Right Ventricular End-diastolic Volume Research Articles

Right ventricular adaptation to pressure-overload: Differences between chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (iPAH) are both associated with right ventricular (RV) failure and mortality. However, CTEPH patients are older, more often male and usually have more co-morbidities than iPAH patients, including a history of venous thromboembolism. Therefore, RV adaptation to pressure-overload in CTEPH may be different than in iPAH. We included all treatment-naive CTEPH and iPAH patients diagnosed in the Amsterdam UMC between 2000 and 2019 if cardiac magnetic resonance imaging (CMR) and a right heart catheterization were performed at time of diagnosis. Load-dependent RV volumes and mass were assessed with CMR. Load-independent RV contractility, afterload and diastolic stiffness in relation to afterload were obtained using single beat pressure-volume loop analysis. Differences in RV characteristics between CTEPH and iPAH were analyzed using multiple linear regression with interaction testing after correcting for confounders. We included 235 patients in this study and performed pressure-volume loop analysis in 136 patients. In addition to being older and more often male, CTEPH patients had a lower pulmonary vascular resistance than iPAH patients at the time of diagnosis. After correcting for these confounders, CTEPH patients had a somewhat higher RV end-diastolic volume index (87 ± 27 ml vs 82 ± 25 ml; p < .01), and a lower RV relative wall thickness (0.6 ± 0,1 g/ml vs 0.7 ± 0,2 g/ml; p < .01). The correlation coefficient of RV diastolic stiffness to afterload was higher in CTEPH compared to iPAH (p < .05; independent of age and gender). Despite differences in patient characteristics, disease etiology and physiology, RV functional parameters in CTEPH and iPAH are mostly similar. The right ventricle in CTEPH is marginally more dilated, stiffer and less hypertrophic than in iPAH.

Outcomes of adults with repaired tetralogy of Fallot from the national Scottish Cohort.

The adult population of repaired tetralogy of Fallot is increasing and at risk of pre-mature death and arrhythmia. This study evaluates risk factors for adverse outcome and the effect of pulmonary valve replacement within a national cohort. A retrospective cohort study of 341 adult repaired tetralogy of Fallot (16-72 years) managed through a single national service was undertaken incorporating over 1200 patient-years of follow-up. Demographics, cardiopulmonary exercise testing, cardiac magnetic resonance, reintervention (including pulmonary valve replacement), and clinical events were analysed. The influence of these parameters on a primary outcome (death or arrhythmia) was evaluated. Compared with an age-/gender-matched population, patients experienced a reduced survival, particularly males over 55 years (standardised mortality ratio : 6.12, 95% CI: 1.64-15.66, p = 0.004). Cox proportional hazards modelling identified increased indexed right ventricle (RV) end-diastolic volume (hazard ratio (HR): 2.86, 95% CI: 1.4-5.85, p = 0.004) and female gender (HR (male): 0.37, 95% CI: 0.14-0.98, p = 0.045) to be predictors significantly associated with the primary outcome. Pulmonary valve replacement undertaken at indexed RV end-diastolic volume = 145ml/m2 reduced RV volumes and QRS duration but did not improve cardiopulmonary exercise testing nor NYHA class. Pulmonary valve replacement during cohort period was associated with increased risk of primary outcome (HR: 2.82, 95% CI: 1.36-5.86, p = 0.005). Although the majority of adult tetralogy of Fallot were asymptomatic in NYHA 1, cardiopulmonary exercise testing revealed important deficits. Tetralogy of Fallot survival was reduced compared to the general population. Female gender and increasing RV end-diastolic volume predicted adverse events. Pulmonary valve replacement reduced RV volumes and QRS duration but did not improve primary outcome.

Differentiation of athlete's heart and hypertrophic cardiomyopathy by the fractal dimension of left ventricular trabeculae

BackgroundDifferentiation between exercise induced adaptive myocardial hypertrophy (athlete's heart) and hypertrophic cardiomyopathy (HCM) is currently based on echocardiographic and cardiac magnetic resonance (CMR) criteria, but these may be insufficient in patients with subtle phenotype expression. This study aimed to assess whether left ventricular (LV) fractal pattern could permit to differentiate athlete's heart from HCM. MethodsWe recruited retrospectively 61 elite marathon runners, 67 patients with HCM, and 33 healthy subjects. A CMR study was performed in all subjects and the LV trabeculae fractal dimension (FD) was measured in end-diastolic frames of each short-axis cine sequence. For group comparison, the ratio of maximal myocardial wall thickness (mMWT)/indexed LV end-diastolic volume (LVED) was determined. ResultsAs compared with athletes, patients with HCM had significantly (p < 0.001) greater FD in the LV basal (1.30 ± 0.07 vs. 1.23 ± 0.05) and apical (1.38 ± 0.06 vs. 1.30 ± 0.07) regions and in the whole heart (1.34 ± 0.05 vs. 1.27 ± 0.05). FD increased with age, left atrial area and indexed left ventricular mass (p < 0.05 for all) and correlated negatively with LV and RV end-diastolic volumes (p < 0.05 each). The addition of whole heart FD to the ratio of maximal myocardial wall thickness/indexed LVEDV lead to an improvement in the ability to discriminate HCM with a net reclassification index (NRI) of 71%. ConclusionsThe FD regional distribution of the LV trabeculae differentiates patients with athlete's heart from patients with HCM. The addition of whole heart FD to the mMWT/indexed LVEDV ratio improves the predictive capacity of the model to differentiate both entities.

