Cardiac MRI-derived ventricular-pulmonary arterial coupling predicts outcomes in connective tissue disease-associated pulmonary arterial hypertension patients.

Introduction Speckle-tracking echocardiography plays an increasingly important role in the assessment of aortic stenosis (AS), the most common valvular heart disease in the Western population. Right ventricular (RV) and left ventricular (LV) global longitudinal strain (GLS) were studied for their association with all-cause mortality in AS patients undergoing transcatheter aortic valve implantation (TAVI). Purpose To investigate whether simultaneous assessment of RV and LV GLS provides a better association with all-cause mortality during long-term follow-up after TAVI. Methods In our prospective registry we identified 109 patients with severe AS who underwent TAVI and had a pre-procedural echocardiography within three months of the procedure allowing complete assessment of RV and LV GLS using TomTec Image Arena. All-cause mortality was defined as endpoint. Results RV GLS was lower among non-survivors (N=36; median [IQR]: −13.94 [−16.31 to −12.62]; p<0.001) than survivors (N=73; −17.04 [−20.16 to −15.22]; p<0.001), while LV GLS did not differ (p=0.249). RV GLS >−16.6% differentiated survivors from non-survivors (sensitivity 78%; specificity 63%; ROC AUC 71%; p<0.001), while LV GLS did not (p=0.243). Kaplan Meier curves showed good differentiation of survivors and non-survivors with the RV GLS (p<0.001; Figure 1), but not the LV GLS cut-off (p=0.058). In univariable Cox regression models, RV GLS was associated with all-cause mortality (HR 1.10 [95% CI 1.02 to 1.18]; χ2=6.64; p=0.01), while LV GLS was not (HR 1.05 [0.96 to 1.16]; χ2=1.08; p=0.299). In bivariable models, association of RV GLS with all-cause mortality was independent of LV GLS or LV ejection fraction (LVEF; Table 1). ANOVA likelihood ratios revealed that inclusion of RV GLS to LV GLS or LVEF improved their model fitness, while that of LV GLS did not (Table). Conclusion RV GLS was associated with all-cause mortality after TAVI, while LV GLS and LVEF were not. The association of RV GLS was independent of LV GLS and LVEF and showed potential incremental value for assessment of outcome association, while LV GLS did not. Funding Acknowledgement Type of funding sources: None.

ObjectiveTo evaluate the prognostic value of stroke volume index (SVI) compared to cardiac index (CI) in risk stratification and outcome prediction in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).MethodsWe performed a retrospective analysis of patients diagnosed with CTD-PAH through right heart catheterization (RHC) from two Chinese medical centers. This retrospective study analyzed 206 CTD-PAH patients, with risk stratification performed using the 2018 World Symposia on Pulmonary Hypertension (WSPH) framework. Restricted cubic splines (RCS) and log-rank tests were utilized to identify the optimal SVI cutof values for categorizing patients into low-, intermediate-, and high-risk groups. Kaplan-Meier (KM) curves were used to analyze survival rates and event-free survival. Receiver operating characteristic (ROC) analysis was used to assess the predictive accuracy of diferent models for prognostic outcomes.ResultsSVI was categorized into low-risk (SVI ≥ 33.35 mL/m2), intermediate-risk (24.66 mL/m2≤ SVI < 33.35 mL/m2), and high-risk (SVI < 24.66 mL/m2) groups. Among the 206 CTD-PAH patients, 55 exhibited discrepancies in risk stratification between CI and SVI. SVI-based stratification provided more accurate risk categorization and demonstrated superior predictive value compared to CI, showing significant diferences in both survival and event-free survival rates across the groups.ConclusionSVI enhances risk stratification and prognosis prediction in CTD-PAH by efectively distinguishing patients at higher risk for adverse outcomes.

