Right Ventricular End-diastolic Volume Index Research Articles

THE EFFECTS OF PREGNANCY IN SUBJECTS WITH REPAIRED TETRALOGY OF FALLOT

Previous reports reveal inconsistent findings of right ventricular (RV) changes following pregnancy in subjects with repaired tetralogy of Fallot (rTOF). A two-center, retrospective cohort study which included women with rTOF who completed pregnancy that were matched to nulliparous women with rTOF by age at the time of baseline cardiac magnetic resonance (CMR), RV ejection fraction (RVEF), and indexed RV end-diastolic volume (RVEDVi). Pre-pregnancy and postpartum cardiac magnetic resonance (CMR) were analyzed and compared to sequential CMR of nulliparous subjects with rTOF. Thirty-six women with rTOF who completed pregnancy were matched to 72 nulliparous women with rTOF. Over a mean period of 3.1 years for the pregnancy group and 2.7 years for the comparison group, there was no significant change in the RVEDVi, RVEF, RV mass, pulmonary regurgitation severity, left ventricular (LV) volumes, LV ejection fraction (LVEF), or LV mass when comparing the baseline CMR and the follow-up CMR in either of the groups. There was a slight increase in RV indexed end-systolic volume (RVESVi) when comparing the baseline CMR and the follow-up CMR in the pregnancy group (68.93, SD 23.34 ml/m2 at baseline vs. 72.97, SD 25.24 ml/m2 at follow-up, p= 0.028). Using a mixed effects model for CMR parameters change over time; when adjusted for time between baseline and follow-up CMR there was no significant difference in rate of change between the pregnancy and comparison groups. Most ventricular remodeling parameters measured by CMR did not significantly change in subjects with rTOF who completed pregnancy or in nulliparous subjects with rTOF. In the pregnancy group, RVESVi is larger in those individuals who have undergone pregnancy without a significant change in ventricular function. These patients should be followed longitudinally to determine the long-term ventricular and clinical effects of pregnancy.

Right ventricular scalloping index as cardiac magnetic resonance-derived marker for diagnosis of arrhythmogenic right ventricular cardiomyopathy.

The cardiac magnetic resonance (CMR) evaluation of right ventricular (RV) morphologic abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is subjective. Here, we aimed to use a quantitative index, the right ventricular scalloping index (RVSI), to standardize the measurement of RV free wall scalloping and aid in the imaging diagnosis. We retrospectively included 15 patients with definite ARVC and 45 age- and sex-matched patients with idiopathic right ventricular outflow tract ventricular arrhythmia (RVOT-VA) as controls. The RVSI was measured from cine images on four-chamber view to evaluate its ability to distinguish between ARVC and RVOT-VA patients. Other cardiac functional parameters including strain analysis were also performed. The RVSI was significantly higher in the ARVC than RVOT-VA group (1.56 ± 0.23 vs 1.30 ± 0.08, p < 0.001). The diagnostic performance of the RVSI was superior to the RV global longitudinal, circumferential, and radial strains, RV ejection fraction, and RV end-diastolic volume index. The RVSI demonstrated high intraobserver and interobserver reliability (intraclass correlation coefficient, 0.94 and 0.96, respectively). RVSI was a strong discriminator between ARVC and RVOT-VA patients (area under curve [AUC], 0.91; 95% CI, 0.82-0.99). A cutoff value of RVSI ≥1.49 provided an accuracy of 90.0%, specificity of 97.8%, sensitivity of 66.7%, positive predictive value (PPV) of 90.9%, and a negative predictive value (NPV) of 89.8%. In a multivariable analysis, a family history of ARVC or sudden cardiac death (odds ratio, 38.71; 95% CI, 1.48-1011.05; p = 0.028) and an RVSI ≥1.49 (odds ratio, 64.72; 95% CI, 4.58-914.63; p = 0.002) remained predictive of definite ARVC. RVSI is a quantitative method with good performance for the diagnosis of definite ARVC.

Meta-Analysis of Normal Reference Values for Right and Left Ventricular Quantification by Cardiovascular Magnetic Resonance.

Cardiovascular magnetic resonance (CMR) reference values are relied upon to accurately diagnose left ventricular (LV) and right ventricular (RV) pathologies. To date, reference values have been derived from modest sample sizes with limited patient diversity and attention to 1 but not both commonly used tracing techniques for papillary muscles and trabeculations. We sought to overcome these limitations by meta-analyzing normal reference values for CMR parameters stemming from multiple countries, vendors, analysts, and patient populations. We comprehensively extracted published and unpublished data from studies reporting CMR parameters in healthy adults. A steady-state free-precession short-axis stack at 1.5T or 3T was used to trace either counting the papillary muscles and trabeculations in the LV volume or mass. We used a novel Bayesian hierarchical meta-analysis model to derive the pooled lower and upper reference values for each CMR parameter. Our model accounted for the expected differences between tracing techniques by including informative prior distributions from a large external data set. A total of 254 studies from 25 different countries were systematically reviewed, representing 12 812 healthy adults, of which 52 were meta-analyzed. For LV parameters counting papillary muscles and trabeculations in the LV volume, pooled normative reference ranges in men and women, respectively, were as follows: LV ejection fraction of 52% to 73% and 54% to 75%, LV end-diastolic volume index of 60 to 109 and 56 to 96 mL/m2, LV end-systolic volume index of 18 to 45 and 16 to 38 mL/m2, and LV mass index of 41 to 76 and 33 to 57 g/m2. For LV parameters counting papillary muscles and trabeculations in the LV mass, pooled normative reference ranges in men and women, respectively, were as follows: LV ejection fraction of 57% to 74% and 57% to 75%, LV end-diastolic volume index of 60 to 97 and 55 to 88 mL/m2, LV end-systolic volume index of 18 to 37 and 15 to 34 mL/m2, and LV mass index of 50 to 83 and 38 to 65 g/m2. For RV parameters, pooled normative reference ranges in men and women, respectively, were as follows: RV ejection fraction of 47% to 68% and 49% to 71%, RV end-diastolic volume index of 64 to 115 and 57 to 99 mL/m2, RV end-systolic volume index of 23 to 52 and 18 to 42 mL/m2, and RV mass index of 14 to 29 and 13 to 25 g/m2. Our Bayesian hierarchical meta-analysis provides normative reference values for CMR parameters of LV and RV size, systolic function, and mass, encompassing both tracing techniques across a diverse multinational sample of healthy men and women.