Evolution of biventricular T1 values in patients with right-sided congenital heart disease

Abstract Funding Acknowledgements Type of funding sources: None. Background Conflicting reports exist on the prevalence and clinical impact of interstitial fibrosis in right ventricular (RV) congenital heart disease (CHD). This study evaluates the longitudinal evolution of native myocardial T1 relaxation time (T1) in RV CHD. Methods On a 1.5T scanner, an ECG-triggered modified Look-Locker inversion recovery sequence (scheme 3(3)3(3)5) was acquired on a short-axis basal slice covering the RV and left ventricle (LV) on two consecutive CMR exams. Global and segmental (LV = 6, RV = 4) RV and LV T1 values were calculated (Figure). Results Mean time between CMR exams for 36 included patients (age 34 ± 2y) was 22 ± 2 months. All LV segments and 81/88% of RV segments of first and second CMR could be analyzed, respectively. T1 increased mildly but not significantly (table). There was no relationship of T1 to pulmonary regurgitation fraction, pulmonary stenosis or RV enddiastolic volume (p &amp;gt; 0.05). Global RV T1 of the second CMR was related to RV ejection fraction (RVEF): r = 0.353, 3.0 ± 1.4, p = 0.038. T1 of the infero-septal LV segment of first and second CMR, global LV T1 of second CMR and increase of T1 of global LV, anterior, antero-lateral and –septal LV segments, were related to age at CMR: r = 0.333 - 0.463, p &amp;lt; 0.05, respectively. Conclusions Native T1 values increased mildly in patients with stable RV CHD, which was not statistically significant probably due to the short to median follow-up. Global RV T1 appears to be related to RVEF which could be sign of increasing interstitial fibrosis whereas the relationship of LV T1 to age might be a physiological finding. First CMR native T1 (ms) Second CMR native T1 (ms) p LV Global 1007 ± 37 1014 ± 39 0.413 LV Anterior 994 ± 53 999 ± 54 0.710 LV Antero-lateral 965 ± 63 981 ± 58 0.186 LV Infero-lateral 1000 ± 52 1004 ± 63 0.695 LV Inferior 1035 42 1037 ± 50 0.744 LV Infero-septal 1028 ± 35 1036 ± 43 0.282 LV Antero-septal 1016 ± 38 1024 ± 48 0.347 RV Global 1091 ± 90 1096 ± 85 0.410 RV Inferior 1112 ± 104 1115 ± 118 0.696 RV Infero-lateral 1061 ± 130 1077 ± 115 0.425 RV Antero-lateral 1046 ± 127 1080 ± 109 0.088 RV Anterior 1088 ± 156 1108 ± 154 0.410 Abstract Figure. Determination of biventricular T1 values

Serial right ventricular assessment in patients with hypoplastic left heart syndrome patients (HLHS): a cardiovascular magnetic resonance study

Abstract Funding Acknowledgements Type of funding sources: None. Background Patients with hypoplastic left heart syndrome (HLHS) are at risk for right ventricular (RV) dysfunction over the course of the three-stage surgical palliation with the final step being the completion of the total cavopulmonary connection (TCPC). However, less is known about RV function during follow-up after TCPC completion. We assessed RV function by analysing serial cardiovascular magnetic resonance (CMR) studies in a large cohort of HLHS patients. Methods CMR studies from 95 HLHS patients (67 males) were retrospectively analysed. Short axis cine images were used to measure RV end systolic and end diastolic volumes and ejection fraction (RVEF). Oblique cine images showing the atria and right ventricle in a similar manner like a standard "4-chamber view" were used to measure tricuspid annular plane systolic excursion (TAPSE) and long axis strain (LAS). Results From the 95 patients, all had at least two and 32 patients had three CMR scans. The first scan was performed at a mean age of 4.9 ± 2.8 years, the second scan at a mean age of 9.3 ± 4 years and the third at a mean age of 14.3 ± 3.7 years. The mean values of RV end diastolic and end systolic volume indexed to body surface area (REDVi, RVESVi) as well as RV ejection fraction (RVEF) at the three time points were: 1) REDVi 92.6 ± 21.9 ml/m2, RVESVi 43 ± 15.1 ml/m2, RVEF 54.2 ± 7.1%; 2) REDVi 93.9 ± 25.6 ml/m2, RVESVi 44.6 ± 18.3 ml/m2, RVEF 53.6 ± 7.8%; 3) REDVi 110.9 ± 41.9 ml/m2, RVESVi 58.1 ± 35 ml/m2, RVEF 50.1 ± 10.1%. There was a statistically significant increase in RVEDVi and RVESVi from the first and the second scan to the third scan (p &amp;lt; 0.01). RVEF was lower at the time of the third scan compared to the first and second scan, but this difference was not statistically significant. TAPSE increased slightly from the first to the third scan (p &amp;lt; 0.05). There was no change in stroke volume and LAS from the first to the third scan. Strong correlations were found between RVEF and LAS as well as between RVEF and TAPSE (r = 0.49 and r=-0.50; p &amp;lt; 0.001, respectively). Conclusion Serial assessment of CMR studies in HLHS patients after TCPC completion could demonstrate an increase in indexed RV volumes over time, whereas RV stroke volume, RVEF and LAS largely remain stable.

