Objective: Follow-up after tetralogy of Fallot (ToF) repair is directed to detect timely right ventricular (RV) dysfunction by following pulmonary regurgitation and global RV size, with little attention for the effective contribution of regional RV dysfunction. This study investigates the contribution of regional RV dysfunction on exercise capacity after ToF repair.
Methods: Forty-two patients were investigated with cardiac magnetic resonance imaging for regional RV dysfunction in relation to global RV function by functional quantification of the sinus and outflow part of the RV. The impact of regional and global RV dysfunction on clinical status was studied by exercise testing.
Results: Global RV function was lower than sinus function (ejection fraction (EF) 52±12% vs 58±10%, P<0.001), attributable to the adverse influence of right ventricular outflow tract (RVOT) dysfunction (EF 34±17%). Percent predicted peak VO2 correlated better with the RV sinus ejection fraction compared with the global RV ejection fraction (r=0.51, P=0.001 vs r=0.44, P=0.004). Multivariate analysis revealed the EF of RV sinus (β=0.34, 95% CI 0.07-0.61, P=0.013) and the extent of RVOT akinesia (β=-0.28, 95% CI -0.50; -0.06, P=0.015) as significant determinants of exercise capacity. Impaired exercise performance occurred in 43% of the patients, and was independently determined by the type of repair (transventricular vs transatrial: OR 6.0, 95% CI 1.31-17.3, P=0.02) by associating greater sinus and RVOT dysfunction.
Conclusion: Functional analysis of the RV components shows that exercise capacity after repair of ToF is better predicted by systolic function of the RV sinus as the extent of RVOT dysfunction commonly leads to underestimation of global RV function. This method of differential quantification of regional RV function might be more appropriate than assessment of global RV function during the long-term follow-up of repaired ToF patients.
Keywords: Congenital heart disease; Right ventricular function; Tetralogy of Fallot.