Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort

Objective: Patients with repaired tetralogy of Fallot (TOF) experience increased rates of mortality and morbidity in adulthood. This study was designed to identify risk factors for death and ventricular tachycardia (VT) in a large contemporary cohort of patients with repaired TOF. Methods: Subjects with repaired TOF from four large congenital heart centres in the USA, Canada and Europe were enrolled. Clinical, ECG, exercise, cardiac magnetic resonance (CMR) and outcome data were analysed. Results: Of the 873 patients (median age 24.4 years), 32 (3.7%) reached the primary outcome (28 deaths, 4 sustained VT; median age at outcome 38 years; median time from CMR to outcome 1.9 years). Cox proportional-hazards regression identified RV mass-to-volume ratio ≥0.3 g/mL (HR, 5.04; 95% CI 2.3 to 11.0; p<0.001), LV EF z score<−2.0 (HR, 3.34; 95% CI 1.59 to 7.01; p=0.001), and history of atrial tachyarrhythmia (HR, 3.65; 95% CI 1.75 to 7.62; p=0.001) as outcome predictors. RV dysfunction was predictive of the outcome similar to LV dysfunction. In subgroup analysis of 315 subjects with echocardiographic assessment of RV systolic pressure, higher pressure (HR 1.39; 95% CI 1.19 to 1.62; p<0.001) was associated with death and sustained VT independent of RV hypertrophy and LV dysfunction. Conclusions: RV hypertrophy, ventricular dysfunction and atrial tachyarrhythmias are predictive of death and sustained VT in adults with repaired TOF. These findings may inform risk stratification and the design of future therapeutic trials

The effects of pregnancy on right ventricular remodeling in women with repaired tetralogy of Fallot.

Objectives The aim of this study was to better understand the quantitative volumetric changes associated with pregnancy in women with repaired tetralogy of Fallot (TOF), utilizing sequential cardiovascular magnetic resonance (CMR) imaging. Background An increasing number of women with repaired TOF are reaching childbearing age. Limited echocardiographic studies suggest accelerated remodeling of the right ventricle (RV) in women with repaired TOF after pregnancy. Methods Sequential CMRs from a group of women with repaired TOF who completed pregnancy and from a matched comparison group of nulliparous women with repaired TOF were evaluated. The two groups were matched according to baseline QRS duration, RV end-diastolic volume (EDV), age at CMR and time between CMRs. Longitudinal change of CMR parameters was compared between the groups. Results Thirteen women (mean age 26.6 ± 7.4 years) with repaired TOF who completed pregnancy and 26 nulliparous women with repaired TOF (mean age 22.6 ± 8.0 years) were included in this analysis. The rate of increase of RV EDV in the pregnancy group was higher than the comparison group (4.1 ± 1.1 ml/m2/year vs. 1.6 ± 0.6 ml/m2/year, p = 0.07). RV EF did not change significantly in either group. No definitive interaction between degree of pulmonary regurgitation and increase of RV EDV was identified. Conclusions Women with repaired TOF who have completed pregnancy appear to experience an accelerated rate of right ventricular remodeling, defined as an increase in end-diastolic volume; however RV systolic function does not deteriorate. Further investigations with a prospective study design, larger cohorts, and longer follow-up are needed to confirm these initial observations. © 2012 Elsevier Ireland Ltd

Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair

Patients with repaired tetralogy of Fallot (TOF) are followed serially by cardiac magnetic resonance (CMR) for surveillance of RV dilation and dysfunction. We sought to define the prevalence of progressive RV disease and the optimal time interval between CMR evaluations. Candidates were selected from a multicentre TOF registry and were included if ≥2 CMR studies performed ≥6 months apart were available without interval cardiovascular interventions. Patients with 'disease progression' (defined as increase in RV end-diastolic volume index (RVEDVi) ≥30 mL/m(2), decrease in RVEF ≥10% or decrease in LVEF ≥10%) were compared with those with 'disease non-progression' (defined as RVEDVi increase ≤5 mL/m(2), RVEF decrease ≤3% and LVEF decrease ≤3%). A total of 849 CMR studies in 339 patients (median age at first CMR 23.6 years) were analysed. Over a median interval of 2.2 years between CMR pairs, RVEDVi increased 4±18 mL/m(2) (p <0.001), RV end-systolic volume index increased 3±13 mL/m(2) (p <0.001), RVEF decreased 1%±6% (p=0.02) and LVEF decreased 1%±6% (p=0.001). Disease progression was observed in 15% (n=76) and non-progression in 26% (n=133). There were no significant differences between those with and without progression in baseline demographic, anatomic, ECG, exercise or baseline CMR characteristics. The optimal time interval between CMR studies for detection of progression was a 3-year interval (63% sensitivity, 65% specificity, area under the receiver operating characteristic curve 0.65). Although progressive RV dilation and decline in biventricular systolic function occur at a slow pace in the majority of adults with repaired TOF, 15% of patients experience rapid disease progression. The results of this study support the practice of serial CMR examinations to identify progressive disease at a time interval of up to 3 year

Edge effects and social behavior in three platyrrhines

Studies the social behavior of three platyrrhine monkey species across 100m edge and interior forest zones in a fragmented tropical rainforest in Costa Rica. </p