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We performed a systematic review of the literature on the assessment of subpulmonary and systemic right ventricular (RV) functional reserve during pharmacological and exercise stress in congenital heart patients and patients with pulmonary arterial hypertension (PAH). Literature search was conducted using PubMed, EMBASE, and MEDLINE from their inception up to August 2020. Of 913 records identified, 56 studies with a total of 1730 patients were included. Of the 56 studies, 23 assessed subpulmonary RV functional reserve in repaired tetralogy of Fallot patients, 19 assessed systemic RV reserve in patients with transposition of the great arteries (TGA) after atrial switch and those with congenitally corrected TGA, and 14 assessed subpulmonary RV research in patients with PAH. Pharmacological and exercise stress was used, respectively, in 22 and 34 studies. The main findings were (1) impairment of RV systolic and diastolic functional reserve, (2) associations between impaired functional reserve and worse baseline functional parameters, and (3) prognostic implications of RV systolic functional reserve on clinical outcomes in patients with volume and/or pressure-loaded subpulmonary and systemic right ventricles. Further studies are required to establish the incremental value of incorporating stress studies of RV systolic and diastolic function in the clinical management algorithm of congenital heart patients and patients with PAH., (© 2022 Wiley Periodicals LLC.)

We assessed right ventricular (RV)-pulmonary arterial (PA) coupling in patients with repaired tetralogy of Fallot (TOF). Sixty patients (34 males) aged 18.6 ± 8.3 years at 14.8 ± 7.4 years after repair and 60 controls were studied. Two-dimensional, tissue Doppler and speckle tracking echocardiography and colour flow mapping were performed to assess RV end-systolic (ESA) and -diastolic areas, tricuspid valve Doppler and myocardial velocities, left ventricular (LV) and RV deformation and pulmonary (PR), tricuspid regurgitation (TR), respectively. The ratios of RV area change to ESA and peak tricuspid annular systolic (s) velocity to RV ESA indexed to body surface area reflected RV-PA coupling. Patients had greater RV areas and reduced tricuspid annular and myocardial velocities, LV and RV myocardial mechanics compared to controls (all p < 0.05). Both RV area change/ESA ratio and peak tricuspid annular s velocity/indexed RV ESA ratio were reduced in patients (all p < 0.001). Sixty-one and 100% of patients had, respectively, RV area change/ESA ratio and peak tricuspid annular s velocity/indexed RV ESA ratio < -2SD of controls. Indices of RV-PA coupling correlated positively with tricuspid myocardial velocities, LV and RV deformation and inversely with PR and TR (all p < 0.05). Multivariate analysis showed RV systolic strain rate, PR and TR as independent predictors of both RV-PA coupling indices, whilst age, gender and LV systolic strain were also predictors of peak tricuspid annular s velocity/indexed RV ESA ratio (all p < 0.05). In conclusion, RV-PA coupling is impaired and is associated with RV and LV mechanics and severity of PR and TR in patients with repaired TOF., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Aims: This study determined the associations between right atrial (RA) and right ventricular (RV) mechanics and liver stiffness in adults with repaired tetralogy of Fallot (TOF), pulmonary atresia with intact ventricular septum (PAVIS), and pulmonary stenosis (PS)., Methods and Results: Ninety subjects including 26 repaired TOF, 24 PAIVS, and 20 PS patients and 20 controls were studied. Hepatic shear wave velocity and tissue elasticity (E), measures of liver stiffness, were assessed by two-dimensional shear wave elastography, while RA and RV mechanics were assessed by speckle tracking echocardiography. Deformation analyses revealed worse RV systolic strain and strain rate, and RA peak positive and total strain, and strain rates at ventricular systole and at early diastole in all of the patient groups compared with controls (all P < 0.05). Compared with controls, all of the patient groups had significantly greater shear wave velocity and hepatic E-value (all P < 0.05). Shear wave velocity and hepatic E-value correlated negatively with RV systolic strain rate, and RA positive strain, total strain, and strain rate at ventricular systole and at early diastole (all P < 0.05). Multivariate analyses revealed RA strain rate at early diastole (P = 0.015, P < 0.001), maximum RA size (P < 0.001, P < 0.001), and severity of pulmonary regurgitation (P = 0.05, Pp = 0.014) as significant correlates of shear wave velocity and hepatic E-value., Conclusion: In adults with repaired TOF, PAIVS, and PS, RA dysfunction and pulmonary regurgitation are associated with liver stiffness., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)

