Prognostic Implication of Right Ventricular Free Wall Longitudinal Strain and Right Atrial Pressure Estimated By Echocardiography in Patients With Severe Functional Tricuspid Regurgitation.

BACKGROUND: The interaction between right ventricular (RV) function and pulmonary hypertension is crucial for prognosis of patients with severe functional tricuspid regurgitation. RV free wall longitudinal strain (RVFWLS) has been reported to detect RV systolic dysfunction earlier than other conventional parameters. Although pulmonary artery systolic pressure measured by Doppler echocardiography is often underestimated in severe functional tricuspid regurgitation, right atrial pressure (RAP) estimated by echocardiography may be viewed as a prognostic factor. Impact of RAP and RVFWLS on outcome in patients with severe functional tricuspid regurgitation remains unclear. The aim of the present study was to investigate prognostic implication of RAP, RVFWLS, and their combination in this population. METHODS AND RESULTS: We retrospectively examined 377 patients with severe functional tricuspid regurgitation. RAP, pulmonary artery systolic pressure, RV fractional area change, and RVFWLS were analyzed. RAP of 15 mm Hg was classified as elevated RAP. All-cause death at 2-year follow-up was defined as the primary end point. RVFWLS provided better prognostic information than RV fractional area change by receiver operating characteristic curve analysis. In the multivariable Cox regression analysis, elevated RAP and RVFWLS of ≤18% were independent predictors of clinical outcome. Patients with RVFWLS of ≤18% had higher risk of all-cause death than those without by Kaplan-Meier curve analysis. Furthermore, when patients were stratified into 4 groups by RAP and RVFWLS, the group with elevated RAP and RVFWLS of ≤18% had the worst outcome. CONCLUSIONS: Elevated RAP and RVFWLS of ≤18% were independent predictors of all-cause death. The combination of elevated RAP and RVFWLS effectively stratified the all-cause death.

Feasibility Value of Right Ventricular Longitudinal Shortening Fraction and the Prognostic Implications in Patients With Heart Transplantation.

BACKGROUND: Right ventricular longitudinal shortening fraction (RVLSF) is a 2-dimensional speckle tracking echocardiography parameter based on tricuspid annular displacement analysis that could be used to assess right ventricular (RV) systolic function. The value of RVLSF in the assessment of RV systolic function in recipients of heart transplantation (HT) and whether RVLSF can replace strain parameters remains unknown. METHODS AND RESULTS: A total of 153 adult patients who underwent HT were consecutively enrolled in this prospective longitudinal study. All subjects were examined by conventional transthoracic 2-dimensional echocardiography and 2-dimensional speckle tracking echocardiography to evaluate the RV end-diastolic basal diameter, RV end-diastolic area, fractional area change, peak systolic velocity of tricuspid annulus, tricuspid annular plane systolic excursion, RV free wall strain, and RVLSF. Cox proportional hazards regression was used to test if the parameters of interest had independent prognostic value for adverse outcome prediction in patients who underwent HT. A significant positive correlation was found between the measurements of RVLSF and RV free wall strain (r=0.927, P<0.001). Compared with the event-free group, the adverse outcome group displayed reduced RVLSF and RV free wall strain and higher age (P<0.001, <0.001, =0.016, respectively) in patients who underwent HT. RVLSF and RV free wall strain were independently associated with poor prognosis in multivariable analysis (both P<0.001). CONCLUSIONS: RVLSF assessment provides an effective evaluation of RV longitudinal systolic function in the transplanted hearts and has prognostic value for adverse outcomes in patients undergoing HT.

Myocardial work across different etiologies of right ventricular dysfunction and healthy controls.