Serial MRI-based right ventricular mechanical wall stress measurements and their association with right ventricle function in patients with repaired Tetralogy of Fallot

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Stichting Hartekind en Thorax Foundation Background Optimal timing of pulmonary valve replacement (PVR) in Tetralogy of Fallot (TOF) patients remains challenging. Wall stress is considered to be a possible early marker of right ventricular (RV) dysfunction. With patient-specific computational models, wall stress can be determined regionally and with high accuracy, especially in complex shaped ventricles such as in TOF patients. We aimed to 1) develop patient-specific computational models to assess RV diastolic wall stresses and 2) investigate the association of wall stresses and their change over time with functional parameters in TOF patients. Methods Repaired TOF patients with at least moderate pulmonary regurgitation (PR) and prior to PVR were included. MRI-based patient-specific computational ventricular models were created (figure). The ventricular geometry was created by stacking endo- and epicardial contours traced on short axis SSFP cine images. Pressure in the right ventricle was estimated from echocardiography. Mid-diastolic wall stress in the RV free wall was analysed globally and regionally (basal, mid, apical, anterior, lateral and posterior) at two time points. RV ejection fraction (RVEF), NT-proBNP and exercise tests (% maximum predicted workload) were used as outcomes for RV function. Associations between wall stresses and outcomes were investigated using linear mixed models adjusted for follow-up duration. Results Five males and five females were included with an age at baseline of 24 (IQR 16-28) years and RV end-diastolic volume of 140 (IQR 127-144) ml/m2. The period between the two time points was 7.0 (IQR 5.8-7.3) years. Global wall stress of the RV free wall combining both time points was 5.8 kPa (IQR 5.2-7.2). There was no statistical difference between baseline and follow-up global wall stress. The mean wall stresses in the mid region was 1.69 kPa (p &amp;lt; 0.01) higher than in the basal region and was 1.05 kPa (p = 0.03) higher than in the apical region cross-sectionally. The wall stress also increased more in the mid region compared to basal and apical region, corrected for duration of follow-up. Patients with more severe PR at baseline demonstrated a higher increase of global wall stress over time (p = 0.02), especially in lateral free wall. Higher global free wall stresses were cross-sectionally independently associated with lower RVEF, adjusted for LVEF and RVEDV (β=-1.29 % RVEF per kPa increase in wall stress, p = 0.01). This association was most prominent in the anterior, basal and mid part. No statistically significant association was found between wall stress, NT-proBNP, and exercise capacity. Conclusions This study generated a novel MRI-based method to calculate wall stress in geometrically complex ventricles. Wall stress associated negatively with RVEF in patients with TOF and PR. This promising tool for RV wall stress analysis can be used in future larger studies to validate these preliminary findings and to assess the predictive value of wall stress in TOF. Abstract Figure.

Right ventricular functional recovery depends on timing of pulmonary valve replacement in tetralogy of Fallot: a video kinematic study.

Indications for and timing of pulmonary valve replacement (PVR) after tetralogy of Fallot repair are controversial. Among magnetic resonance imaging indices proposed to time valve replacement, a right ventricular (RV) end-diastolic volume index greater than 160 ml/m2 is often used. Recent evidence suggests that this value may still identify patients with irreversible RV dysfunction, thus hindering recovery. Our goal was to define, using intraoperative video kinematic evaluation, whether a relationship exists between timing of PVR and early functional recovery after surgery. Between November 2016 and November 2018, a total of 12 consecutive patients aged 27.1 ± 19.1 years underwent PVR on average 22.2 ± 13.3 years after tetralogy of Fallot repair. Mean RV end-diastolic volume evident on the magnetic resonance images was 136.9 ± 35.7 ml/m2. Intraoperative cardiac kinematics were assessed by video kinematic evaluation via a high-speed camera acquiring videos at 200 fps before and after valve replacement. Patients presenting with RV end-diastolic volume <147 ml/m2 were significantly younger (11.2 ± 5.0 vs 38.4 ± 17.0; P = 0.005) and had a shorter time interval to valve replacement (11.0 ± 5.2 vs 30.1 ± 11.3; P = 0.03). The entire population showed a moderate correlation among energy expenditure, cardiac fatigue, perimeter of contraction and preoperative RV end-diastolic volume index. Both groups showed a reduction in all kinematic parameters after PVR, but those with end-diastolic volume >147 ml/m2 showed an unpredictable outcome. Video kinematic evaluation provides insight into intraoperative RV recovery in patients with tetralogy of Fallot undergoing PVR. Accordingly, functional recovery can be expected in patients with preoperative end-diastolic volume <147 ml/m2.

