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Background The determinants and prognostic value of high‐sensitivity cardiac troponin T (hs‐cTnT) among patients with a systemic right ventricle are largely unknown. Methods and Results Ninety‐eight patients from the randomized controlled SERVE (Effect of Phosphodiesterase‐5 Inhibition With Tadalafil on Systemic Right Ventricular Size and Function) trial were included. The correlation between baseline hs‐cTnT concentrations and biventricular volumes and function quantified by cardiac magnetic resonance or cardiac multirow detector computed tomography was assessed by adjusted linear regression models. The prognostic value of hs‐cTnT was assessed by adjusted Cox proportional hazards models, survival analysis, and concordance statistics. The primary outcome was time to the composite of clinically relevant arrhythmia, hospitalization for heart failure, or all‐cause death. Median age was 39 (interquartile range, 32–48) years, and 32% were women. Median hs‐cTnT concentration was 7 (interquartile range, 4–11) ng/L. Coefficients of determination for the relationship between hs‐cTnT concentrations and right ventricular end‐systolic volume index and right ventricular ejection fraction (RVEF) were +0.368 (P=0.046) and −0.381 (P=0.018), respectively. The sex‐ and age‐adjusted hazard ratio for the primary outcome of hs‐cTnT at 2 and 4 times the reference level (5 ng/L) were 2.89 (95% CI, 1.14–7.29) and 4.42 (95% CI, 1.21–16.15), respectively. The prognostic performance quantified by the concordance statistics for age‐ and sex‐adjusted models based on hs‐cTnT, right ventricular ejection fraction, and peak oxygen uptake predicted were comparable: 0.71% (95% CI, 0.61–0.82), 0.72% (95% CI, 0.59–0.84), and 0.71% (95% CI, 0.59–0.83), respectively. Conclusions Hs‐cTnT concentration was significantly correlated with right ventricular ejection fraction and right ventricular end‐systolic volume index in patients with a systemic right ventricle. The prognostic accuracy of hs‐cTnT was comparable to that of right ventricular ejection fraction and peak oxygen uptake predicted. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03049540.

Trisomy 21 (T21) is associated in 40–45% of cases with heart defects, most commonly shunt lesions. These defects, if not repaired, can lead to irreversible shunt-induced pulmonary hypertension (i.e. Eisenmenger syndrome [ES]). In ES patients, intracardiac repair is no longer possible, but selective pulmonary vasodilators may increase exercise capacity and improve prognosis. This study aimed to estimate the prevalence of cardiac defects and ES in adult T21 patients and to assess the impact of T21 on treatment modalities and outcome in ES patients. A questionnaire was sent to 6906 Swiss physicians inviting them to indicate the number of adults with T21 under their care (survey report). We also analyzed all adults with ES (with and without T21) included in the Swiss Adult Congenital HEart disease Registry (SACHER) and studied the impact of T21 on the use of selective pulmonary vasodilators and survival. In the survey, 348 physicians cared for 695 adult T21 patients. Overall, 24% of T21 survey patients were known to have a cardiac defect, one in four with a defect had developed ES and 13% of those with ES were on specific pulmonary vasodilators. In SACHER, ES was present in 2% of adults with congenital heart disease and selective pulmonary vasodilators were used in 68% of ES patients with T21. In SACHER, survival during follow-up was worse with higher nt-proBNP levels (hazard ratio [HR] = 1.15 per 1000 units, 95% confidence interval [CI] = 1.02–1.29) and lower left ventricular ejection fraction (HR = 1.07 per percent decrease, 95% CI = 1.01–1.13). Age at inclusion and T21 did not affect survival. The prevalence of cardiac defects in adults with T21 in Switzerland is half the prevalence in children. T21 is over-represented among adults with ES. Raised awareness of the therapeutic options for T21 patients with ES is warranted.

BackgroundThe prognostic value of the change in heart rate from the supine to upright position (∆HR) in patients with chronic heart failure (HF) is unknown. Methods and Results∆HR was measured in patients enrolled in the Trial of Intensified Medical Therapy in Elderly Patients with Congestive Heart Failure (TIME‐CHF) who were in sinus rhythm and had no pacemaker throughout the trial (n=321). The impact of ∆HR on 18‐month outcome (HF hospitalization‐free survival) was assessed. In addition, the prognostic effect of changes in ∆HR between baseline and month 6 on outcomes in the following 12 months was determined. A lower ∆HR was associated with a higher risk of death or HF hospitalization (hazard ratio 1.79 [95% confidence interval {95% CI} 1.19‐2.75] if ∆HR ≤3 beats/min [bpm], P=0.004). In the multivariate analysis, lower ∆HR remained an independent predictor of death or HF hospitalization (hazard ratio 1.75 [95% CI, 1.18‐2.61] if ∆HR ≤3 bpm, P=0.004) along with ischemic HF etiology, lower estimated glomerular filtration rate, presence and extent of rales, and no baseline β‐blocker use. In patients without event during the first 6 months, the change in ∆HR from baseline to month 6 predicted death or HF hospitalization during the following 12 months (hazard ratio=2.13 [95% CI 1.12–5.00] if rise in ∆HR