Early Outcomes of Pulmonary Valve Replacement With the Edwards Inspiris Resilia Pericardial Bioprosthesis.

Controversy regarding the optimal pulmonary valve substitute remains, with no approved surgical valve for pulmonary valve replacement (PVR). Furthermore, unfavorable anatomy often precludes transcatheter PVR in patients with congenital heart disease. We therefore sought to evaluate the feasibility of the Edwards Inspiris pericardial aortic bioprosthesis in the pulmonary position in pediatric and adult patients requiring PVR. Data from consecutive patients who underwent PVR from February 2019 to February 2021 at our institution were retrospectively reviewed. Postoperative adverse events included paravalvular or transvalvular leak, endocarditis, explant, thromboembolism, valve thrombosis, valve-related bleeding, hemolysis, and structural valve degeneration. Progression of valve gradients was assessed from discharge to 30 days and one year. Of 24 patients with median age of 26 years (interquartile range [IQR]: 17-33; range: 4-60 years), 22 (91.7%) patients had previously undergone tetralogy of Fallot repair and 2 (8.3%) patients had undergone double-outlet right ventricle repair in the neonatal period or infancy. All patients had at least mild right ventricular (RV) dilatation (median RV end-diastolic volume index 161.4, IQR: 152.3-183.5 mL/m2) and at least moderate pulmonary insufficiency (95.8%) or stenosis (8.3%). Median cardiopulmonary bypass and cross-clamp times were 71 (IQR: 63-101) min and 66 (IQR: 60-114) min, respectively. At a median postoperative follow-up of 2.5 years (IQR: 1.4-2.6; range: 1.0-3.0 years), there were no mortalities, valve-related reoperations, or adverse events. Postoperative valve gradients and the severity of pulmonary regurgitation did not change significantly over time. At short-term follow-up, the bioprosthesis in this study demonstrated excellent safety and effectiveness for PVR. Further studies with longer follow-up are warranted.

Impact of Interventricular Interaction on Ventricular Function: Insights From Right Ventricular Pressure-Volume Analysis.

Interventricular interactions may be responsible for the decline in ventricular performance observed in various disease states that primarily affect the contralateral ventricle. This study sought to quantify the impact of such interactions on right ventricular (RV) size and function using clinically stable individuals with left ventricular assist devices (LVADs) as a model for assessing RV hemodynamics while LV loading conditions were acutely manipulated by changing device speed during hemodynamic optimization studies (ie, ramp tests). The investigators recorded RV pressure-volume loops with a conductance catheter at various speeds during ramp tests in 20 clinically stable HeartMate3 recipients. With faster LVAD speeds and greater LV unloading, indexed RV end-diastolic volume increased (72.28 ± 15.07mL at low speed vs 75.95 ± 16.90 at high speed; P = 0.04) whereas indexed end-systolic volumes remained neutral. This resulted in larger RV stroke volumes and shallower end-diastolic pressure-volume relationships. Concurrently, RV end-systolic pressure decreased (31.58 ± 9.75mL at low speed vs 29.58 ± 9.41mL at high speed; P = 0.02), but contractility, as measured by end-systolic elastance, did not change significantly. The reduction in RV end-systolic pressure was associated with a reduction in effective arterial elastance from 0.65 ± 0.43mmHg/mL at low speed to 0.54 ± 0.33mmHg/mL at high speed (P = 0.02). Interventricular interactions resulted in improved RV compliance, diminished afterload, and did not reduce RV contractility. These data challenge the prevailing view that interventricular interactions compromise RV function, which has important implications for the understanding of RV-LV interactions in various disease states, including post-LVAD RV dysfunction.

Abstract 13830: Pheochromocytoma in a Patient With Tetralogy of Fallot and Chromosome 17p12 Duplication

Description of Case: A 22-year-old male with Tetralogy of Fallot (TOF) status post transannular patch repair, hypothyroidism, and known partial duplication of chromosome 17p12 concerning for Charcot-Marie-Tooth disease (CMT), presented to his cardiologist with exertional angina and dyspnea, difficulty gaining weight, and diaphoresis. Cardiac MRI demonstrated moderate to severe pulmonic regurgitation with regurgitation fraction of 35% and RV end-diastolic volume index of 150 cc/m 2 . He was referred for pulmonic valve replacement surgery. While awaiting surgical evaluation, he presented to an ER with new hypertension with blood pressure to 240/140 and tachycardia to 107. A CT scan demonstrated a 4.3 x 5.0 x 4.4 cm mass in the right adrenal gland. On admission he was started on phenoxybenzamine and carvedilol. Workup revealed elevated catecholamines and metanephrines ( Table ). He underwent surgical adrenalectomy without complications. Immunohistochemical staining was consistent with pheochromocytoma. Post-procedurally, he was normotensive off medications. He was seen in clinic three weeks after surgery with significant improvement in symptoms. Discussion: We report on a novel genetic association of chromosome 17p12 duplication and pheochromocytoma. The region transcribes peripheral myelin protein-22, which has associations with CMT. Overlap has been demonstrated in other CMT-associated mutations and pheochromocytoma syndromes (e.g. kinesin family member 1B). In congenital heart disease, risk for pheochromocytoma is felt to be driven by hypoxia causing catecholamine secretion, adrenal hyperplasia, and eventual neoplasia. Further study is needed to characterize genetic associations between pheochromocytoma, neuropathy, and congenital heart disease. This patient’s presentation is a lesson in maintaining a high index of suspicion for pheochromocytoma in congenital heart disease, especially with associated genetic syndromes.