Background: The QRS-T angle has been associated with adverse cardiovascular events and sudden cardiac deaths. We determined frontal QRS-T angle in patients with complete transposition of the great arteries (TGA) after atrial switch operation and repaired tetralogy of Fallot (TOF) and explored its relationships with ventricular mechanics., Methods: Thirty TGA patients aged 32.3 ± 4.4 years after atrial switch operation and 47 repaired TOF patients aged 28.7 ± 6.0 years were studied. The frontal planar QRS-T angle and QRS duration were measured from 12-lead electrocardiograms. Right (RV) and left ventricular (LV) strain parameters were determined using speckle tracking echocardiography., Results: Compared with TOF patients, TGA patients after atrial switch operation had significantly greater frontal QRS-T angle (136.3° ± 43.5° vs 74.5° ± 59.6°, p < 0.001), greater prevalence of QRS-T angle ≥ 100° (83.3% vs 29.8%, p < 0.001), and showed progressive increase in QRS-T angle over a duration of 3.3 ± 1.0 years (p = 0.035). The QRS-T angle correlated positively with QRS duration in both the TGA (r = 0.61, p < 0.001) and TOF (r = 0.30, p < 0.043) groups. Among TGA patients, QRS-T angle was found to correlate negatively with systemic RV global longitudinal strain (r = - 0.49, p = 0.007), early diastolic strain rate (r = - 0.41, p = 0.026), and fractional area change (r = - 0.38, p = 0.045), but not subpulmonary LV strain indices. By contrast, among repaired TOF patients, there were no significant correlations between QRS-T angle and systemic and subpulmonary ventricular strain indices (all p > 0.05)., Conclusion: Increased frontal QRS-T angle is prevalent in TGA patients after atrial switch operation and is related to worse systemic RV mechanics.

In patients with repaired tetralogy of Fallot (TOF), dysfunction of the right and left ventricles remains an important issue. Adverse right ventricular (RV) remodeling has been related to RV dilation secondary to pulmonary regurgitation, electromechanical dyssynchrony, and myocardial fibrosis. Left ventricular (LV) dysfunction is attributed among other factors to altered ventricular-ventricular interaction. Advancements in echocardiography and cardiac magnetic resonance imaging have enabled direct interrogation of myocardial deformation of both ventricles in terms of myocardial strain and strain rate. Emerging evidence suggests that myocardial deformation imaging may provide incremental information for clinical use. In children and adults with repaired TOF, there is a growing body of literature on the use of myocardial deformation imaging in the assessment of ventricular mechanics and its clinical and prognostic values. The present review aims to provide an overview of impairment in RV and LV mechanics, associations between RV and LV deformation, changes in ventricular deformation after pulmonary valve replacement, and associations between measures of RV and LV deformation and outcomes and to highlight the clinical translational potential of myocardial deformation imaging in patients with repaired TOF., (Copyright © 2020 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

This study aimed to assess left (LA) and right atrial (RA) function in patients with beta-thalassaemia major. Thirty-eight patients (19 males) aged 34.5 ± 10.7 years and 43 (18 males) controls aged 30.3 ± 12.6 years (p = 0.12) were studied. The maximum RA and LA areas were measured using two-dimensional planimetry, while atrial and ventricular strain and strain rates were quantified using speckle-tracking echocardiography. Compared with controls, patients had significantly reduced LA and RA peak positive strain and total strain, and LA strain rate during ventricular systole and at atrial contraction (all p < 0.05). The LA and RA strain parameters were significantly associated (all p < 0.05). The maximum LA (10.2 ± 1.6 cm 2 /m 2 vs. 8.6 ± 1.3 cm 2 /m 2 , p < 0.001) and RA (9.2 ± 1.2 cm 2 /m 2 vs. 7.5 ± 1.3 cm 2 /m 2 , p < 0.001) areas were significantly greater in patients than controls. The LV and RV strain and early strain rates were similar between patients and controls (all p > 0.05). Four patients with significant myocardial iron overload had larger LA area (p < 0.001) than those without. Functional and structural remodeling of both the right and left atria occurs in patients with beta-thalassaemia major, even in the absence of ventricular diastolic dysfunction.