Background\nIt is important to assess cardiac preload for management of patients undergoing off pump coronary artery bypass surgery (OPCAB). Recently, several studies have documented the good correlation between right ventricular end-diastolic volume index (RVEDVI) and stroke volume index (SVI), compared with cardiac filling pressures. However, none of these studies have evaluated relationship between predictors of preload and SVI measured with volumetric pulmonary artery catheter during OPCAB. The correlation of continuous RVEDVI and SVI measured with volumetric pulmonary artery catheter during OPCAB was evaluated in this study\n\nMethods\nFifty three patients undergoing OPCAB were included. Hemodynamic parameters were measured 10 min after induction (T1), 10 min after Y-graft formation started (T2) and 10 min after sternum closure (T3). The correlation of parameters were assessed by simple linear regression.\n\nResults\nCentral venous pressure (CVP) and pulmonary artery occlusion pressure (PAOP) did not correlate with SVI during OPCAB. On the other hand, a statistically significant result was found between RVEDVI and SVI at T2 (r2 = 0.133, P = 0.007) and T3 (r2 = 0.380, P &lt; 0.000). But RVEDVI and SVI were weakly correlated. And at T1, RVEDVI and SVI did not correlate.\n\nConclusions\nRVEDVI is a more reliable predictor of preload compared to CVP and PAOP during OPCAB. But in post-induction period (T2), RVEDVI did not correlate with SVI.

CMR-based estimation of mean pulmonary arterial pressure and pulmonary vascular resistance in connective tissue disease-associated pulmonary arterial hypertension.

Background Patients with chronic thromboembolic pulmonary disease (CTEPD) may have residual dyspnea on exertion, even after improvement of pulmonary hypertension (PH). Therefore, evaluation of dynamic right ventricular (RV) function using exercise cardiac magnetic resonance (exCMR) in CTEPD may offer insights into its hemodynamic pathophysiology and help explain exercise intolerance. While RV ejection fraction (RVEF) is recognized as a prognosticator in PH, global longitudinal strain (RVGLS) has an advantage in its load insensitiveness. However, the dynamic changes of RVGLS and its relationship to exercise tolerance in CTEPD have not yet been investigated. Objective To assess the dynamic RV function and the association between RVGLS and exercise tolerance in CTEPD. Methods ExCMR was conducted in CTEPD with PH (CTEPH, n=9), CTEPD without PH (CWoPH, n=10), and healthy subjects (Control, n=18). A cardiopulmonary exercise test (CPX) was performed to measure maximal workload (MWL). CMR was scanned at rest and during exercise at 40% MWL with pedaling in the supine position. Images of short axis views were analyzed by Aquarius to acquire left ventricular end-diastolic volume index (LVEDVI), end-systolic volume index (LVESVI), right ventricular end-diastolic volume index (RVEDVI), and end-systolic volume index (RVESVI). RV stroke volume (RVSV) index (RVSVI) was calculated as the difference of RVEDVI and RVESVI. RVEF was calculated by RVSV divided by RVEDV. RVGLS was analyzed from 4 chamber views using cvi42. Data are presented as mean ± SD. Two-way repeated measures analysis of variance post-hoc Bonferroni’s multiple comparisons test or Pearson correlation analysis was performed. Results Heart rate increased similary in each group during exercise (Figure1A). LVEDVI was smaller in CTEPH and decreased further during exercise (Figure 1B). Compared to Control, RVEDVI and RVESVI were higher in CTEPH, with no significant difference in CWoPH at rest (Figures 1C,1D). Exercise decreased RVESVI and increased RVSVI in Control, whereas it increased both RVEDVI and RVESVI, and did not increase RVSVI in CWoPH (Figures 1C, 1D, 1E). Exercise increased RVEF in Control, but decreased in CWoPH (Figure 1F). RVGLS was analyzed in CWoPH (n=6) and CTEPH (n=7). Exercise did not change RVGLS in CWoPH but worsened in CTEPH (Figure 2A). RVGLS during exercise (exRVGLS) correlated with %peak VO2 (R²=0.47, p&amp;lt;0.01, Figure 2B), VE vs. VCO2 slope (R²=0.37, p=0.03, Figure 2C), the ratio of the change in mean pulmonary artery pressure to cardiac output (ΔP/Q, R²=0.45, p=0.02, Figure 2D), but did not correlate with RVGLS at rest. Conclusions Exercise-induced RV dilatation and impaired RVSV augmentation were found in CWoPH. RVGLS at rest was impaired in both CTEPH and CWoPH, while exRVGLS did not change during exercise. exRVGLS in CTEPD correlated with exercise tolerance and severity of exercise-induced PH. exRVGLS may be a valuable marker for evaluating exercise tolerability.