Right ventricular dilatation score: a new assessment to right ventricular dilatation in adult patients with repaired tetralogy of Fallot

BackgroundPatients with repaired tetralogy of Fallot (rTOF) experience long-term chronic pulmonary valve regurgitation resulting in right ventricular (RV) dilatation. According to current guidelines, the evaluation of patients with rTOF for RV dilatation should be based on cardiac magnetic resonance (CMR). However, for many asymptomatic patients, routine CMR is not practical. Our study aims to identify screening methods for CMR based on echocardiographic data, with the goal of establishing a more practical and cheap method of screening for severity of RV dilatation in patients with asymptomatic rTOF.MethodsThirty two rTOF patients (mean age, 21(10.5) y, 21 males) with moderate to severe pulmonary regurgitation (PR) were prospectively recruited. Each patient received CMR and echocardiogram examination within 1 month prior to operation and collected clinical data, and then received echocardiogram examination at discharge and 3–6 months post-surgery.ResultsRV moderate-severe dilatation was defined as right ventricular end-diastolic volume index (RVEDVI) ≥ 160 ml/m2 or right ventricular end-systolic volume index (RVESVI) ≥ 80 ml/m2 in 15 of 32 patients (RVEDVI, 202.15[171.51, 252.56] ml/m2, RVESVI, 111.99 [96.28, 171.74] ml/m2). The other 17 (RVESDI, 130.19 [117.91, 139.35] ml/m2, RVESVI = 67.91 [63.35, 73.11] ml/m2) were defined as right ventricle mild dilatation, i.e., RVEDVI < 160 ml/m2 and RVESVI < 80 ml/m2, and the two parameters were higher than normal values. Compared with the RV mild dilatation group, patients of RV moderate-severe dilatation have worse cardiac function before surgery (right ventricular ejection fraction, 38.92(9.19) % versus 48.31(5.53) %, p < 0.001; Left ventricular ejection fraction, 59.80(10.26) versus 66.41(4.15), p = 0.021). Patients with RV moderate-severe dilatation faced longer operation time and more blood transfusion during operation (operation time, 271.53(08.33) min versus 170.53(72.36) min, p < 0.01; Intraoperative blood transfusion, 200(175) ml versus 100(50) ml, p = 0.001). Postoperative RV moderate-severe dilatation patients have poor short-term prognosis, which was reflected in a longer postoperative hospital stay (6.59 [2.12] days versus 9.80 [5.10] days, p = 0.024) and a higher incidence of hypohepatia (0[0] % versus 4[26.7] %, p = 0.023). Patients with RV dilatation score > 2.35 were diagnosed with RV moderate-severe dilatation (AUC = 0,882; Sensitivity = 94.1%; Specificity = 77.3%).ConclusionsRV moderate-severe dilatation is associated with worse preoperative cardiac function and short-term prognosis after PVR in rTOF patients with moderate to severe PR. The RV dilatation score is an effective screening method. When RV dilatation score > 2.35, the patient is indicated for further CMR examination and treatment.

Long-Term Outcomes of Unrepaired Isolated Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum

The natural history of an unrepaired isolated partial anomalous pulmonary venous connection(s) (PAPVC) and the absence of other congenital anomalies remains unclear. This study aimed to expand the understanding of the clinical outcomes in this population. Isolated PAPVC with an intact atrial septum is a relatively uncommon condition. There is the perception that patients with isolated PAPVC are usually asymptomatic, that the lesion generally has a limited hemodynamic impact, and that surgical repair is rarely justified. For this retrospective study, we reviewed our institutional database to identify patients with either 1 or 2 anomalous pulmonary veins that drain a portion of but not the complete ipsilateral lung. Patients with previous surgical cardiac repair, coexistence of other congenital cardiac anomalies that would result in either pretricuspid or post-tricuspid loading of the right ventricle (RV), or scimitar syndrome were excluded. We reviewed their clinical course over the follow-up period. We identified 53 patients; 41 with a single and 12 with 2 anomalous PAPVC. A total of 30 patients (57%) were men, with a mean age at the latest clinic visit of 47 ± 19years (18 to 84 years). Turner syndrome (6 of 53, 11.3%), bicuspid aortic valve (6 of 53, 11.3%), and coarctation of the aorta (5 of 53, 9.4%) were commonly associated anomalies. A single anomalous left upper lobe vein was the most commonly identified variation. More than half of the patients were asymptomatic. Cardiopulmonary exercise test demonstrated a maximal oxygen consumption of 73 ± 20% expected (36 to 120). Transthoracic echocardiography demonstrated a mean RV basal diameter of 4.4 ± 0.8cm, RV systolic pressure of 38 ± 13 (16 to 84) mm Hg. A total of 8 patients (14.8%) had ≥moderate tricuspid regurgitation. Cardiac magnetic resonance in 42 patients demonstrated a mean RV end-diastolic volume index of 122 ±3 0ml/m2 (66 to 188ml/m2), of which in 8 (14.8%), it was >150ml/m2. Magnetic resonance imaging-based Qp:Qs was 1.6 ± 0.3. A total of 5 patients (9.3%) had established pulmonary hypertension (mean pulmonary artery pressure ≥25mm Hg). In conclusion, isolated single or dual anomalous pulmonary venous connection is not necessarily a benign congenital anomaly because a proportion of patients develop pulmonary hypertension and/or RV dilation. Regular follow-up and on-going patient surveillance with cardiac imaging is advised.