Short-Term Ventricular Structural Changes Following Left Ventricular Assist Device Implantation.

Reverse remodeling of the left ventricle has been reported following left ventricular assist device (LVAD) implantation. However, left ventricular (LV) and right ventricular (RV) volumetric and shape changes have not been described. Consecutive candidates for LVAD were prospectively enrolled. Comprehensive 2- and 3-dimensional echocardiographic (2DE, 3DE) images were acquired before and 1 to 2 months following LVAD implantation. 3D endocardial surfaces were analyzed to derive shape indices, including LV sphericity and conicity and RV septal and free-wall curvatures. Sixty patients were enrolled with a mean age 56 ± 13 years, 77% male, and 83% destination therapy. 3DE showed that LV end-diastolic volume (EDV) improved from 461 ± 182 to 287 ± 144 ml (p < 0.001) and RV EDV showed no change (p = 0.08). RV longitudinal strain (LS) worsened from -9.1 ± 3.1 to -5.9 ± 2.6% (p < 0.01). LV sphericity and conicity improved (p < 0.001 for both), whereas the curvature of the interventricular septum and RV free wall did not change (p = 0.79 and 0.26, respectively). At 1 month following LVAD implantation, LV volumes decrease dramatically, and there is a favorable LV shape improvement, indicating reverse remodeling. RV shape did not change, whereas RV LS worsened, indicating an absence of RV reverse remodeling.

Cardiac MRI assessment of the right ventricle pre-and post-kidney transplant.

Worsening renal function in chronic kidney disease correlates with worsening right ventricular (RV) systolic function. We evaluated the association between kidney transplantation (KT) and RV structure and systolic function, and the relationships between RV and left ventricular (LV) changes, blood pressure, and specific cardiac biomarkers, in patients with end-stage kidney disease using cardiac magnetic resonance imaging (CMR).In this prospective, multi-centre, cohort study, 39 adult patients on dialysis receiving KT and 42 patients eligible for, but not yet receiving KT, were recruited. CMR was performed at baseline, and repeated at 12 months.Among 81 patients (mean age 51 years, 30% female), RV end-diastolic volume index (RVEDVi), end-systolic volume index (RVESVi), mass index (RVMi), and ejection fraction (RVEF) did not change significantly within either the dialysis or KT group over 12months (all p ≥ 0.10). There were no significant differences in the 12-month changes of these parameters between the dialysis and KT groups (all p ≥ 0.10). RVMI demonstrated positive correlations with NT-proBNP and systolic blood pressure, but not GDF-15, at baseline and at 12months. Changes in RVEDVi, RVESVi, and RVEF were positively correlated with changes in LVEDVi, LVESVi, and LVEF, respectively over 12months (Spearman r = 0.72, 0.52, and 0.41; all p < 0.001), but not mass index (Spearman r = 0.20, p = 0.078).In conclusion,there were no significant changes in RV mass, volumes, or systolic function 12 months after KT, as compared with continuation of dialysis. The associations between RV and LV remodeling may suggest similar underlying pathophysiologic mechanisms.

The Pathophysiological Link between Right Atrial Remodeling and Functional Tricuspid Regurgitation in Patients with Atrial Fibrillation: A Three-Dimensional Echocardiography Study

Atrial fibrillation (AF) itself may lead to functional tricuspid regurgitation (FTR) through tricuspid annulus (TA) dilation. However, the pathophysiological determinants of TA enlargement in AF patients remain to be clarified. The objectives of this study were (1) to compare the TA size and function in AF patients versus healthy subjects; (2) to identify the determinants of TA remodeling in patients with AF and FTR; and (3) to assess the relationships among right heart structures and severity of FTR in AF patients. Eighty-three consecutive patients with long-term persistent AF and FTR (61±9.9years, 67% women) were prospectively enrolled and compared with 83 sex and body surface area-matched healthy subjects. Heart chamber size and function and TA geometry were analyzed using three-dimensional echocardiography. Among AF patients, 33%, 34%, and 33% had mild, moderate, and severe FTR, respectively. Right atrial (RA) dilation was detected in 93% of AF patients, while only 27% and 12% of them showed dilated or dysfunctional right ventricle (RV), respectively. End-diastolic TA area had the strongest correlation with the minimum volume of the RA (RAVmin r=0.6981, P<.0001) but only mild correlation with RV end-diastolic volume and sex (r=0.3405, P=.0019; r=0.2914, P=.0075). At multivariable analysis, only RAVmin was independently associated with TA area in AF patients (r=0.665, P<.0001). The RAVmin and TA area were the only predictors of FTR severity. In patients with AF, RA dilation seems to be more important than RV dilation to determine TA enlargement and subsequent FTR development. The RAVmin and TA area were directly correlated to FTR severity.