Background: This study tested the hypothesis that right atrial (RA) mechanics are altered in patients after repair of pulmonary atresia with intact ventricular septum (PAIVS) and pulmonary stenosis (PS) and to explore their relationships with right ventricular (RV) diastolic function and P-wave indices., Methods: Thirty-six adult patients, 16 with PAIVS and 20 with PS patients, and 20 age-matched healthy subjects were studied. Right atrial mechanics were assessed using speckle tracking echocardiography (STE) with quantification of positive, negative, and total strain, and strain rates at ventricular systole (aSRs ), early diastole (aSRed ), and atrial contraction (aSRac ). Right ventricular diastolic function was assessed by Doppler interrogation and STE. Maximum P-wave duration and P-wave dispersion were measured., Results: Compared with controls, PAIVS and PS patients had significantly lower RA positive and total strain, aSRs and aSRed (all P < 0.05). Parameters of RV diastolic function including transtricuspid early (E) and late (A) diastolic inflow velocities, E/A ratio, early diastolic tricuspid annular velocity (e), early to late diastolic annular velocity ratio, E/e ratio, and RV early diastolic strain rate were found to correlate significantly with RA positive and total strain and aSRed (all P < 0.05). Maximum P-wave duration and P-wave dispersion increased progressively across control, PS, and PAIVS groups (P for trend <0.001). Both P-wave indices correlated negatively with RA positive strain, total strain, and aSRed (all P < 0.05)., Conclusion: Impairment of RA mechanics occurs in patients long term after repair of PAIVS and PS and is associated with RV diastolic dysfunction, longer P-wave duration, and greater P-wave dispersion., (© 2015, Wiley Periodicals, Inc.)

Background: Disruption of pulmonary vasculogenesis occurs in bronchopulmonary dysplasia (BPD). Increased impedance to pulmonary flow secondary to abnormal vascular development may affect ventricular mechanics., Objectives: We aimed to test the hypothesis that cardiac mechanics are altered in prematurely born children with BPD., Methods: A cohort of 47 children was studied: 22 aged 48.9 ± 6.4 months born preterm with BPD (group I), 13 aged 46.3 ± 8.1 months born preterm without BPD (group II), and 12 healthy children aged 53.4 ± 12.2 months born at term (group III). Left (LV) and right ventricular (RV) strain and strain rate were assessed by speckle-tracking echocardiography., Results: The global RV systolic strain rate (p = 0.022) was significantly lower, while RV systolic strain (p = 0.05) and early diastolic strain rate (p = 0.05) and LV longitudinal systolic strain rate (p = 0.06) also tended to be lower in group I than group III. Group I also tended to have lower RV systolic strain (p = 0.09) and early diastolic strain rate (p = 0.049) and LV longitudinal systolic strain rate (p = 0.08) than group II. An increasing trend from group I to III was observed for RV lateral wall and septal systolic strain and strain rate (all p < 0.05). The LV but not RV size was significantly smaller in group I compared with group III (p < 0.05). Multiple regression identified duration of invasive ventilation (β = -0.66, p = 0.032) as an independent determinant of RV systolic strain after adjustment for perinatal risk factors., Conclusion: Impairment of RV myocardial deformation occurs in children with BPD, the magnitude of which is associated negatively with the duration of invasive ventilation., (© 2015 S. Karger AG, Basel.)

