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Pathophysiologic and Prognostic Implications of Right Atrial Hypertension in Adults With Tetralogy of Fallot.

ABSTRACT: Background Right atrial pressure (RAP), a composite metric of right ventricular diastolic function, volume status, and right heart compliance, is a predictor of mortality in patients with heart failure due to acquired heart disease. Because patients with tetralogy of Fallot (TOF) might have abnormal right atrial and ventricular mechanics caused by myocardial injury and remodeling, we hypothesized that RAP would be associated with disease severity and cardiovascular adverse events in this population. Methods and Results We performed a cohort study of adults with TOF who underwent right heart catheterization at the Mayo Clinic Rochester between 1990 and 2017. The objective was to determine the association between RAP and multiple domains of disease severity in TOF (percentage of predicted peak oxygen consumption, atrial or ventricular arrhythmia, and heart failure hospitalization), as well as cardiovascular adverse events, defined as sustained ventricular tachycardia, resuscitated or aborted sudden death, heart transplantation, or death. Among 225 patients (113 male; mean age: 39±14 years), mean RAP was 10.7±5.2 mm Hg and median was 10 mm Hg (interquartile range: 7-13 mm Hg). Increasing RAP was associated with atrial or ventricular arrhythmias (odds ratio: 5.01; 95% CI, 1.22-23.49; P<0.001), heart failure hospitalization (odds ratio: 1.47; 95% CI, 1.10-2.39; P=0.033) per 5 mm Hg, and worsening exercise capacity (peak oxygen consumption; R²=0.74, r=-0.86, P<0.001). RAP was a predictor of cardiovascular adverse events (hazard ratio: 1.28; 95% CI, 1.10-1.47; P=0.028) per 5 mm Hg. Conclusions In symptomatic patients with TOF, increasing RAP correlates with multiple domains of disease severity (risk stratification) and predicts future cardiovascular events (prognostication). These data have potential clinical implications in the target population of symptomatic TOF patients.

SUBMITTER: Egbe AC

PROVIDER: S-EPMC6915294 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature

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Pathophysiologic and Prognostic Implications of Right Atrial Hypertension in Adults With Tetralogy of Fallot.

Egbe Alexander C AC Bonnichsen Crystal C Reddy Yogesh N V YNV Anderson Jason H JH Borlaug Barry A BA

Journal of the American Heart Association 20191108 22

Background Right atrial pressure (RAP), a composite metric of right ventricular diastolic function, volume status, and right heart compliance, is a predictor of mortality in patients with heart failure due to acquired heart disease. Because patients with tetralogy of Fallot (TOF) might have abnormal right atrial and ventricular mechanics caused by myocardial injury and remodeling, we hypothesized that RAP would be associated with disease severity and cardiovascular adverse events in this populat ...[more]

PMID: 31701796

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Project description:Background and purposeElectromechanical dyssynchrony, manifested by right bundle branch block and regional wall mechanical dysfunction, contributes to inefficient RV function in repaired Tetralogy of Fallot (ToF). This study aims to evaluate the synchronicity of multiple RV walls using two-dimensional multi-plane echocardiography (2D-MPE) in order to augment current understanding of the mechanisms behind RV dyssynchrony.MethodsSixty-nine adult ToF patients [aged 33 (23-45) years; 61% male] and twenty-five matched healthy controls underwent deformational analysis of the RV lateral, anterior, inferior and septal walls following 2D-MPE acquisitions. RV synchronicity was assessed by the intra-RV deformation delay between each basal RV wall and mid-septal segment in addition to mechanical dispersion calculated across four, six and eight segments (MD).ResultsAll RV wall-septum delays plus MD-4 and MD-6 indices were significantly greater in ToF patients compared to healthy controls (p < 0.001-0.03). In ToF patients, the lateral and anterior RV walls were last to reach peak deformation and anterior wall longitudinal strain was lower (p = 0.001). Post systolic shortening of at least one RV wall segment was identified in 19 (28%) ToF patients. Despite similar ECG characteristics, lateral and anterior wall-septum delays were significantly longer in patients with greater degrees of dyssynchrony (73 [37-108]ms vs. 37 [0-63]ms, p = 0.006; 91 [52-116]ms vs. 41 [1-69]ms, p = 0.013), although RV ejection fraction (RVEF) was not significantly lower. MD-4 and MD-8 indices displayed moderate negative associations with RVEF, strengthened by inclusion of lateral wall longitudinal strain (r = 0.64/0.65; p ≤0.01).ConclusionRV dyssynchrony in ToF is characterised by electromechanical delays between the lateral, anterior and septal walls, with anterior wall dysfunction likely associated with surgical repair of the RV outflow tract. Prospectively, 2D-MPE may have an emerging role evaluating RV mechanical response to electrical resynchronisation therapy.

Project description:BackgroundRelationships between right ventricular (RV) and left ventricular (LV) shape and function may be useful in determining optimal timing for pulmonary valve replacement in patients with repaired tetralogy of Fallot (rTOF). However, these are multivariate and difficult to quantify. We aimed to quantify variations in biventricular shape associated with pulmonary regurgitant volume (PRV) in rTOF using a biventricular atlas.MethodsIn this cross-sectional retrospective study, a biventricular shape model was customized to cardiovascular magnetic resonance (CMR) images from 88 rTOF patients (median age 16, inter-quartile range 11.8-24.3 years). Morphometric scores quantifying biventricular shape at end-diastole and end-systole were computed using principal component analysis. Multivariate linear regression was used to quantify biventricular shape associations with PRV, corrected for age, sex, height, and weight. Regional associations were confirmed by univariate correlations with distances and angles computed from the models, as well as global systolic strains computed from changes in arc length from end-diastole to end-systole.ResultsPRV was significantly associated with 5 biventricular morphometric scores, independent of covariates, and accounted for 12.3% of total shape variation (p < 0.05). Increasing PRV was associated with RV dilation and basal bulging, in conjunction with decreased LV septal-lateral dimension (LV flattening) and systolic septal motion towards the RV (all p < 0.05). Increased global RV radial, longitudinal, circumferential and LV radial systolic strains were significantly associated with increased PRV (all p < 0.05).ConclusionA biventricular atlas of rTOF patients quantified multivariate relationships between left-right ventricular morphometry and wall motion with pulmonary regurgitation. Regional RV dilation, LV reduction, LV septal-lateral flattening and increased RV strain were all associated with increased pulmonary regurgitant volume. Morphometric scores provide simple metrics linking mechanisms for structural and functional alteration with important clinical indices.

Dataset Information

Pathophysiologic and Prognostic Implications of Right Atrial Hypertension in Adults With Tetralogy of Fallot.

Publications

Pathophysiologic and Prognostic Implications of Right Atrial Hypertension in Adults With Tetralogy of Fallot.

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