Electromechanical Dyssynchrony Is Associated With Right Ventricular Remodeling and Dysfunction Independently of Pulmonary Regurgitation Late After Tetralogy of Fallot Repair

The aim of this study was to investigate the relationship among right ventricular (RV) dilatation, dysfunction, and electromechanical dyssynchrony (EMD) in patients with repaired tetralogy of Fallot (rTOF). Data from a prospective rTOF registry of subjects with moderate or greater pulmonary regurgitation (PR) and contemporary imaging were analyzed. Electrocardiograms and echocardiograms were analyzed for EMD (prolonged QRS duration [QRSd], echocardiographic septal flash, and mechanical delay) and mechanical dispersion. The relationship among these, RV measurements on cardiac magnetic resonance, exercise capacity, and incident arrhythmia or death was analyzed with adjustment for PR. In total, 271 patients with rTOF (42% women; median age, 32years; interquartile range [IQR], 23-34 years) were included. Patients had moderate to severe PR (median PR fraction, 38%; IQR, 30%-47%), moderate to severe RV enlargement (median RV end-diastolic volume index, 161 mL/m2; IQR, 138-186 mL/m2) and mild RV systolic dysfunction (median RV ejection fraction [RVEF], 44%; IQR, 38%-48%). Eleven patients (4%) experienced ventricular arrhythmia or death. Presence of EMD was associated with larger RV size (RV end-diastolic volume index and RV end-systolic volume index, P=.006 and P<.001, respectively) and lower RVEF (P<.001). A sharp inflection in the relation among QRSd, RV size, and RVEF was observed when QRSd exceeded 150msec (3.1% decrease in RVEF for every 20-msec increase in QRSd between 160 and 200msec). Similar inflection points were observed for the mechanical delay between the RV basal-lateral and midseptal segments. The mechanical delay was higher in patients with vs without incident atrial arrhythmia (371 vs 276msec, P=.014). In adults with rTOF, EMD is independently associated with larger RV size, lower RVEF, and incident atrial arrhythmias.

Increased hepatic T1 values are correlated with right ventricular involvement and congestion after ST-segment elevation myocardial infarction

Abstract Funding Acknowledgements Type of funding sources: None. Background ST-segment elevation myocardial infarction (STEMI) may have a multiorgan impact. Due to its proximity to the heart, a large portion of the liver is imaged during T1 mapping in Cardiovascular Magnetic Resonance (CMR) and recently higher T1 have been found associated with signs of right heart failure (RHF) in patients with non-ischemic cardiomyopathy, but no data are available in STEMI population. Purpose To evaluate the role of early hepatic native T1 in detecting the presence of right ventricle (RV) involvement after STEMI. Methods 133 patients with STEMI who underwent early CMR after primary angioplasty (PPCI) were enrolled. Patients with known hepatic disease were excluded from the study. Hepatic T1 was evaluated considering the mean value obtained from three regions of interest, free from fat and blood vessels. RV involvement was diagnosed in the presence of RV ejection fraction (EF) reduction according to current guidelines, RV wall motion abnormalities and the presence of necrosis in late gadolinium enhancement (LGE) sequences. Results Overall, the mean age was 59.72 ± 13.32 years and 28.2% were female. Fifty-two (39.1%) patients had an anterior myocardial infarction. The median time from PPCI to CMR was 3 [IQR 2.00–5.00] days. T1 mapping was available in all patients and mean hepatic T1 value of the population was 622.91 ± 100.14 ms. According to the Youden index the optimal hepatic T1 cut-off value to predict RV involvement was 605 ms. A total of 72 (54.1%) patients presented high hepatic T1 values (&amp;gt;605 ms). No significant correlations were found between high hepatic T1 values and left ventricle EF, infarct size, area at risk and the presence of microvascular obstruction. High hepatic T1 values were correlated with larger RV end-diastolic volume index (RVEDVi) (median 83.03 ml/m2 vs 53.16 ml/m2, p&amp;lt;0.001), lower RVEF (median 39.4% vs 57.62%, p&amp;lt;0.001) and presence of LGE in RV (26.5% vs 9.8%, p = 0.015). White blood cells (WBC), C-reactive protein and hs-Tn T peak values did not differ in the two study groups but NT-proBNP values were significantly higher in patients with high hepatic T1 (median 3531.22 vs 111.25 pg/mL, p&amp;lt;0.001). A strong positive correlation was found between hepatic T1 and NTproBNP (r = 0.754, p&amp;lt;0.001, Figure 1). At the univariable analysis, RV EF (OR 0.848, 95%CI 0.806–0.893, p&amp;lt;0.001), RVEDVi (OR 1.026, 95%CI 1.013–1.038, p&amp;lt;0.001) and the presence of RV infarct at the CMR evaluation (OR 3.286, 95%CI 1.218–8.865, p = 0.019) were found predictors of high hepatic T1 values. On multivariable logistic regression, RVEDVi (OR 1.022, 95%CI 1.006–1.038, p = 0.008) and RV-EF (OR 0.854, 95%CI 0.809–0.902, p&amp;lt;0.001) resulted as independent predictors of increased hepatic T1 values. Conclusions RV involvement after STEMI may lead to hepatic congestion and high hepatic T1 values are associated with early RV dysfunction parameters (RVEDVi, RV-EF and LGE) at CMR and higher levels of NT-proBNP.