Background: Alteration of right ventricular (RV) function has been found in patients with pressure-loaded left ventricles due to systemic hypertension and aortic stenosis. We tested the hypothesis that RV mechanics are altered in adolescents and adults with repaired coarctation of the aorta (CoA)., Methods: Twenty-eight (15 male) patients, aged 23.7 ± 6.5 years, and 28 (14 male) controls were studied. M-mode, tissue Doppler imaging, and speckle tracking echocardiography were performed to assess, respectively, LV mass and RV wall thickness, RV myocardial tissue velocities, and deformation. Systolic (P = 0.14) and diastolic (P = 0.32) blood pressures were similar between patients and controls., Results: Compared with controls, patients had significantly greater LV mass (P = 0.016) and RV anterior wall thickness (P = 0.012). Tricuspid annular systolic (P < 0.001) and early diastolic (P < 0.001) velocities, isovolumic acceleration (P = 0.004), global RV systolic longitudinal strain (P = 0.03), systolic strain rate (P = 0.012), and early (P = 0.021) and late (P = 0.012) diastolic strain rates were significantly lower in patients than controls. Patients with an associated ventricular septal defect (n = 6) requiring closure compared to those without had even lower tricuspid annular systolic (P = 0.01) and early diastolic (P = 0.041) velocities. Left ventricular mass correlated negatively with RV systolic strain rate (r = -0.27, P = 0.045) and tricuspid annular early diastolic velocity (r = -0.40, P = 0.002), while RV anterior wall thickness correlated negatively with tricuspid annular systolic (r = -0.42, P = 0.002) and late diastolic (r = -0.40, P = 0.003) velocities, and positively with e/a ratio (r = 0.31, P = 0.024)., Conclusion: RV systolic and diastolic mechanics are impaired in patients late after repair of CoA and related to increased LV mass and RV thickness., (© 2014, Wiley Periodicals, Inc.)

Background: Cardiomyocyte apoptosis has been implicated in ventricular remodeling and initiation of cardiac failure. We sought to determine the severity of right ventricular (RV) cardiomyocyte apoptosis in cyanotic and acyanotic children with RV pressure overload., Methods: Fourteen patients, seven with tetralogy of Fallot (group I) and seven with pulmonary stenosis and ventricular septal defect (group II), undergoing open-heart surgery were studied. Right ventricular biopsies were examined for cardiomyocyte apoptosis by terminal deoxynucleotide transferase-mediated dUTP nick-end labeling. The magnitude of cardiomyocyte apoptosis was related to preoperative oxygen saturation and postoperative inotrope use and hospital stay., Results: Compared with group I patients, group II patients were significantly older at operation (p = 0.002) and had a larger body size (p < 0.01) and higher preoperative oxygen saturation (p = 0.01). The prevalence of cardiomyocyte apoptosis in both group I and II patients as a whole was 0.24 ± 0.29% (range, 0% to 1.10%). The prevalence was similar between group I (median 0.30%, range 0% to 1.10%) and group II (median 0.20, range 0% to 0.40%, p = 0.65). The prevalence of cardiomyocyte apoptosis correlated positively with preoperative oxygen saturation on room air (r = -0.69, p < 0.005) and postoperative inotrope score (r = 0.67, p = 0.001). A higher postoperative inotrope score (r = 0.68, p = 0.001) was associated with a significant longer duration of postoperative stay in the hospital., Conclusions: The prevalence of cardiomyocyte apoptosis in the pressure-overloaded right ventricle is related to the severity of hypoxia and may have an impact on postoperative course in terms of early postoperative use of inotropes and duration of hospital stay., (© 2014 Wiley Periodicals, Inc.)