Right ventricle reverse remodelling following transcatheter and surgical secundum: Atrial septal defect closure in adults by cardiac magnetic resonance imaging

Background: Securely closed Atrial Septal Defect (ASD) leads to reduction in right cardiac dimensions and improvement of symptoms. Device has become an accepted alternative to surgical closure. CMR is an accurate imaging modality for the assessment of cardiac function and volumes. Objective: We aimed at studying electrical and mechanical remodeling in RV preclosure and 3 months after transcatheter and surgical closure. Results: Our prospective study included 30 consecutive adult patients with isolated secundum ASD referred to either transcatheter or surgical ASD closure. There was significant reduction in the QT dispersion among the same group as compared with the baseline values (QTc dispersion was 70.33 ± 24.04 to 60.26 ± 28.56 in device group vs. 80.73 ± 30.38 to 60.27 ± 28.57 in surgical group). There was no significant difference between two groups indicating that transcatheter and surgical closure had led to equivalent value of electrical remodelling (P-value >0.05). In CMR study we measured Right Ventricle End Diastolic Volume (RVEDV), Right Ventricle End Systolic Volume (RVESV), Right ventricle function and Right ventricle mass index. RVEDV and RVESV decreased significantly in both groups as compared to the baseline values (P-value <0.001). Transcatheter closure resulted in more significant reduction in the RVEDV and RVESV than the surgical closure (P-value=0.03 and 0.02 respectively), (Figures 1 and 2). In device closure; RV-function after closure was significantly higher in comparison to surgical closure (60.67 ± 5.12 vs. 52.73 ± 8.62 (%); P<0.001). The RV mass decreased significantly in each group as absolute value from the baseline (P-value (0.001) but it was statistically insignificant different between the two groups (P-value 0.31) while percentage of change was higher in device group (-) 20.38 ± 10.80 versus (-) 16.63 ± 14.91 in surgical group. Conclusion: Our study showed significant changes from baseline measurements with significant RV mechanical reverse remodeling in the device group but there were no significant changes in electrical remodeling between the two groups.

Comprehensive Assessment of Right Ventricular Function by Three-Dimensional Speckle-Tracking Echocardiography: Comparisons with Cardiac Magnetic Resonance Imaging

Three-dimensional speckle-tracking echocardiography (3D-STE) has been increasingly used to quantify right ventricular (RV) function. However, direct comparisons of 3D-STE with cardiac magnetic resonance (CMR) imaging for evaluation of RV function are limited. This study aimed to test the feasibility and accuracy of 3D-STE for the quantification of RV volumes, ejection fraction (EF), and longitudinal strain in comparison with CMR imaging and to determine whether 3D-STE for RV strain is superior to two-dimensional (2D) STE in comparison with CMR imaging. A total of 195 consecutive patients referred for both CMR imaging and echocardiography were studied. Right ventricular end-diastolic volume (RVEDV), RV end-systolic volume (RVESV), RVEF, and 3D RV longitudinal strain (3D-RVLS) of the free wall by 3D-STE and 2D-RVLS of the free wall by 2D-STE, were compared with CMR measurements. Pearson correlation and Bland-Altman analyses were used to assess the intertechnique agreement. Right ventricular 3D-STE was feasible in 174 patients (89%). Right ventricular volumes and EF determined by 3D-STE strongly correlated with CMR values (RVEDV, r=0.94; RVESV, r=0.96; RVEF, r=0.91; all P<.001). Three-dimensional STE slightly underestimated the RV volumes and longitudinal strain and overestimated the RVEF. The 3D-RVLS values correlated better than 2D-RVLS values with CMR values (0.85 vs 0.64, P<.001) with smaller bias and narrower limits of agreement (bias: 2.0 and 2.6; limits of agreement: 8.5 and 12.5, respectively). The bias and limits of agreement for 3D-STE-obtained RVLS were increased in patients with RV dilation, RVEF < 45%, or lower frame rate compared with those with normal RV size, RVEF ≥ 45%, or higher frame rate, respectively. Right ventricular 3D-STE measurements were highly reproducible. The 3D-STE measurements of RV volumes, EF, and longitudinal strain are highly feasible and reproducible, and data measured by 3D-STE correlate strongly with those determined using CMR imaging. Thus, 3D-STE may be a valid alternative to CMR imaging for the quantification of RV function in everyday clinical practice.