Mechanical adaptation of the right ventricle to increased volume overload associated with secondary tricuspid regurgitation

Abstract Funding Acknowledgements Type of funding sources: None. Background Right ventricular (RV) function is strongly associated with the prognosis of patients with secondary tricuspid regurgitation (STR). However, the mechanical adaptations (contraction pattern) of the RV to volume overload associated with STR remain to be clarified. Purpose To investigate the changes in RV contraction pattern associated with the increase in severity of STR and to analyse whether the assessment of each RV motion component of RV ejection fraction (RVEF) (longitudinal, radial, and anteroposterior) adds prognostic value in patients with STR. Methods Consecutive patients with STR who underwent clinically indicated transthoracic echocardiography were enrolled in a multi-center prospective observational study. The primary outcome was defined as heart failure hospitalization or cardiac death. 3-dimensional echocardiography studies were performed at baseline and the ReVISION software package (Argus Cognitive, Inc, Lebanon, NH) was used to quantify the contribution of the longitudinal, radial, and anteroposterior motion components to total RVEF (Figure). Results We enrolled 172 patients (43% men, age: 75±15 years), followed for a median of 9 months. The patients were divided in two groups by STR severity (non-severe n=125, severe n=47). The characteristics of patients with massive/torrential STR (n=12) vs. "only" severe STR (n=35) were also assessed. RV end-diastolic volume index (r=0.33, p&amp;lt;0.001) and the longitudinal component of RVEF demonstrated significant correlation with STR severity (r=-0.22, p&amp;lt;0.01), while total RVEF did not (r=0.14; p = NS). The anteroposterior component of RVEF was the only parameter showing relatively strong correlation with left ventricular ejection fraction (r=0.39; p&amp;lt;0.001). RVEF was comparable between non-severe and severe patients (49±10 vs. 49±11%, p = NS). In patients with severe STR, the radial and also anteroposterior component of RVEF were comparable to non-severe STR patients, while the longitudinal component was significantly lower in severe STR (19±6 vs.17±7%, p&amp;lt;0.05; shown on Figure). Notably, in patients with massive/torrential STR, the radial contribution markedly decreased (27±8 vs. 20±9%, p&amp;lt;0.05), with consequently reduced global RVEF (52±9 vs. 41±12%, p&amp;lt;0.01) (Figure). In a multivariable Cox regression, using parameters proved to be significant in the univariable analysis, longitudinal component of total RVEF was a significant and independent predictor of outcome (hazard ratio, 0.947 [CI, 0.903–0.992], P= 0.022). Conclusions Patients with severe STR demonstrate significant morphological and functional RV remodeling with chamber enlargement and maintained RVEF, but a significant decrease in the longitudinal component of RVEF. In patients with massive/torrential STR, the radial mechanism markedly deteriorates, leading to decreased global RVEF. The longitudinal component of RV motion demonstrated independent prognostic value in patients with STR.

PO-02-228 ASSOCIATION BETWEEN SIGNAL-AVERAGED ECG INDICES AND RV STRUCTURAL ABNORMALITIES IN PATIENTS WITH ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY: INSIGHTS FROM NORDIC ARVC REGISTER

The value of signal-averaged ECG (SAECG) derived ventricular late potentials (LP) as a minor depolarization criterion of arrhythmogenic right ventricular cardiomyopathy (ARVC) has been debated. Another criterion from the same category, terminal activation duration (TAD>55 ms), is obtained from standard V1 ECG lead, which makes it easier for use. However little data exist regarding the relationship between structural right ventricular (RV) ARVC substrate, LP and TAD. We aimed to explore the association between structural and functional RV abnormalities assessed using cardiac magnetic resonance (CMR), SAECG parameters, and abnormal TAD. Patients with definite ARVC diagnosis or genotype-positive family members who were recruited in the Nordic ARVC Registry with SAECG and CMR were included (n=268, mean age 42±16 years, female 46%, probands 49%). RV end-diastolic volume index (RVEDVI), RV ejection fraction (RVEF), and late gadolinium enhancement (LGE) presence were assessed. RV was defined as enlarged if RVEDVI > 100 ml/m2 for men and 90 ml/m2 for women. RVEF was considered reduced if lower than 45%. SAECG components were assessed using TFC2010 definitions: filtered QRS duration (fQRSd)>114 ms, duration of terminal low-amplitude QRS signal below 40 μV (LAS40)> 38 ms and root-mean-square voltage of the terminal 40 ms (RMS40)< 20 μV. An abnormal value of any one or more of these indexes was considered as the presence of LP. We assessed sensitivity and specificity of the SAECG indexes and TAD for association with abnormal RVEF, LGE and/or RVEDVI. C-statistics was used to assess the association between each of SAECG parameters as continuous variables and RV abnormalities. All 3 SAECG indexes were significantly associated with RV structural abnormalities using c-statistics (Table), each of them characterized by higher specificity and lower sensitivity than LP. TAD demonstrated high specificity but poor sensitivity in prediction of RV structural abnormalities. LP had lower specificity, but much higher sensitivity for association with RV structural and functional abnormalities than TAD. In a large cohort of patients with definite ARVC or genotype-positive family members SAECG indexes appear to have lower specificity for detection of structural and functional RV abnormalities compared to TAD, however its sensitivity far exceeds the one of the TAD and supports SAECG value as an ECG indicator of RV substrate.

