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Impact of Right Ventricular Pressure Load After Repair of Tetralogy of Fallot.

ABSTRACT: Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow-up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow-up of 10.1 (0.1-12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00-1.06; P=0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47-9.27; P=0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15-30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

SUBMITTER: Latus H

PROVIDER: S-EPMC9075442 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Background The relative impact of right ventricular ( RV ) electromechanical dyssynchrony versus pulmonary regurgitation ( PR ) on exercise capacity and RV function after tetralogy of Fallot repair is unknown. We aimed to delineate the relative effects of these factors on RV function and exercise capacity. Methods and Results We retrospectively analyzed 81 children with tetralogy of Fallot repair using multivariable regression. Predictor parameters were electrocardiographic QRS duration reflecting electromechanical dyssynchrony and PR severity by cardiac magnetic resonance. The outcome parameters were exercise capacity (percentage predicted peak oxygen consumption) and cardiac magnetic resonance ejection fraction (RV ejection fraction). To understand the relative effects of RV dyssynchrony versus PR on exercise capacity and RV function, virtual patient simulations were performed using a closed-loop cardiovascular system model (CircAdapt), covering a wide spectrum of disease severity. Eighty-one patients with tetralogy of Fallot repair (median [interquartile range { IQR} ] age, 14.48 [11.55-15.91] years) were analyzed. All had prolonged QRS duration (median [IQR], 144 [123-152] ms), at least moderate PR (median [IQR], 40% [29%-48%]), reduced exercise capacity (median [IQR], 79% [68%-92%] predicted peak oxygen consumption), and reduced RV ejection fraction (median [IQR], 48% [44%-52%]). Longer QRS duration, more than PR , was associated with lower oxygen consumption and lower RV ejection fraction. In a multivariable regression analysis, oxygen consumption decreased with both increasing QRS duration and PR severity. CircAdapt modeling showed that RV dyssynchrony exerts a stronger limiting effect on exercise capacity and on RV ejection fraction than does PR , regardless of contractile function. Conclusions In both patient data and computer simulations, RV dyssynchrony, more than PR , appears to be associated with reduced exercise capacity and RV systolic dysfunction in patients after TOF repair.

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:The aim of the study was to determine whether segmental interactions, as expressed by postsystolic shortening (PSS), affects RV mechanics and are connected with impaired systolic and diastolic function in rTOF children.Patients and methods55 rTOF adolescent (study group), and 34 healthy volunteers (control group) were examined using classical Doppler flow (Doppler), Tissue Doppler Imaging (TDI) and Speckle Tracking Echocardiography (STE). PSS was found to occur when time to peak (TTP) was longer than pulmonary valve closure time (PVCT). TTP and strain were derived from RV lateral segments-basal (BL), medial (ML) and apical (AL) in STE. PVCT was measured from the beginning of QRS complex in the ECG to the termination of Doppler flow at the pulmonary valve. TDI was obtained at the lateral tricuspid annulus site and the systolic (S'), early (E') and late diastolic (A') peak velocities were measured along with isovolumic contraction (IVCT), and relaxation (IVRT) time. PW was used to measure early tricuspid inflow velocity (E) for calculating the E/E' ratio. The TDI data in patients with PSS presence (TTP>PVCT) and those in whom it did not occur (TTP≤PVCT) were compared.ResultsPSS in BL, ML and AL were observed respectively in: 27(51,9%), 9 (18%), and 8 (16,7%) patients. Mean values of TTP in BL, ML, and AL were respectively: 420.6±55.5ms, 389.8±50.0ms and 366.7±59.0ms. PVCT mean value was 396.6±33.5ms. In the study group, the mean E' in TTP>PVCT was significantly lower (4.8±1.8 cm/s) compared to mean E' in TTP≤PVCT (8.4±2.6 cm/s), p<0.01. The average E/E' was significantly higher in TTP>PCVT than in TTP≤PVCT, respectively 21.6±7.3 vs 12.2±5.1, p<0.05. IVRT was significantly prolonged in TTP>PVCT compared to IVRT in TTP≤PVCT, respectively 95.9±38.7 vs 77.0±35.1, p<0.05. Furthermore, in TTP>PVCT, significantly higher strain in BL (-28.8±8.7%) was observed when compared to that parameter in TTP≤PVCT (-35.3±13.1%), p <0.05.ConclusionsTissue Doppler Echocardiography and Speckle Tracking Echocardiography are useful techniques for detecting regional systolic and diastolic dysfunction in children after Tetralogy of Fallot surgical repair. Postsystolic shortening in the basal lateral segment is commonly seen in children after the Tetralogy of Fallot surgical repair, and is associated with altered right ventricular systolic and diastolic function.