Evaluating the 12-Lead Electrocardiogram for Diagnosing ARVC in Young Populations: Implications for Preparticipation Screening of Athletes

BackgroundArrhythmogenic right-ventricular cardiomyopathy (ARVC) is an identified cause of sport-related sudden cardiac arrest (SCA). Identifying athletes with ARVC and restricting them from exercise is believed to reduce the risk of SCA. The electrocardiogram (ECG) is considered to be an important component of screening for ARVC; however, the sensitivity of the 12-lead ECG to identify ARVC in young asymptomatic persons is unknown. MethodsIn this retrospective study, we identified 70 patients (49 ARVC-positive, based on Task Force Criteria, and 21 age-matched ARVC-negative persons from a paediatric arrhythmia database (<18 years of age); ECGs were analyzed for abnormalities, based on International Criteria for Interpretation of ECGs in Athletes, and ECG findings were adjudicated by group consensus. ResultsOf the 49 ARVC-positive patients (median age: 17 [interquartile range: 16-18], 65% male), 22% were found to have abnormal ECGs; the most common ECG findings were T-wave inversions. Patients with symptoms were more likely to have abnormal ECGs than asymptomatic patients (28% compared with 17%, respectively; P = 0.002). Of 16 gene-positive patients, 31% had abnormal ECGs. Patients with abnormal ECGs had larger right-ventricular end-diastolic volume indexes on magnetic resonance imaging than those with normal ECGs (P = 0.03). ConclusionsThe ECG was insensitive for detecting ARVC in young (age <18 years), asymptomatic patients, and is unlikely to provide significant diagnostic value for identifying ARVC on routine preparticipation screening of adolescent athletes.

Right atrial volume is a major determinant of tricuspid annulus area in functional tricuspid regurgitation: a three-dimensional echocardiographic study.

The aim of this study is to explore the relationships of tricuspid annulus area (TAA) with right atrial maximal volume (RAVmax) and right ventricular end-diastolic volume (RVEDV) in healthy subjects and patients with functional tricuspid regurgitation (FTR) of different aetiologies and severities. We enrolled 280 patients (median age 66 years, 59% women) with FTR due to left heart disease (LHD), pulmonary hypertension (PH), corrected tetralogy of Fallot (TOF), chronic atrial fibrillation (AF), and 210 healthy volunteers (45 years, 53% women). We measured TAA at mid-systole and end-diastole, tenting volume of tricuspid leaflets, RAVmax, and RVEDV by 3D echocardiography. Irrespective of TA measurement timing, TAA correlated more closely with RAVmax than with RVEDV in both controls and FTR patients. On multivariable analysis, RAVmax was the most important determinant of TAA, accounting for 41% (normals) and 56% (FTR) of TAA variance. In FTR patients, age, RVEDV, and left ventricular ejection fraction were also independently correlated with TAA. RAVmax (AUC = 0.81) and TAA (AUC = 0.78) had a greater ability than RVEDV (AUC = 0.72) to predict severe FTR (P < 0.05). Among FTR patients, those with AF had the largest RAVmax and smallest RVEDV. RAVmax and TA were significantly dilated in all FTR groups, except in TOF. PH and TOF had largest RVEDV, yet tenting volume was increased only in PH and LHD. RA volume is a major determinant of TAA, and RA enlargement is an important mechanism of TA dilation in FTR irrespective of cardiac rhythm and RV loading conditions.

Case 288.

History A 46-year-old woman was admitted to our hospital with decompensated congestive heart failure and pericardial effusion diagnosed on echocardiography. She had no family history of sudden cardiac death. She was born at term and experienced no cardiac events until 4 years of age, at which point she was hospitalized because of three syncopal episodes that were not related to exercise. Over the next 10 years, she experienced two additional episodes of syncope not related to exercise. She had another hospital admission at 12 years of age. Clinical examination did not reveal cyanosis or clubbing, peripheral pulses were normal, and blood pressure was 90/60 mmHg. Her venous pressure was elevated, but the liver was not enlarged, and the lung fields were clear. Electrocardiography showed sinus rhythm, right bundle branch block, T-wave inversion in V6, and evidence of right atrial dilatation. Two-dimensional echocardiography showed normal intracardiac connections, with the tricuspid valve in the normal position and normal size of the left atrium and left ventricle with a normal ejection fraction. The right ventricle was dilated without evidence of right ventricular outflow tract obstruction. Implantation of an implantable cardioverter-defibrillator was considered but was ultimately contraindicated because of right ventricle anatomy. Thus, the patient received conservative care and was started on digoxin and diuretics. At 32 years of age, she experienced an episode of atrial flutter that was treated with electrical cardioversion. As stated earlier, at 46 years of age, she was admitted to our hospital with decompensated heart failure to be evaluated for a heart transplant. She underwent electrocardiography, echocardiography, cardiac MRI with and without administration of contrast media, and non-cardiac-gated multidetector CT with and without contrast media to rule out pulmonary embolism. The following quantitative results were obtained with MRI: Left ventricular end-diastolic volume (LVDV) was 40 mL (LVDV per body surface area [BSA], 25 mL/m2); left ventricular end-systolic volume (LVSV), 21 mL (LVSV/BSA, 13 mL/m2); stroke volume (SV), 19 mL (SV/BSA, 12 mL/m2); and ejection fraction, 47%. Right ventricular end-diastolic volume (RVDV) was 262 mL (RVDV/BSA, 164 mL/m2); right ventricular end-systolic volume (RVSV), 198 mL (RVSV/BSA, 124 mL/m2); stroke volume (SV), 64 mL (SV/BSA, 40 mL/m2); and ejection fraction, 24%. Phase contrast sequences in the aorta and pulmonary artery showed systemic output of 20 mL and pulmonary output of 18 mL. Tricuspid regurgitation was massive (46 mL) (Figs 1-4).