Evaluating right ventricular (RV) function remains a challenge. Recently, novel echocardiographic assessment of RV myocardial work (RVMW) by non-invasive pressure-strain loops was proposed. This enables evaluation of right ventriculoarterial coupling and quantifies RV dyssynchrony and post-systolic shortening. We aimed to assess RVMW in patients with different etiologies of RV dysfunction and healthy controls. We investigated healthy controls (n=17), patients with severe functional tricuspid regurgitation (FTR; n=22), and patients with precapillary pulmonary hypertension (PCPH; n=20). Echocardiography and right heart catheterization were performed to assess 1) RV global constructive work (RVGCW; work needed for systolic myocardial shortening and isovolumic relaxation), 2) RV global wasted work (RVGWW; myocardial shortening following pulmonic valve closure), and 3) RV global work efficiency (RVGWE; describes the relation between RV constructive and wasted work). RVGCW correlated with invasive RV stroke work index (r=0.66, P<0.001) and increased in tandem with higher afterload, i.e., was low in healthy controls (454±73 mmHg%), moderate in patients with FTR (687±203 mmHg%), and highest among patients with PCPH (881±255 mmHg%). RVGWE was lower and RVGWW was higher in patients with FTR (86±8% and 91 mmHg% [53-140]) or PCPH (86±10% and 110 mmHg% [66-159]) as compared with healthy controls (96±3% and 10 mmHg%). RVMW by echocardiography provides a promising index of RV function to discriminate between patients with RV volume or pressure overload. The prognostic value of this measure needs to be settled in future studies.

Right ventricular-pulmonary arterial coupling ratio derived from 3-dimensional echocardiography predicts outcomes in systemic lupus erythematosus-associated pulmonary arterial hypertension patients.

BACKGROUND: Systemic lupus erythematosus (SLE) is a complex autoimmune connective tissue disease (CTD) that is an important cause of devastating pulmonary arterial hypertension (PAH), and persistent progression of PAH can lead to right heart failure, predicting a poor prognosis for SLE patients. Right ventricular-pulmonary arterial (RV-PA) coupling with echocardiography has been demonstrated to be a noninvasive alternative method for evaluating PAH patients' predictive outcomes. Whether the ratio of right ventricular stroke volume (RVSV) to right ventricular end-systolic volume (RVESV) measured by three-dimensional echocardiography (3DE) is a new index of RV-PA coupling has not been discussed as a new predictor for the clinical outcome of systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH). METHODS: From June 2019 to February 2023, 46 consecutive patients with SLE-PAH were enrolled prospectively, and their clinical data and echocardiographs were studied and analyzed. The control group consisted of 30 healthy subjects matched for age, sex, and body surface area (BSA). The main endpoints of this study were a composite of all-cause mortality and adverse clinical events. Baseline clinical characteristics and echocardiographic assessments were analyzed. RESULTS: During a median of 24 months (IQR 18-31), 16 of 46 SLE-PAH patients (34.7%) experienced endpoint-related events. At baseline, patients who experienced mortality or adverse events had a worse WHO functional class (WHO FC) and lower anti-double-stranded DNA (dsDNA) antibody levels. The right ventricular (RV) systolic dysfunction in SLE-PAH subjects was significantly worse than that in the healthy control group, especially in SLE-PAH patients in the endpoint event group. Compared to controls, patients with SLE-PAH had a lower RVSV/RVESV ratio. In the group comparison, patients who had experienced an endpoint event had a sequentially worse ratio (1.86 (1.65-2.3) versus 1.30 (1.09-1.46) versus 0.64 (0.59-0.67), p < .001). There were statistically significant associations between the RVSV/RVESV ratio to routine RV systolic function and clinical parameters. The RVSV/RVESV ratio was negatively correlated with the WHO FC (r = -0.621, p < .001) and positively correlated with the anti-dsDNA level. The ROC curve showed that the optimal cutoff for RVSV/RVESV < 0.712 determined a higher risk of poor prognosis. Kaplan‒Meier survival curves showed that an RVSV/RVESV ratio >0.712 was associated with more favorable long-term outcomes. CONCLUSIONS: The 3DE-derived SV/ESV ratio as a noninvasive alternative surrogate of RV-PA coupling was an eximious indicator for identifying endpoint events in SLE-PAH patients and can provide a diagnostic basis for clinical intervention.

Predictive value of Cardiac Magnetic Resonance: new and old parameters in the natural history of repaired Tetralogy of Fallot.