Project description:ObjectivesThe study objectives were to reconfirm the superiority of the pulmonary valve-sparing procedure versus the transannular patch procedure for repair of tetralogy of Fallot and to evaluate the influence of a right ventriculotomy in the pulmonary valve-sparing procedure.MethodsBetween 1978 and 2003, 440 patients (aged <10 years) underwent tetralogy of Fallot repair. Of these patients, 242 (55.0%) underwent the transannular patch procedure, 106 (24.1%) underwent the pulmonary valve-sparing procedure without right ventriculotomy, and 92 (20.9%) underwent the pulmonary valve-sparing procedure with right ventriculotomy. End points focused on adverse events and included all-cause mortality, reoperation, catheter intervention, and symptomatic arrhythmias. To compare the outcomes of pulmonary valve sparing with and without right ventriculotomy, inverse probability weighting was applied to adjust for potential confounding factors.ResultsThe median follow-up period was 20.3 years (interquartile range, 10.7-27.6). In all cohorts, the pulmonary valve-sparing procedure was the independent factor that reduced adverse events after tetralogy of Fallot repair (hazard ratio, 0.47; 95% confidence interval, 0.23-0.94; P = .033). After weighting, there was no difference in overall survival or event-free survival in the pulmonary valve-sparing with and without right ventriculotomy group. However, the pulmonary valve-sparing with right ventriculotomy group exhibited a larger cardiothoracic ratio (beta: 6.01; 95% confidence interval, 2.36-9.66; P = .001), lower medication-free rate (odds ratio, 0.29; 95% confidence interval, 0.098-0.79; P = .019), and higher New York Heart Association functional classification (odds ratio, 2.99; 95% confidence interval, 1.36-6.80; P = .007) at the latest follow-up.ConclusionsRight ventriculotomy for tetralogy of Fallot repair with pulmonary valve-sparing did not increase major adverse events. However, negative impacts on current status cannot be ignored.

Project description:Background: Current observational studies may not have large samples to investigate the relationship between pulmonary valve (PV) morphology differences and outcomes after complete repair for tetralogy of Fallot (TOF) by right ventricular outflow tract (RVOT) incision. This study aimed to assess the impact of PV morphology differences on outcomes after complete repair for TOF. Methods: This is a retrospective cohort study. Consecutive patients who underwent TOF repair with RVOT incision at Fuwai Hospital from January 2012 to December 2017 were included and compared according to PV morphology differences (unicuspid or bicuspid was abnormal morphology, while the tricuspid valve was normal morphology). The primary outcome was defined as a composite of death, or reintervention, or significant annular peak gradient (APG), or significant pulmonary regurgitation (PR), whichever occurred first. Multivariable Cox model analysis was used to assess the relationships between PV morphology differences and outcomes. Subgroup analysis and Propensity-score analysis were performed as sensitivity analyses to assess the robustness of our results. Results: The cohort included a total of 1,861 patients with primary diagnosis of TOF, with 1,688 undergoing CR-TOF with RVOT incision. The median age was 318 days [interquartile range (IQR): 223-534 days], a median weight of 8.9 kg (IQR: 7.6-10.5 kg) and 60.0% (1,011) were male. Complete follow-up data were available for 1,673 CR-TOF patients with a median follow-up duration of 49 months. Adjusted risks for the primary outcome and significant APG were lower for patients with normal PV morphology at follow up [adjusted hazard ratio (HR): 0.68; 95% CI: 0.46-0.98; adjusted HR: 0.22; 95% CI: 0.07-0.71, respectively]. The trend for the primary outcome during follow-up remained unchanged, even in subgroups and propensity score matching analyses. Conclusions: In this analysis of data from a large TOF cohort, patients with normal tricuspid PVs were associated with a decreased risk of the primary outcome and a lower risk of significant APG, as compared with patients with abnormal unicuspid or bicuspid PVs.

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.

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Impact of Right Ventricular Pressure Load After Repair of Tetralogy of Fallot.

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