Background Patients with mid-range ejection fraction heart failure (HFmrEF) are a new category defined in actual guidelines. The aim of this study was to establish the prognostic value of several parameters obtained by Cardiac Magnetic Resonance (CMR) and Transthoracic Echocardiography (TTE) in patients with HFmrEF. Methods Thirty patients, defined as HFmrEF by TTE and CMR, were included between 2012-2018. Patients with structural heart disease different from Left Ventricular (LV) dysfunction were excluded. Cine sequences in CMR (SSFP) were used to obtain atrial and ventricular volumes and mass. Myocardial fibrosis was quantified by late gadolinium enhancement. TTE was used to obtain anatomical and functional parameters as LV and Right Ventricular (RV) ejection-fraction, LV and RV global longitudinal strain, and RV free-wall longitudinal strain. The primary endpoint was the combination of all-cause death or heart failure admission. The median follow-up was 1.9 (0.5-3.3) years. Results Mean age was 59.3 ± 12.4 years, and 67.9% patients were male. The aetiology of LV dysfunction was mainly ischemic (n = 16, 53.3%). Results are shown in Table1. Patients who presented the primary endpoint had a lower RV ejection-fraction by CMR and a lower absolute value of RV free-wall longitudinal strain by TTE(Figure 1). Conclusions In HFmrEF patients, worse RV function (by CMR and TTE-Speckle Tracking) may be associated with a worse prognosis. Larger studies are needed to confirm this hypothesis. Table1 All-cause death or HF admission (n = 5; 16.7%) No all-cause death of HF admission (n = 25; 83.3%) p iRVEDV (ml/m2) by CMR 65.5 ± 13.5 66.2 ± 12.3 0.906 iRVESV (ml/m2) by CMR 30.4 ± 7.5 24.4 ± 6.2 0.065 iLVEDV (ml/m2) by CMR 85.8 ± 23.7 98 ± 19.5 0.225 iLVESV (ml/m2) by CMR 47.8 ± 15.3 54.9 ± 11.7 0.246 Indexed LA Volume (ml/m2) by CMR 42.8 (36.5 - 49) 48.4 (42 - 63.5) 0.386 LVEF (%) by CMR 44.9 ± 3.3 44 ± 2.6 0.506 RVEF (%) by CMR 52.2 ± 7.2 61.7 ± 7.2 0.012 RV-FAC (%) by TTE 43.4 ± 4.4 44.7 ±7.5 0.378 TAPSE by TTE 2.1 ± 0.3 2.5 ± 0.1 0.032 LV Longitudinal Global Strain by TTE -14.3 ± 3.3 -15.5 ± 4.9 0.663 RV Longitudinal Global Strain by TTE -11.3 (-13.2 - -7.0) -19.5 (-23.7 - -10) 0.089 RV Free-Wall Longitudinal Strain by TTE -11.5 (-14.2 - -8.2) -20 (-26 - -13.7) 0.043 HF: Heart Failure; CMR: Cardio Magnetic Resonance; LV: Left Ventricle; RV: Right Ventricle; iRVEDV: Indexed RV End-Diastolic Volume; iRVESV: Indexed RV End-Systolic Volume; iLVEDV: Indexed LV End-Diastolic Volume; iLVESV: Indexed LV End-Systolic Volume; LA: Left Atrium; LVEF: LV Ejection Fraction; RVEF: RV Ejection Fraction; RV-FAC: RV Fractional Area Change; TAPSE: Tricuspid Annular Plane Systolic Excursion Abstract P816 Figure 1