BACKGROUND: Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients. METHODS: 130 rTOF patients who underwent CMR (2006-2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome. RESULTS: Univariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p < .001; p = .037), HF (p = .049; p = .005), composite outcome (p = .039; p = .009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p < .001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy. CONCLUSIONS: Besides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role.

How to assess and treat right ventricular electromechanical dyssynchrony in post-repair tetralogy of Fallot: insights from imaging, invasive studies, and computational modelling.

BACKGROUND AND AIMS: Right bundle branch block (RBBB) and resulting right ventricular (RV) electromechanical discoordination are thought to play a role in the disease process of subpulmonary RV dysfunction that frequently occur post-repair tetralogy of Fallot (ToF). We sought to describe this disease entity, the role of pulmonary re-valvulation, and the potential added value of RV cardiac resynchronization therapy (RV-CRT). METHODS: Two patients with repaired ToF, complete RBBB, pulmonary regurgitation, and significantly decreased RV function underwent echocardiography, cardiac magnetic resonance, and an invasive study to evaluate the potential for RV-CRT as part of the management strategy. The data were used to personalize the CircAdapt model of the human heart and circulation. Resulting Digital Twins were analysed to quantify the relative effects of RV pressure and volume overload and to predict the effect of RV-CRT. RESULTS: Echocardiography showed components of a classic RV dyssynchrony pattern which could be reversed by RV-CRT during invasive study and resulted in acute improvement in RV systolic function. The Digital Twins confirmed a contribution of electromechanical RV dyssynchrony to RV dysfunction and suggested improvement of RV contraction efficiency after RV-CRT. The one patient who underwent successful permanent RV-CRT as part of the pulmonary re-valvulation procedure carried improvements that were in line with the predictions based on his Digital Twin. CONCLUSION: An integrative diagnostic approach to RV dysfunction, including the construction of Digital Twins may help to identify candidates for RV-CRT as part of the lifetime management of ToF and similar congenital heart lesions.

Role of Oxygen Starvation in Right Ventricular Decompensation and Failure in Pulmonary Arterial Hypertension.

Right ventricular (RV) function and eventually failure determine outcome in patients with pulmonary arterial hypertension (PAH). Initially, RV responds to an increased load caused by PAH with adaptive hypertrophy; however, eventually RV failure ensues. Unfortunately, it is unclear what causes the transition from compensated RV hypertrophy to decompensated RV failure. Moreover, at present, there are no therapies for RV failure; those for left ventricular (LV) failure are ineffective, and no therapies specifically targeting RV are available. Thus there is a clear need for understanding the biology of RV failure and differences in physiology and pathophysiology between RV and LV that can ultimately lead to development of such therapies. In this paper, we discuss RV adaptation and maladaptation in PAH, with a particular focus of oxygen delivery and hypoxia as the principal drivers of RV hypertrophy and failure, and attempt to pinpoint potential sites for therapy.

Postoperative Pulmonary Artery Pulsatility Index Improves Prediction of Right Ventricular Failure After Left Ventricular Assist Device Implantation.

OBJECTIVES: This study evaluated whether the postoperative pulmonary artery pulsatility index (PAPi) is associated with postoperative right ventricular dysfunction after durable left ventricular assist device (LVAD) implantation. DESIGN: Single-center retrospective observational cohort study. SETTING: The University of Kansas Medical Center, a tertiary-care academic medical center. PARTICIPANTS: Sixty-seven adult patients who underwent durable LVAD implantation between 2017 and 2019. INTERVENTIONS: All patients underwent open cardiac surgery with cardiopulmonary bypass under general anesthesia with pulmonary artery catheter insertion. MEASUREMENTS AND MAIN RESULTS: Clinical and hemodynamic data were collected before and after surgery. The Michigan right ventricular failure risk score and the European Registry for Patients with Mechanical Circulatory Support score were calculated for each patient. The primary outcome was right ventricular failure, defined as a composite of right ventricular mechanical circulatory support, inhaled pulmonary vasodilator therapy for 48 hours or greater, or inotrope use for 14 days or greater or at discharge. Thirty percent of this cohort (n = 20) met the primary outcome. Preoperative transpulmonary gradient (odds ratio [OR] 1.15, 95% CI 1.02-1.28), cardiac index (OR 0.83, 95% CI 0.71-0.98), and postoperative PAPi (OR 0.85, 95% CI 0.75-0.97) were the only hemodynamic variables associated with the primary outcome. The addition of postoperative PAPi was associated with improvement in the predictive model performance of the Michigan score (area under the receiver operating characteristic curve 0.73 v 0.56, p = 0.03). An optimal cutoff point for postoperative PAPi of 1.56 was found. CONCLUSIONS: The inclusion of postoperative PAPi offers more robust predictive power for right ventricular failure in patients undergoing durable LVAD implantation, compared with the use of existing risk scores alone.