Acute Unloading Effects of Sildenafil Enhance Right Ventricular–Pulmonary Artery Coupling in Heart Failure

BackgroundPhosphodiesterase-5A inhibitors (PDE5i) are sometimes used in patients with advanced heart failure with reduced ejection fraction before heart transplant or left ventricular assist device implantation to decrease right ventricular (RV) afterload and mitigate the risk of right heart failure. Conflicting evidence exists regarding the impact of these drugs on RV contractility. The aim of this study was to explore the acute effects of PDE5i on ventricular–vascular coupling and load-independent RV contractility. MethodsTwenty-two patients underwent right heart catheterization and gated equilibrium blood pool single photon emission computed tomography, before and after 20 mg intravenous sildenafil. Single photon emission computed tomography and right heart catheterization-derived data were used to calculate RV loading and contractility. ResultsPDE5i induced a decrease in the right atrial pressure (–43%), pulmonary artery (PA) mean pressure (–26%), and PA wedge pressure (PAWP; –23%), with favorable reductions in pulmonary vascular resistance (–41%) and PA elastance (–40%), and increased cardiac output (+13%) (all P < 0.01). The RV ejection fraction increased with sildenafil (+20%), with no change of RV contractility (P = 0.74), indicating that the improvement in the RV ejection fraction was related to enhanced RV–PA coupling (r = 0.59, P = 0.004) by a decrease in the ventricular load. RV diastolic compliance increased with sildenafil. The decrease in the PAWP correlated with RV end-diastolic volume decrease; no relationship was observed with the change in LV transmural pressure, suggesting decreased pericardial constraint. ConclusionsAcute PDE5i administration has profound RV afterload-reducing effects, improves the RVEF, decreases RV volumes, and decreases the PAWP, predominantly through relief of pericardial constraint, without effects on RV chamber contractility. These findings support further study of PDE5i in protection of RV function in advanced heart failure with reduced ejection fraction who are at risk of RV failure.

Abstract 13099: Evaluation of Right Ventricular Myocardial Mechanics by 2D-and 3D-STE in Patients With End-stage Heart Failure

Background: Speckle-tracking echocardiography (STE) has emerged as a more accurate tool for assessing the right ventricular (RV) function.We aimed to assess RV myocardial mechanics by 2D- and 3D-STE in patients with end-stage heart failure (HF). Methods: We enrolled 105 patients with end-stage HF and 50 normal subjects. The results of coronary artery angiography divided these patients into two subgroups : ischaemic cardiomyopathies (ICM, n=23) and non-ischaemic cardiomyopathies subgroups (NICM, n=82). RV end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF) were measured by 3D-STE. RV free wall longitudinal strain (RVLS-fre),and RV septal wall LS (RVLS-sep) were also determined by 3D-and 2D-STE. The conventional RV function parameters (fractional area change [FAC], tricuspid annular plane systolic excursion [TAPSE]) were obtained. We compared these parameters between patients and normal subjects. RVLS-fre and RVLS-sep obtained by 3D- and 2D-STE were compared between ICM and NICM. Results: Compared with controls, patients had higher RVEDV, RVESV(P&lt;0.001),and lower RVLS-fre, RVLS-spe, RVEF, RVSV, FAC, TAPSE(P&lt;0.001).In 105 patients, 3D-RVLS-fre was lower than 2D-RVLS-fre (-11.43±3.83% VS -12.30±3.95%;P=0.003); whereas 3D-RVLS-sep was not different from 2D-RVLS-sep. Patients with NICM had significant lower 3D-RVLS-fre(-10.68±3.55% VS -14.06±3.70%;P&lt;0.001)and 3D-RVLS-sep (-6.68±2.76% VS -8.26±2.64%;P=0.006)than patients with ICM. There was no difference in 2D-STE between the two subgroups. Conclusions: End-stage HF patients present significant difference in RVLS values between 3D- and 2D-STE. 3D-STE is superior to 2D-STE for RV systolic function assessment in end-stage HF patients with different pathologies.