Background: Cardiovascular magnetic resonance Feature Tracking (CMR-FT) is a well-established method to assess myocardial contraction with diagnostic and prognostic value in many diseases. We aimed to evaluate the role of right ventricular (RV) CMR-FT in the perioperative assessment of Ebstein patients undergoing Cone repair. Methods: We analyzed the CMR data of 18 Ebstein patients before and after Cone repair including CMR-FT-derived global radial (GRS), global circumferential (GCS) and global longitudinal strain (GLS). Results: Following Cone repair, tricuspid regurgitation decreased from 48% to 6%, p = 0.0001. RV ejection fraction (51% to 33%, p = 0.0002), indexed RV stroke volumes (74 mL/m2 to 43 mL/m2, p = 0.0013) and GLS (−15.01% to −14.53%, p = 0.0155) decreased postoperatively. Conversely, GRS (15.00% to 17.83%, p = 0.0202) and GCS (−8.82% to −13.02%, p = 0.0026) improved. Indexed RV end-diastolic volumes (RVEDVis) decreased, although not significantly, from 161 mL/m2 to 122 mL/m2, p = 0.3465. Eight patients exhibited a higher RVEDVi after surgery. Pulmonary artery and aortic flow and left ventricular (LV) functional parameters remained unchanged. Conclusions: RV GLS appears to be affected by the hemodynamic alterations caused by Cone repair. RV GCS and GRS might serve as more independent parameters of myocardial function.

Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to patients with idiopathic/hereditary pulmonary arterial hypertension (IPAH). We aimed to investigate differences in clinical characteristics, outcome and performance of the European Society of Cardiology (ESC)/ European Respiratory Society (ERS) risk stratification tool in these patient groups. This retrospective analysis included incident patients with CTD-PAH (n=197, of which 64 had interstitial lung disease, ILD) or IPAH (n=305) enrolled in the Swedish PAH Register (SPAHR) 2008–2019. Patients were classified as low, intermediate or high risk at baseline, according to the “SPAHR-equation”. One-year survival, stratified by type of PAH, was investigated by Cox proportional regression. At baseline, CTD-PAH patients had lower diffusing capacity for carbon monoxide and lower haemoglobin but, at the same time, lower N-terminal prohormone-brain natriuretic peptide, longer 6 min walk distance, better haemodynamics and more often a low-risk profile. No difference in age, World Health Organisation functional class (WHO-FC) or renal function between groups was found. One-year survival rates were 75, 82 and 83% in patients with CTD-PAH with ILD, CTD-PAH without ILD and IPAH, respectively. The 1-year mortality rates for low-, intermediate- and high-risk groups in the whole cohort were 0, 18 and 34% (p<0.001), respectively. Corresponding percentages for CTD-PAH with ILD, CTD-PAH without ILD and IPAH patients were: 0, 26, 67% (p=0.008); 0, 19, 39% (p=0.004); and 0, 16, 29% (p=0.001), respectively. The ESC/ERS risk assessment tool accurately identified low-risk patients but underestimated the 1-year mortality rate of CTD-PAH and IPAH patients assessed as having intermediate risk at diagnosis.

BackgroundPatients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), in particular systemic sclerosis (SSc), had an attenuated response compared with idiopathic PAH in most trials. Thus, there is uncertainty regarding...

The primary objective of this study is to investigate the potential of cardiac magnetic resonance (CMR) parameters to augment prognostic evaluation in patients with CTD-associated pulmonary arterial hypertension (CTD-PAH). A retrospective, single-centre cohort study was conducted on 110 patients with CTD-PAH who were diagnosed via right heart catheterization between 2017 and 2023. These patients underwent CMR examinations based on clinical indications. After a mean follow-up period of 27 months, 27 patients experienced clinical deterioration events. After adjusting for age, sex and COMPERA 2.0 risk assessment model parameters, five CMR metrics were identified as independent risk factors for clinical deterioration in CTD-PAH patients. Receiver operating characteristic curve analysis showed that combining COMPERA 2.0 risk assessment model with CMR metrics improved predictive performance, with interventricular septum extracellular volume (IVS ECV) providing the greatest benefit among tissue metrics and right ventricular ejection fraction (RVEF) showing the most improvement among right heart function metrics. Kaplan-Meier（KM）survival curves revealed that patients with RVEF <39.2% and IVS ECV >31.4% had the poorest prognosis. Calibration curves indicated that integrating RVEF and IVS ECV significantly enhanced the accuracy and reliability of the COMPERA 2.0 risk assessment model in predicting 1-, 2- and 3-year event-free survival rates in CTD-PAH patients, with the C-index improving from 0.626 to 0.805. Combining RVEF and IVS ECV with COMPERA 2.0 risk assessment model significantly enhances the model's predictive accuracy for clinical deterioration in CTD-PAH patients.