Clinical predictors of right ventricular dysfunction and association with adverse outcomes in peripartum cardiomyopathy.

AIMS: We sought to identify factors associated with right ventricular (RV) dysfunction and elevated pulmonary artery systolic pressure (PASP) and association with adverse outcomes in peripartum cardiomyopathy (PPCM). METHODS AND RESULTS: We conducted a multi-centre cohort study to identify subjects with PPCM with the following criteria: left ventricular ejection fraction (LVEF) < 40%, development of heart failure within the last month of pregnancy or 5 months of delivery, and no other identifiable cause of heart failure with reduced ejection fraction. Outcomes included a composite of (i) major adverse events (need for extracorporeal membrane oxygenation, ventricular assist device, orthotopic heart transplantation, or death) or (ii) recurrent heart failure hospitalization. RV function was obtained from echocardiogram reports. In total, 229 women (1993-2017) met criteria for PPCM. Mean age was 32.4 ± 6.8 years, 28% were of African descent, 50 (22%) had RV dysfunction, and 38 (17%) had PASP ≥ 30 mmHg. After a median follow-up of 3.4 years (interquartile range 1.0-8.8), 58 (25%) experienced the composite outcome of adverse events. African descent, family history of cardiomyopathy, LVEF, and PASP were significant predictors of RV dysfunction. Using Cox proportional hazards models, we found that women with RV dysfunction were three times more likely to experience the adverse composite outcome: hazard ratio 3.21 (95% confidence interval: 1.11-9.28), P = 0.03, in a multivariable model adjusting for age, race, body mass index, preeclampsia, hypertension, diabetes, kidney disease, and LVEF. Women with PASP ≥ 30 mmHg had a lower probability of survival free from adverse events (log-rank P = 0.04). CONCLUSIONS: African descent and family history of cardiomyopathy were significant predictors of RV dysfunction. RV dysfunction and elevated PASP were significantly associated with a composite of major adverse cardiac events. This at-risk group may prompt closer monitoring or early referral for advanced therapies.

Aortic stenosis and right ventricular dysfunction.

At the present time, right ventricular function in patients with aortic stenosis is insufficiently taken into account in the decision-making process of aortic valve replacement. The aim of our study was to evaluate significance of right ventricular dysfunction in patients with severe aortic stenosis by modern 3D echocardiographic methods. This is prospective analysis of 68 patients with severe high and low-gradient aortic stenosis. We evaluated function of left and right ventricle on the basis of 3D reconstruction. Enddiastolic, endsystolic volumes, ejection fraction and stroke volumes of both chambers were assessed. There were more patients with right ventricular dysfunction in low-gradient group (RVEF < 45%) than in the high-gradient group (63.6% vs 39%, p = 0.02). Low-gradient patients had worse right ventricular function than high-gradient patients (RVEF 36% vs 46%, p = 0.02). There wasn't any significant correlation between the right ventricular dysfunction and pulmonary hypertension (r = - 0.25, p = 0.036). There was significant correlation between left and right ejection fraction (r = 0.78, p < 0.0001). Multiple regression analysis revealed that the only predictor of right ventricular function is the left ventricular function. According to our results we can state that right ventricular dysfunction is more common in patients with low-gradient than in high-gradient aortic stenosis and the only predictor of right ventricular dysfunction is left ventricular dysfunction, probably based on ventriculo-ventricular interaction. Pulmonary hypertension in patients with severe AS does not predict right ventricular dysfunction.