Abstract 14221: Right Ventricular Adaptation to Pressure Overload: Differences Between Chronic Thromboembolic Pulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (iPAH)) are both associated with right ventricular (RV) failure and death. Although both conditions develop in the pre-capillary pulmonary vasculature, patient characteristics are different. CTEPH patients are older, predominantly male and more often have a history of venous thromboembolism. Therefore, the RV might be affected differently in CTEPH compared to iPAH. We aimed to compare RV adaptation in CTEPH and iPAH. Methods: Between 2000 and 2019 all treatment naive iPAH and CTEPH patients diagnosed in the Amsterdam UMC were included if a right heart catheterization and cardiac magnetic resonance imaging (CMR) were performed at the time of diagnosis. RV volumes, mass and function were assessed with CMR. RV contractility, afterload, RV-pulmonary artery (RV-PA) coupling and diastolic stiffness (Eed) were obtained using single beat pressure-volume loop analysis. Differences in RV phenotypes between iPAH and CTEPH were analyzed using multiple linear regression with interaction testing after correcting for confounders. Results: A total of 235 patients were included, 116 with CTEPH and 119 with iPAH. CTEPH patients were older, predominantly male, had a higher systemic blood pressure and a lower pulmonary vascular resistance at the time of diagnosis. After correcting for these confounders, RV function and RV-PA coupling were similar in both groups. However, CTEPH patients had a higher RV end-diastolic volume index (87±27 ml/m2 vs. 82±25 ml/m2), and a lower RV wall thickness (0,6±0,1 g/ml vs. 0,7±0,2 g/ml; figure 1A). The increase in afterload in CTEPH was associated with a disproportionally larger increase in diastolic stiffness compared to iPAH, independent of RV wall thickness (figure 1B). Conclusions: Despite a similar RV function, the RV in CTEPH is more dilated and stiffer than the RV in iPAH, independent of age, sex and afterload.

Balloon pulmonary angioplasty reverse right ventricular remodelling and dysfunction in patients with inoperable chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis.

Right ventricular (RV) function is considered the major determinant of prognosis in patients with chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this meta-analysis was to evaluate RV remodelling and function following balloon pulmonary angioplasty (BPA) in patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension (PH) after pulmonary endarterectomy (PEA). We reviewed all studies evaluating RV function by cardiac magnetic resonance (CMR) and/or echocardiography pre- and post-BPA from PubMed/Medline prior to 15 December 2019. Ten (299 patients) of the 29 studies retrieved met the inclusion criteria: 5 CMR and 5 echocardiography studies. The systematic review and meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Guidelines. Pooled data from CMR studies revealed BPA resulted in a significantly decreased RV end-diastolic volume index (weighted mean difference (WMD) - 28.33 ml/m2, p < 0.00001) and RV end-systolic volume index (WMD - 29.06 ml/m2, p < 0.00001) accompanied by an increased RV ejection fraction (RVEF, WMD 8.97%, p < 0.00001). Data from the echocardiography studies showed BPA resulted in decreased RV basal diameter (WMD - 0.37 cm, p = 0.0009) and an increase of RV fractional area change (WMD 5.97 %, p = 0.003), but improvements of tricuspid annular plane systolic excursion (TAPSE) and S' were not significant. BPA improves RVEF and decreases RV volumes in patients with inoperable CTEPH or persistent/recurrent PH after PEA. • Balloon pulmonary angioplasty improves RVEF and decreases RV volumes in patients with inoperable CTEPH or persistent/recurrent PH after PEA.

Comparison of Right Ventricular Function Between Three-Dimensional Transesophageal Echocardiography and Pulmonary Artery Catheter

This study aimed to compare measurements of right ventricular function using three-dimensional transesophageal echocardiography (3D TEE), and pulmonary artery catheters (PACs) in patients undergoing cardiac surgery. The authors examined the practicality of using the 3D TEE. Prospective observational. Cardiac operating room at a single university hospital. All adult patients undergoing elective cardiac surgery at a single tertiary care university hospital over two years. None. Right ventricular end-diastolic volume (RVEDV), right ventricular end-systolic volume (RVESV), stroke volume (SV), and right ventricular ejection fraction (RVEF) were measured with both 3D TEE and PACs. Assessments were performed using correlation coefficients, paired t tests, and Bland-Altman plots. Thirty-one patients participated in this study. Each measurement showed good agreement. RVEDV and RVESV were slightly lower on 3D TEE than on PAC (205.9 mL v 220.2 mL, p = 0.0018; 143.0 mL v 155.5 mL, p = 0.0143, respectively), whereas no significant differences were observed for SV and RVEF (31.0% v 31.1%, p = 0.0569; 61.6 mL v 66.9 mL, p = 0.92, respectively). Linear regression analysis showed high correlation between 3D TEE and PAC for RVEDV (r = 0.87) and RVESV (r = 0.81), and moderate correlation for SV (r = 0.67) and RVEF (r = 0.67). In the Bland-Altman plot, most patients were within the 95% limits of the agreement throughout all measurements. A high correlation was found between measurements made with a PAC and with 3D TEE in the assessment of right ventricular function. Three-dimensional TEE would be a potential alternative to PAC for assessment of right ventricular function during intraoperative periods.