Tricuspid annular peak systolic velocity (s′) in children and young adults with pulmonary artery hypertension secondary to congenital heart diseases, and in those with repaired tetralogy of fallot: Echocardiography and MRI Data

Background: Tricuspid annular peak systolic velocity (S′), as an echocardiographic index to assess right ventricular (RV) systolic function, has not been investigated thoroughly in children and young adults with repaired tetralogy of Fallot (TOF) and pulmonary artery hypertension secondary to congenital heart disease (PAH-CHD). Methods: S′ values in patients with TOF (n = 183) and PAH-CHD (n = 55) were compared with those in normal subjects. S′ values were compared with RV ejection fraction and RV end-diastolic volume index (RVEDVi) determined by magnetic resonance imaging. Results: S′ values became significantly reduced in PAH-CHD patients after 10.4 years of age and after 13.6 years of age in patients with TOF compared with the lower boundary of the ±2-SD interval of normal subjects. Significant positive correlations between S′ and RV ejection fraction were seen in patients with TOF (r = 0.66, P <.001) and those with PAH-CHD (r = 0.82, P <.001). Significant negative correlations between S′ and RVEDVi were also seen in patients with repaired TOF (r = -0.29, P =.002) and in those with PAH-CHD (r = -0.59, P <.001). Conclusions: Although initially preserved, in this prospective study, impaired S′ values with increasing age were found in patients with repaired TOF and PAH-CHD. Persistent pressure overload in patients with PAH-CHD as well as volume overload in those with repaired TOF might lead to systolic RV functional impairment and increased RVEDVi. The validity of S′ data was supported by magnetic resonance imaging data (RVEDVi and RV ejection fraction).