The association of Pulmonary Hypertension and right ventricular systolic function - updates in diagnosis and treatment.

Right ventricular (RV) systolic function is an essential but neglected component in cardiac evaluation, and its importance to the contribution to overall cardiac function is undermined. It is not only sensitive to the effect of left heart valve disease but is also more sensitive to changes in pressure overload than the left ventricle. Pulmonary Hypertension is the common and well-recognized complication of RV systolic dysfunction. It is also the leading cause of pulmonary valve disease and right ventricular dysfunction. Patients with a high pulmonary artery pressure (PAP) and a low RV ejection fraction have a seven-fold higher risk of death than heart failure patients with a normal PAP and RV ejection fraction. Furthermore, it is an independent predictor of survival in these patients. In this review, we examine the association of right ventricular systolic function with Pulmonary Hypertension by focusing on various pathological and clinical manifestations while assessing their impact. We also explore new 2022 ESC/ERS guidelines for diagnosing and treating right ventricular dysfunction in Pulmonary Hypertension.

Clinical and functional effects of beta-blocker therapy discontinuation in patients with biventricular heart failure.

BACKGROUND: Nearly two-thirds of patients with heart failure with reduced ejection fraction (HFrEF) have right ventricular dysfunction, previously identified as an independent predictor of reduced functional capacity and poor prognosis. Beta-blocker therapy (ß-BT) reduces mortality and hospitalizations in patients with HFrEF and is approved as first-line therapy regardless of concomitant right ventricular function. However, the exact role of sympathetic nervous system activation in right ventricular dysfunction and the potential usefulness (or harmfulness) of ß-BT in these patients are still unclear. OBJECTIVES: The aim of the study is to evaluate the medium-term effect of ß-BT discontinuation on functional capacity and right ventricular remodelling based on cardiopulmonary exercise testing (CPET), echocardiography and serum biomarkers in patients with clinically stable biventricular dysfunction. METHODS: In this single-centre, open-label, prospective trial, 16 patients were enrolled using the following criteria: patients were clinically stable without signs of peripheral congestion; NYHA II-III while on optimal medical therapy (including ß-BT); LVEF 40% or less; echocardiographic criteria of right ventricular dysfunction. Patients were randomized 1 : 1 either to withdraw (group 0) or continue (group 1) ß-BT. In group 0, optimal heart rate was obtained with alternative rate-control drugs. Echo and serum biomarkers were performed at baseline, after 3 and 6 months; CPET was performed at baseline and 6 months. Mann--Whitney U test was adopted to determine the relationships between ß-BT discontinuation and effects on right ventricular dysfunction. RESULTS: At 6 months' follow up, S' DTI improved (ΔS': 1.01 vs. -0.92 cm/s; P = 0.03), while estimated PAPs (ΔPAPs: 0.8 vs. -7.5 mmHg; P = 0.04) and echo left ventricular-remodelling (ΔEDVi: 19.55 vs. -0.96 ml/mq; P = 0.03) worsened in group 0. In absolute terms, the only variables significantly affected by ß-BT withdrawal were left ventricular EDV and ESV, appearing worse in group 0 (mean EDVi 115 vs. 84 ml/mq; mean ESVi 79 vs. 53.9 ml/mq, P = 0.03). No significant changes in terms of functional capacity were observed after ß-BT withdrawal. CONCLUSION: In HFrEF patients with concomitant right ventricular dysfunction, ß-BT discontinuation did not produce any beneficial effects. In addition, despite maintenance of optimal heart rate control, ß-BT discontinuation induced worsening of left ventricular remodelling. Our study corroborates the hypothesis that improvement in left ventricular function may likewise be a major determinant for improvement in right ventricular function, reducing pulmonary wedge pressure and right ventricular afterload, with only a marginal action of its negative inotropic effect. In conclusion, ß-BT appears beneficial also in heart failure patients with biventricular dysfunction.

Utility of Left and Right Ventricular Strain in Arrhythmogenic Right Ventricular Cardiomyopathy: A Prospective Multicenter Registry.