BackgroundRight ventricular (RV) dysfunction in ischemic cardiomyopathy (ICM) is associated with poor prognosis, but RV assessment by conventional echocardiography remains difficult. We sought to validate RV global longitudinal strain (RVGLS) and global longitudinal strain rate (RVGLSR) against cardiac magnetic resonance (CMR) and outcome in ICM.MethodsIn 57 patients (43 men, 64 ± 12 years) with ICM who underwent conventional and strain echocardiography and CMR, RVGLS and RVGLSR were measured off-line. RV dysfunction was determined by CMR [RV ejection fraction (RVEF) < 50%]. Patients were followed over 15 ± 9 months for a composite of death and hospitalization for worsening heart failure.ResultsRVGLS showed significant correlations with CMR RVEF (r = -0.797, p < 0.01), RV fractional area change (RVFAC, r = -0.530, p < 0.01), and tricuspid annular plane systolic excursion (TAPSE, r = -0.547, p < 0.01). RVGLSR showed significant correlations between CMR RVEF (r = -0.668, p < 0.01), RVFAC (r = -0.394, p < 0.01), and TAPSE (r = -0.435, p < 0.01). RVGLS and RVGLSR showed significant correlations with pulmonary vascular resistance (r = 0.527 and r = 0.500, p < 0.01, respectively). The best cutoff value of RVGLS for detection of RV dysfunction was -15.4% [areas under the curve (AUC) = 0.955, p < 0.01] with a sensitivity of 81% and specificity 95%. The best cutoff value for RVGLSR was -0.94 s-1 (AUC = 0.871, p < 0.01), sensitivity 72%, specificity 86%. During follow-up, there were 12 adverse events. In Cox-proportional hazard regression analysis, impaired RVGLS [hazard ratio (HR) = 5.46, p = 0.030] and impaired RVGLSR (HR = 3.95, p = 0.044) were associated with adverse clinical outcome.ConclusionCompared with conventional echocardiographic parameters, RVGLS and RVGLSR correlate better with CMR RVEF and outcome.

Associations Between Right Ventricular Remodeling, Exercise and Circulating Metabolites in Volume and Pressure Overload States.

Longitudinal Assessment of Right Ventricular Myocardial Strain in Relation to Transplant-Free Survival in Children with Idiopathic Pulmonary Hypertension

The purpose of this investigation was to assess hypoxic test effects on left and right ventricular contractility in patients with progressive systemic sclerosis (PSS) and pulmonary arterial hypertension (PAH). Ten patients (mean age 48.8±13.2 years) who were diagnosed with PSS and PAH were included in the study. All the patients underwent left and right heart catheterization. Right ventricular (RV) contractility was measured according to the method of Ferlinz [1] and left ventricular (LV) contractility according to the method of Kennedy et al. [2] using indirect digital substraction angiography. The mean pulmonary artery pressures (\(\bar P\)PA) and oxygen saturation of the pulmonary artery (SaO2) were registered at each stage of graded hypoxic exposure 14%, 12%, and 10% of O2. Right atrial pressures (PRA,syst, PRA,diast,\(\bar P\)RA), right ventricular pressures (PRV,syst, PRV,diast,\(\bar P\)RV, PRV,end-diast), right and left ventricular end-diastolic volume index (EDVI), end-systolic volume index (ESVI), stroke volume index (SVI), cardiac index (CI), ejection fraction (EF), and heart rate (HR) were calculated before and after breathing a hypoxic mixture of 10% of O2 for 30 minutes. The hypoxic test induced significant elevation (p<0.05) of\(\bar P\)PA, PRA,syst, PRA,diast,\(\bar P\)RA, PRV,syst, PRV,end-diast, RV EDVI, LV, EDVI, CI, and HR, whereas, SaO2 decreased significantly after the hypoxic test. These findings suggest that patients with PSS and PAH are characterized by hyperreactivity of pulmonary artery to hypoxia and the preservation of compensatory mechanism of RV and LV contractility.

ObjectivesTo evaluate initial combination therapy with ambrisentan plus tadalafil (COMB) compared with monotherapy of either agent (MONO), and the utility of baseline characteristics and risk stratification in predicting outcomes, in...