Intracardiac Flow Analysis of the Right Ventricle in Pediatric Patients With Repaired Tetralogy of Fallot Using a Novel Color Doppler Velocity Reconstruction

Repaired tetralogy of Fallot (RTOF) patients will develop right ventricular (RV) dysfunction from chronic pulmonary regurgitation (PR). Cardiac magnetic resonance sequences such as four-dimensional flow can demonstrate altered vorticity and flow energy loss (FEL); however, they are not as available as conventional echocardiography (echo). The study determined whether a novel, vendor-independent Doppler velocity reconstruction (DoVeR) could measure RV intracardiac flow in conventional echo of RTOF patients. The primary hypothesis was that DoVeR could detect increased vorticity and diastolic FEL in RTOF patients. Repaired tetralogy of Fallot patients with echo were retrospectively paired with age-/size-matched controls. Doppler velocity reconstruction employed the stream function-vorticity equation to approximate intracardiac flow fields from color Doppler. A velocity field of the right ventricle was reconstructed from the apical 4-chamber view. Vortex strength (VS, area integral of vorticity) and FEL were derived from DoVeR. Cardiac magnetic resonance and exercise stress parameters (performed within 1year of echo) were collected for analysis. Twenty RTOF patients and age-matched controls were included in the study. Mean regurgitant fraction was 40.5%±7.6%, and indexed RV end-diastolic volume was 158±36mL/m2. Repaired tetralogy of Fallot patients had higher total, mean diastolic, and peak diastolic VS (P=.0013, P=.0012, P=.0032, respectively) and higher total, mean diastolic, and peak diastolic body surface area-indexed FEL (P=.0016, P=.0022, P<.001, respectively). Peak diastolic indexed FEL and peak diastolic VS had weak-to-moderate negative correlation with RV ejection fraction (r=-0.52 [P=.019] and r=-0.49 [P=.030], respectively) and left ventricular ejection fraction (r=-0.47 [P=.034] and r=-0.64 [P=.002], respectively). Mean diastolic indexed FEL and VS had moderate-to-strong negative correlation with percent predicted maximal oxygen consumption (r=-0.69 [P=.012] and r=-0.75 [P=.006], respectively). DoVeR can detect alterations to intracardiac flow in RTOF patients from conventional color Doppler imaging. Echo-based measures of diastolic VS and FEL correlated with ventricular function. DoVeR has the potential to provide serial evaluation of abnormal flow dynamics in RTOF patients.

Characterization of Favorable Right Ventricular Dimensions for Optimal Reverse Remodeling Following Pulmonary Valve Replacement.

We sought to couple current cardiac magnetic resonance (CMR) thresholds of right ventricular (RV) size and function with longitudinal trajectories of RV recovery, after pulmonary valve replacement (PVR). We aimed to identify optimal timing of PVR and couple CMR-based metrics with contemporaneous echocardiographic metrics. From June 2002 to January 2019, 174 patients with severe pulmonary regurgitation and peak RV outflow tract gradient <30 mm Hg underwent PVR at Cleveland Clinic. Mean age was 35 ± 16 years and 60 (34%) had concomitant tricuspid valve surgery. RV end diastolic area index (RVEDAi) and function metrics were measured by offline image review on preoperative and 794 postoperative echocardiograms. Contemporaneous RV end diastolic volume index (RVEDVi) was assessed on CMR and correlated to RVEDAi. Multiphase nonlinear mixed-effects models were used to analyze the longitudinal change in RV size and function after PVR. RVEDAi was correlated with RVEDVi (P < 0.0001, r = 0.59). RVEDAi decreased slowly over 10 years following PVR. An inflection point at 24 cm2/m2 was noted at 1 year post-PVR and was associated with failure of RV reverse remodeling and RVEDVi ≥150 mL/m2. Compared to patients with preoperative RVEDVi ≥150 mL/m2, patients with RVEDVi <150 mL/m2 had accelerated recovery of longitudinal trajectories of RV size and function metrics on echocardiograms. Reverse remodeling of RV following PVR is an ongoing process. Current accepted threshold values for PVR are associated with greatest RV recovery, suggesting that earlier PVR is warranted. Echocardiography can potentially be utilized in lieu of CMR for surveillance and interventional triage.

Radial and Circumferential CMR-Based RV Strain Predicts Low R Wave Amplitude after ICD Implantation in Patients with Arrhythmogenic Cardiomyopathy.

Inadequate R wave amplitude (RWA) after implantable cardiac defibrillator (ICD) implantation in patients with arrhythmogenic cardiomyopathy (ACM) was suspected to relate to right ventricle impairment. However, little data-based evidence was provided to quantify the association. We retrospectively enrolled ACM patients receiving CMR examinations before transvenous ICD implantation from Fuwai Hospital. The RWA was obtained within 24 h and at 2-6-month follow-up after the operation. Structural, functional, as well as tissue characterization of the left ventricle (LV) and right ventricle (RV), were analyzed in relation to RWA. Among the 87 ACM patients (median RWA: 8.0 mV), 19 (21.8%) patients were found with low initial RWA (<5 mV) despite attempts in multiple positions. RV end diastolic diameter (RVEDD), (r = -0.44), RV ejection fraction (RVEF, r = 0.43), RV end diastolic volume index (RVEDVi, r = -0.49), RV end systolic volume index (RVESVi, r = -0.53), RV global circumferential (RVGCS, r = -0.64), and radial strain (RVGRS, r = 0.61, all p < 0.001) rather than LV metrics correlated strongly with initial RWA. RVGCS, RVESVi, and RVGRS were decent predictors of low RWA (areas under the curve AUC: 0.814, 0.769, 0.757, respectively) early after implantation and during 2-6-month follow-up. To summarize, low RWA of ICD lead in ACM patients was associated with RV abnormalities. The RVGCS, RVGRS, and RVESVi can be valuable predictors for identifying low RWA prior to ICD implantation.

Impact of Pulmonary Arterial Elastance on Right Ventricular Mechanics and Exercise Capacity in Repaired Tetralogy of Fallot.