BACKGROUND: Imaging evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains challenging. Myocardial strain assessment by echocardiography is an increasingly utilized technique for detecting subclinical left ventricular (LV) and right ventricular (RV) dysfunction. We aimed to evaluate the diagnostic and prognostic utility of LV and RV strain in ARVC. METHODS: Patients with suspected ARVC (n = 109) from a multicenter registry were clinically phenotyped using the 2010 ARVC Revised Task Force Criteria and underwent baseline strain echocardiography. Diagnostic performance of LV and RV strain was evaluated using the area under the receiver operating characteristic curve analysis against the 2010 ARVC Revised Task Force Criteria, and the prognostic value was assessed using the Kaplan-Meier analysis. RESULTS: Mean age was 45.3±14.7 years, and 48% of patients were female. Estimation of RV strain was feasible in 99/109 (91%), and LV strain was feasible in 85/109 (78%) patients. ARVC prevalence by 2010 ARVC Revised Task Force Criteria is 91/109 (83%) and 83/99 (84%) in those with RV strain measurements. RV global longitudinal strain and RV free wall strain had diagnostic area under the receiver operating characteristic curve of 0.76 and 0.77, respectively (both P<0.001; difference NS). Abnormal RV global longitudinal strain phenotype (RV global longitudinal strain > -17.9%) and RV free wall strain phenotype (RV free wall strain > -21.2%) were identified in 41/69 (59%) and 56/69 (81%) of subjects, respectively, who were not identified by conventional echocardiographic criteria but still met the overall 2010 ARVC Revised Task Force Criteria for ARVC. LV global longitudinal strain did not add diagnostic value but was prognostic for composite end points of death, heart transplantation, or ventricular arrhythmia (log-rank P=0.04). CONCLUSIONS: In a prospective, multicenter registry of ARVC, RV strain assessment added diagnostic value to current echocardiographic criteria by identifying patients who are missed by current echocardiographic criteria yet still fulfill the diagnosis of ARVC. LV strain, by contrast, did not add incremental diagnostic value but was prognostic for identification of high-risk patients.

Right Ventricular Function in Surgical Treatment of Left Heart.

Aim    The aim of this study was to evaluate right ventricular (RV) function during left chamber surgery.Material and methods    This was a single-site prospective cohort study. The study included 197 patients with valvular pathology of heart left chambers. Mean age of patients was 58 [47; 65] years. Precordial echocardiography was performed preoperatively and within one week after surgery.Results    Decreased parameters of the right ventricular (RV) longitudinal function and global contractile function were observed postoperatively in the majority of patients. More noticeable decreases were observed in parameters of the longitudinal function (p<0.001). Analysis of the changes in RV contractility depending on the underlying pathology revealed the greatest changes in the contractile function in the mitral insufficiency group. In the mitral stenosis group, the greatest difference was observed in the tricuspid annular systolic excursion (TAPSE) (p=0.027). In the groups with aortic defects, all parameters of RV contractile function, except for the fractional area change (FAC), showed statistically significant decreases after correction of the underlying defect (p<0.05).Conclusions    Surgical intervention for left heart valvulopathy can result in a decrease in RV function unrelated with systolic deficit of the left ventricle. Modern technologies allow multi-vector assessment of the RV contractile function. To assess the RV function, it is advisable to use a combination of parameters that reflect both global and longitudinal function.

Sleep-Related Hypoxia, Right Ventricular Dysfunction, and Survival in Patients With Group 1 Pulmonary Arterial Hypertension.

