategies for optimizing the long-term outcomes of this population.”

TOF is a cyanotic congenital heart disease characterized by four main findings; pulmonary stenosis, a narrowing of the exit from the right ventricle; a ventricular septal defect; right ventricular hypertrophy; and an overriding aorta, which allows blood from both ventricles to enter the aorta. Symptoms include episodes of bluish color to the skin. Other symptoms may include a heart murmur, finger clubbing, and easy tiring upon breastfeeding. The cause is typically not known.

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Emory University
University of Minnesota