Pathophysiological changes of right ventricle (RV) after repair of tetralogy of Fallot (TOF) are coupled with a highly compliant low-pressure pulmonary artery (PA) system. This study aimed to determine whether pulmonary vascular function was associated with RV parameters and exercise capacity, and its impact on RV remodeling after pulmonary valve replacement. In a total of 48 patients over 18 years of age with repaired TOF, pulmonary arterial elastance (Ea), RV volume data, and RV-PA coupling ratio were calculated and analyzed in relation to exercise capacity. Patients with a low Ea showed a more severe pulmonary regurgitation volume index, greater RV end-diastolic volume index, and greater effective RV stroke volume (p=0.039, p=0.013, and p=0.011, respectively). Patients with a high Ea had lower exercise capacity than those with a low Ea (peak oxygen consumption [peak VO2] rate: 25.8±7.7 vs. 34.3±5.5 mL/kg/min, respectively, p=0.003), while peak VO2 was inversely correlated with Ea and mean PA pressure (p=0.004 and p=0.004, respectively). In the univariate analysis, a higher preoperative RV end-diastolic volume index and RV end-systolic volume index, left ventricular end-systolic volume index, and higher RV-PA coupling ratio were risk factors for suboptimal outcomes. Preoperative RV volume and RV-PA coupling ratio reflecting the adaptive PA system response are important factors in optimal postoperative results. We found that PA vascular dysfunction, presenting as elevated Ea in TOF, may contribute to exercise intolerance. However, Ea was inversely correlated with pulmonary regurgitation (PR) severity, which may prevent PR, RV dilatation, and left ventricular dilatation in the absence of significant pulmonary stenosis.

Abstract 12930: Plasma Proteome Distinguishes Adaptive and Maladaptive Signaling in Repaired Tetralogy of Fallot

Introduction: Patients who have undergone tetralogy of Fallot (TOF) repair are subject to chronic volume and/or pressure overload, leading to a 40% probability of right ventricular (RV) failure by the third decade of life. We sought to identify a plasma proteome profile of the systemic response to right heart stress to better understand the mechanisms of RV failure and identify novel RV remodeling biomarkers. Methods: Patients with repaired TOF were recruited at the time of standard of care cardiac MRI (CMR) (N=14), and blood samples were collected. Plasma proteomics was assessed using data independent acquisition mass spectrometry. Proteins that were significantly affected between groups were identified and used for pathway enrichment analysis. Results: Patients underwent primary TOF repair at median 2.9m (IQR 0.8, 4.1) and CMR at median 16yrs (IQR 15, 18). Patients were grouped based on median indexed RV end diastolic volume (RVEDVi). Group 1: median RVEDVi 146ml/m 2 (IQR 139, 164) (N=9); Group 2: median RVEDVi 114ml/m 2 (IQR 104, 130) (N=5); p&lt;0.001. RV ejection fraction was equal between groups (mean 56% vs. 55%; p=0.68). Forty-one proteins were significantly differentially abundant: 19 enriched in Group 1 and 22 enriched in Group 2 (Fig 1; p&lt;0.05). Adaptive signaling in Group 1 was characterized by upregulation of proteins involved in antioxidant mechanisms (SOD1, TXN, PRDX1&amp;2), calcium handling (S100A6, CALM1), and proteostasis (UBB, HSPA8). Maladaptive signaling was characterized by upregulation of glycolytic pathways (MDH1, PGK1) and heme protein oxidation (ALAD) and downregulation of proteins to counter acute stress (complements, ORM1&amp;2). Conclusion: Patients with repaired TOF and moderate-severe RV dilation have a distinct maladaptive plasma proteomic profile independent of ejection fraction. These data suggest that profiling the circulating plasma proteome may be used to monitor disease progression prior to the onset of clinical heart failure.

Prediction of response to cardiac resynchronization therapy using cardiac magnetic resonance imaging in non-ischemic dilated cardiomyopathy

Abstract Background Cardiac resynchronization therapy (CRT) is a well-established therapy for symptomatic heart failure with reduced ejection fraction, but the response is different for individuals. Although many modalities have been conducted to predict CRT response, cardiac magnetic resonance (CMR) to predict CRT response has still insufficient usefulness. Purpose We determine whether the parameters including late gadolinium enhancement (LGE) identified in CMR could act as predictors of CRT response. Methods We retrospectively investigated 124 patients with non-ischemic dilated cardiomyopathy who underwent CMR before CRT implantation between Jan 2010 and July 2021 in a single center. CRT response was defined as a decrease in left ventricular end-systolic volume (LVESV) &amp;gt;15% on echocardiography after at least 3 months after CRT implantation. Results Among the study population (mean age 65.7±11.2 years, mean EF 25±6.5%, 50% of female), 85 (69%) patients were defined as CRT responder. The CRT responders had more left bundle branch block (LBBB) compared with non-responders [79 (92.9%) vs. 23 (59.0%), p&amp;lt;0.001], but there was a no difference of QRS duration (158.7 vs 165.0ms, p=0.054) between two groups. CMR analysis showed that there were no significant differences in the left ventricular (LV) chamber volume and LV ejection fraction between CRT-responder and non-responder. However, the right ventricular (RV) chamber volume was smaller (RV end-diastolic volume index, 86.3 vs 103.5 ml/m2, p=0.039; RV end-systolic volume index, 49.3 vs 68.5 ml/m2, p=0.013) and the RV ejection fraction (RVEF) was higher (46.9 vs 37.6%, p=0.002) in CRT-responders compared with non-responders. The LGE on CMR was more shown in non-responders than in CRT-responders [33 (84.6%) vs 45 (52.9%), p&amp;lt;0.001]. In CMR parameters, RV dysfunction (RVEF &amp;lt;45%) [Odds ratio (OR), 0.21 (0.05–0.93), p=0.045] and LGE [OR, 0.21 (0.05–0.58), p=0.01] were significantly associated with poor CRT response. Conclusions The presence of LGE and RV dysfunction on CMR were associated with poor CRT response in patients with non-ischemic dilated cardiomyopathy. Further investigation with CMR for pre-CRT patients is needed to support these results. Funding Acknowledgement Type of funding sources: None.