Background: The clinical relevance of evaluating right ventricular (RV) myocardial deformation in congenital heart disease is increasingly recognized. The aim of this study was to explore, using three-dimensional (3D) speckle-tracking echocardiography, RV mechanics in terms of 3D global area strain and mechanical dyssynchrony in adults with repaired tetralogy of Fallot., Methods: Twenty patients (12 men) aged 24.7 ± 8.6 years and 22 age-matched controls (11 men) were studied. Global RV peak area strain and area strain-derived systolic dyssynchrony index (SDI) were determined using 3D speckle-tracking echocardiography. RV end-diastolic volume and end-systolic volume, ejection fraction (EF), and pulmonary regurgitation fraction were measured in patients using cardiac magnetic resonance., Results: Coefficients of variation for intraobserver and interobserver measurements of RV global area strain were 6.1% and 7.9%, respectively, and those for SDI were 7.6% and 10.1%, respectively. Compared with controls, patients had significantly lower global area strain (P = .005) and greater SDI (P = .008). The prevalence of RV mechanical dyssynchrony (SDI > control mean + 2 SDs) in patients was 30%. In patients, global area strain correlated inversely with SDI (r = -0.42, P = .04), RV end-diastolic volume (r = -0.48, P = .032), and RV end-systolic volume (r = -0.48, P = .031) and positively with EF (r = -0.51, P = .02), while RV SDI correlated positively with RV end-systolic volume (r = 0.55, P = .012), pulmonary regurgitation fraction (r = 0.54, P = .031), and QRS duration (r = 0.51, P = .022) and negatively with RV EF (r = -0.62, P = .004). Multivariate analysis showed that RV EF (β = 0.22, P = .048) was a significant correlate of global area strain in patients., Conclusions: In adults after tetralogy of Fallot repair, 3D RV deformation is impaired in association with RV dyssynchrony, volume overloading, and reduced EF., (Copyright © 2014 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Background: The hypertrophic myocardium, myocardial fiber disarray, and endocardial fibroelastosis in pulmonary atresia and intact ventricular septum (PAIVS) may provide anatomic substrates for restrictive filling of the right ventricle., Hypothesis: Restrictive right ventricle (RV) physiology is related to RV fibrosis and exercise capacity in patients after biventricular repair of PAIVS., Methods: A total of 27 patients, age 16.5 +/- 5.6 years, were recruited after biventricular repair of PAIVS. Restrictive RV physiology was defined by the presence of antegrade diastolic pulmonary flow and RV fibrosis assessed by late gadolinium enhancement (LGE) cardiac magnetic resonance. Their RV function was compared with that of 27 healthy controls and related to RV LGE score and exercise capacity., Results: Compared with controls, PAIVS patients had lower tricuspid annular systolic and early diastolic velocities, RV global longitudinal systolic strain, systolic strain rate, and early and late diastolic strain rates (all P < 0.05). A total of 22 (81%, 95% confidence interval: 62%-94%) PAIVS patients demonstrated restrictive RV physiology. Compared to those without restrictive RV physiology (n = 5), these 22 patients had lower RV global systolic strain, lower RV systolic and early diastolic strain rates, higher RV LGE score, and a greater percent of predicted maximum oxygen consumption (all P < 0.05)., Conclusion: Restrictive RV physiology reflects RV diastolic dysfunction and is associated with more severe RV fibrosis but better exercise capacity in patients after biventricular repair of PAIVS., (Copyright 2010 Wiley Periodicals, Inc.)

Background: Assessment of systemic right ventricular (RV) function is inherently difficult. In adults, plasma brain natriuretic peptide (BNP) level has been shown to reflect systemic ventricular dysfunction. We sought to test the hypothesis that plasma BNP is a biomarker of systemic RV function in patients after atrial switch operation., Methods: We determined the RV function in 44 patients, 35 after Senning and 9 after Mustard operation, aged 19.7+/-4.0 years, by tissue Doppler echocardiography and determination of myocardial performance index (MPI). The results were compared to the left ventricular function of 14 age-matched controls. Their plasma BNP levels were correlated with indices of systemic ventricular function., Results: Compared with controls, the patients had greater MPI (p<0.001), lower systemic ventricular free wall-annular early diastolic (p<0.001), late diastolic (p<0.001), and systolic velocities (p=0.001), lower septal-annular early diastolic (p<0.001), late diastolic (p<0.001), and systolic velocities (p<0.001), and higher BNP levels (p=0.03). Plasma BNP levels correlated positively with MPI (r=0.43, p=0.001) and negatively with the free wall- and septal-annular myocardial velocities (r=-0.32 to -0.47, p<0.05). The area under the receiver operating characteristic curve for BNP to detect ventricular dysfunction (MPI >0.45) in patients was 0.67 (p=0.04). A BNP level of 36 pg/ml had a sensitivity of 55%, specificity of 86%, positive predictive value 80%, negative predictive value of 64%, and an accuracy of 70% for detecting systemic ventricular dysfunction., Conclusions: Plasma BNP has modest accuracy in the detection of systemic RV dysfunction in patients after atrial switch operation.

