Impact of Right Ventricular Pressure Load After Repair of Tetralogy of Fallot

J Am Heart Assoc. 2022 Apr 5;11(7):e022694. doi: 10.1161/JAHA.121.022694. Epub 2022 Mar 18.


Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow-up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow-up of 10.1 (0.1-12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00-1.06; P=0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47-9.27; P=0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15-30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

Keywords: magnetic resonance imaging; prognosis; right ventricular pressure overload; strain; tetralogy of Fallot.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Cardiac Surgical Procedures* / adverse effects
  • Cardiac Surgical Procedures* / methods
  • Child
  • Humans
  • Prospective Studies
  • Pulmonary Valve Insufficiency* / diagnostic imaging
  • Pulmonary Valve Insufficiency* / etiology
  • Pulmonary Valve Insufficiency* / surgery
  • Pulmonary Valve* / diagnostic imaging
  • Pulmonary Valve* / surgery
  • Retrospective Studies
  • Tetralogy of Fallot*
  • Treatment Outcome
  • Ventricular Pressure
  • Young Adult