Ratio between left and right ventricular end-diastolic volumes and outcomes in patients with heart failure and preserved ejection fraction

Abstract Background Reference left and right ventricular (LV/RV) volumes normalized to age and gender have been published. However, the relative dilation of the LV compared to the RV in patients with heart failure (HF) symptoms and its prognostic association have not been evaluated. Purpose The present study investigated the relative dilation of the LV compared to the RV among patients with HF and preserved LV ejection fraction (HFpEF). We explored the association between LV/RV ratio (defined as the ratio between LV end-diastolic volume index [LVEDVi] and RV end-diastolic volume index [RVEDVi]) and outcomes. Methods Clinical and imaging data from consecutive ambulatory patients diagnosed with HFpEF between April 2011 and November 2021, and undergoing a cardiac magnetic resonance examination were retrieved. The endpoints were 1) all-cause death or first HF hospitalization, and 2) cardiovascular death or first HF hospitalization, 3) repeated HF hospitalizations. Results A total of 159 patients (median age 58 years [interquartile range 49–69], 64% men) were included. Median LVEF was 60% (54–70%), and the LV/RV ratio was 1.21 (1.07–1.40). Over a 3.5-year follow-up (1.5–5.0), all-cause death or first HF hospitalization occurred in 23 patients (15%) and cardiovascular death or first HF hospitalization in 22 (14%). Spline curve analysis showed a bimodal relationship between LV/RV and both outcomes, with a steep increase in risk &amp;lt;1.0 and ≥1.4 (Figure 1). Accordingly, patients with either LV/RV &amp;lt;1.0 or ≥1.4 had a much shorter survival free from both endpoints than patients with LV/RV 1.0–1.3 (Figure 2). An LV/RV &amp;lt;1 was associated with a higher risk of all-cause death or first HF hospitalization (hazard ratio [HR] 5.95, 95% confidence interval [CI] 1.67–21.28; p=0.006) and a higher risk of cardiovascular death or first HF hospitalization (HR 5.68, 95% CI 1.58–20.35; p=0.008). Furthermore, an LV/RV ≥1.4 was associated with a higher risk of all-cause death or first HF hospitalization (HR 4.10, 95% CI 1.58–10.61; p=0.004) and a higher risk of cardiovascular death or first HF hospitalization (HR 3.71, 95% CI 1.41–9.79; p=0.008). Nine patients (6%) had more than 1 HF hospitalization. The crude incidence of HF hospitalizations was much higher in patients with an LV/RV &amp;lt;1.0 (16.6 per 100 patient/years) or ≥1.4 (10.29 per 100 patient/years) than in those with LV/RV 1–1.3 (1.88 per 100 patient/years). Multivariable binomial negative regression showed significant association between LV/RV and recurrent HF hospitalizations after adjustment by age, gender and New York Heart Association class: LV/RV &amp;lt;1.0 vs. 1.0–1.3, incidence rate ratio 9.0 per 100 patient/years (4.1–19.6), p&amp;lt;0.001; LV/RV ≥1.4 vs. 1.0–1.3, incidence rate ratio 5.3 per 100 patient/years (1.5–8.4), p=0.009. Conclusions Among patients with HFpEF, an RVEDVi larger than the LVEDVi, or an LVEDVi ≥40% larger than the RVEDVi were significantly associated with worse outcomes. Funding Acknowledgement Type of funding sources: None.

Abnormal flow conduction through pulmonary arteries is associated with right ventricular volume and function in patients with repaired tetralogy of Fallot: does flow quality affect afterload?

Flow through the proximal pulmonary arteries (PAs) of patients with repaired Tetralogy of Fallot (TOF) is known to be highly disordered and associated with significant regurgitation. The purpose of this study was to evaluate 4D-Flow MRI-derived viscous energy loss [Formula: see text])-as a result of non-efficient flow propagation, and relate this parameter to standard right ventricular (RV) size and function markers in patients with repaired TOF. Thirty-five patients with TOF and 14 controls underwent comprehensive 4D-Flow MRI evaluation for qualitative flow analysis and to calculate [Formula: see text] in the main and right pulmonary arteries. Sampled [Formula: see text] indices were correlated with the MRI-derived RV size and functional indices. All patients with TOF exhibited abnormal, supra-physiologic helical/vortical formations in the PAs. Patients with TOF had significantly increased peak systolic [Formula: see text] (8.0 vs 0.5 mW, p < 0.001), time-averaged [Formula: see text] (2.5 vs. 0.2 mW, p < 0.001), and peak systolic [Formula: see text] indexed to stroke volume (0.082 vs. 0.012 mW/mL, p < 0.001). [Formula: see text] indexed to stroke volume correlated with the RV end-diastolic volume (R = 0.68, p < 0.001), end-systolic volume (R = 0.62, p < 0.001), ejection fraction (R = -0.45, p = 0.002), and cardiac index (R = 0.45, p = 0.002). The mean estimated energy loss due to [Formula: see text] with regard to input RV mechanical power was 4.7%. This study demonstrates that patients with repaired TOF have highly abnormal flow conduction through the PAs which result into extensive viscous energy loss. This significant flow-mediated energy loss is associated with the RV volume and function, and might represent considerable loss of mechanical power generated by each cardiac cycle. Future studies are required to assess whether the abnormal flow conduction adds to the RV afterload and remodeling. • Abnormal flow patterns through proximal pulmonary arteries in patients with TOF are associated with excessive viscous energy loss. • Inefficient flow conduction is associated with the RV dilation and reduced function and might contribute to the RV adaptive remodeling.