Recent data suggest potential benefits of cardiac resynchronization therapy in the management of right ventricular (RV) dysfunction in congenital heart disease. The aim of this study was to determine the nature, prevalence, and functional implications of mechanical RV dyssynchrony in patients after Senning or Mustard procedures for transposition of the great arteries. Twenty-eight patients (mean age 21.1 +/- 3.5 years) at 19.9 +/- 3.2 years after atrial switch operations and 29 healthy controls were studied. The times from the onset of QRS to peak systolic strain (T epsilon) at the base of and the mid RV free wall, the ventricular septum (VS), and the left ventricular (LV) free wall were determined using tissue Doppler echocardiography. Intraventricular mechanical delay was defined as Delta T epsilon(RV-VS) and interventricular mechanical delay as Delta T epsilon(RV-LV). In patients, the magnitude of RV intra- and interventricular mechanical delay was correlated with cardiac magnetic resonance-derived RV volumes and ejection fractions (n = 26) and treadmill exercise testing parameters (n = 20). Compared with controls, patients had significantly longer Delta T epsilon(RV-VS) (48.1 +/- 50.9 vs 17.0 +/- 16.1 ms, p <0.001) and Delta T epsilon(RV-LV) (63.1 +/- 49.5 vs 19.0 +/- 12.9, p <0.001). Nine patients (32%) exhibited RV dyssynchrony (Delta T epsilon(RV-VS) >49 ms, control mean +/- 2SD), and 16 patients (57%) showed interventricular dyssynchrony (Delta T epsilon(RV-LV) >45 ms). In patients, RV intra- and interventricular mechanical delay was correlated negatively with the RV ejection fraction (both r = -0.42, p = 0.03) and percentage predicted maximum oxygen consumption (r = -0.50, p = 0.03, and r = -0.52, p = 0.02, respectively) and positively with minute ventilation/carbon dioxide production slope (r = 0.49, p = 0.03, and r = 0.56, p = 0.01, respectively). In conclusion, RV dyssynchrony is common in young adults after atrial switch operations and is associated with RV systolic dysfunction and impaired exercise performance.

Background: Right ventricular (RV) volume overload secondary to pulmonary regurgitation contributes to long-term morbidities in patients after tetralogy of Fallot (TOF) repair. We tested the hypothesis that plasma brain natriuretic peptide (BNP) levels relate to RV volume overload, pulmonary regurgitation, and exercise capacity in adolescents after TOF repair., Methods: We assessed the RV function echocardiographically and plasma BNP levels in 32 postoperative TOF patients aged 14.7+/-3.1 years and 20 age-matched controls. Eighteen patients further underwent cardiovascular magnetic resonance imaging and 26 had exercise testing., Results: Compared with controls, patients had significantly higher BNP levels (p=0.027), greater indexed RV end-diastolic dimension (p<0.001), increased RV myocardial performance index (p=0.005), and reduced tricuspid annular systolic velocity (p=0.008). Multivariate analysis identified indexed RV end-diastolic dimension as the only significant determinant of plasma BNP levels (beta=0.69, p<0.001). Plasma BNP levels correlated positively with indexed RV end-diastolic volume (r=0.6, p=0.009) and pulmonary regurgitant fraction (r=0.54, p=0.026), and negatively with exercise duration (r=-0.45, p=0.021), peak oxygen consumption (r=-0.43, p=0.03), and minute ventilation at maximal exercise (r=-0.52, p=0.006). Multivariate analysis demonstrated BNP levels (beta=-0.43, p=0.034) and body mass index (beta=-0.40, p=0.036) to be independent predictors of peak oxygen consumption. No relations were found between BNP levels and RV myocardial performance index, tricuspid annular velocities and RV ejection fraction., Conclusions: In adolescent patients after TOF repair, plasma BNP levels relate to RV volume overload, pulmonary regurgitation and exercise capacity.

The long-term ventricular function of children after surgical and transcatheter closure of secundum atrial septal defects was assessed by Doppler tissue imaging and compared with that of age-matched controls. Long-term right ventricular function was impaired in patients after surgical repair, as reflected by decreased systolic and diastolic tricuspid annular velocities and an increased Tei index. In contrast, right ventricular function was preserved after transcatheter device closure.