BACKGROUND: Group 1 pulmonary arterial hypertension (PAH) is a progressive fatal condition characterized by right ventricular (RV) failure with worse outcomes in connective tissue disease (CTD). Obstructive sleep apnea and sleep-related hypoxia may contribute to RV dysfunction, though the relationship remains unclear. OBJECTIVES: The aim of this study was to prospectively evaluate the association of the apnea-hypopnea index (AHI) and sleep-related hypoxia with RV function and survival. METHODS: Pulmonary Vascular Disease Phenomics (National Heart, Lung, and Blood Institute) cohort participants (patients with group 1 PAH, comparators, and healthy control participants) with sleep studies were included. Multimodal RV functional measures were examined in association with AHI and percentage of recording time with oxygen saturation <90% (T90) per 10-unit increment. Linear models, adjusted for demographics, oxygen, diffusing capacity of the lungs for carbon monoxide, pulmonary hypertension medications, assessed AHI and T90, and RV measures. Log-rank test/Cox proportional hazards models adjusted for demographics, oxygen, and positive airway pressure were constructed for transplantation-free survival analyses. RESULTS: Analysis included 186 participants with group 1 PAH with a mean age of 52.6 ± 14.1 years; 71.5% were women, 80.8% were Caucasian, and there were 43 events (transplantation or death). AHI and T90 were associated with decreased RV ejection fraction (on magnetic resonance imaging), by 2.18% (-2.18; 95% CI: -4.00 to -0.36; P = 0.019) and 0.93% (-0.93; 95% CI: -1.47 to -0.40; P < 0.001), respectively. T90 was associated with increased RV systolic pressure (on echocardiography), by 2.52 mm Hg (2.52; 95% CI: 1.61 to 3.43; P < 0.001); increased mean pulmonary artery pressure (on right heart catheterization), by 0.27 mm Hg (0.27; 95% CI: 0.05 to 0.49; P = 0.019); and RV hypertrophy (on electrocardiography), 1.24 mm (1.24; 95% CI: 1.10 to 1.40; P < 0.001). T90, but not AHI, was associated with a 17% increased 5-year risk for transplantation or death (HR: 1.17; 95% CI: 1.07 to 1.28). In non-CTD-associated PAH, T90 was associated with a 21% increased risk for transplantation or death (HR: 1.21; 95% CI: 1.08 to 1.34). In CTD-associated PAH, T90 was associated with RV dysfunction, but not death or transplantation. CONCLUSIONS: Sleep-related hypoxia was more strongly associated than AHI with measures of RV dysfunction, death, or transplantation overall and in group 1 non-CTD-associated PAH but only with RV dysfunction in CTD-associated PAH. (Pulmonary Vascular Disease Phenomics Program [PVDOMICS]; NCT02980887).

Multiparametric assessment of right ventricular function in heart transplant recipients by echocardiography and relations with pulmonary hemodynamics.

OBJECTIVE: Right ventricular (RV) dilatation and dysfunction are usually present in heart transplant (HTx) patients and worsened with residual pulmonary hypertension (PH). We aimed to determine the ability of different echocardiographic modalities to evaluate RV function in comparison with cardiac magnetic resonance (CMR) and their relations with pulmonary hemodynamics in HTx patients. METHODS: A total of 62 data sets [echocardiographic, hemodynamic, and CMR] were acquired from 35 HTx patients. Comprehensive echocardiography, including two-dimensional (2D) transthoracic echocardiography, speckle tracking echocardiography, and three-dimensional (3D) echocardiography, was performed. Mean pulmonary artery pressure (mPAP) was obtained invasively from right heart catheterization. The correlations between all echocardiographic parameters and CMR imaging data and the differences between patients with and without residual PH were evaluated. RESULTS: Diastolic and systolic RV volumes and RV ejection fraction (RVEF) by 3D echocardiography correlated strongly with CMR-derived volumes and RVEF (r = .91, r = .79, r = .64; p < .0001 for each, respectively). Among other parameters, RV fractional area change (r = .439; p < .001) and RV free wall longitudinal strain (RVFW-LS) (r = -.34; p < .05) correlated moderately with CMR-RVEF, whereas tricuspid annulus S' velocity (r = .29; p < .05) and tricuspid annular systolic plane excursion (r = .27; p < .05) correlated weakly with CMR-RVEF. Additionally, 3D-RVEF and RVFW-LS were significantly decreased in studies with mPAP ≥ 20 mm Hg in comparison to those with mPAP < 20 mm Hg (47.7 ± 3.7 vs. 50.9 ± 5.3, p = .04 and -15.5 ± 3.1 vs. -17.5 ± 3, p = .03, respectively). CONCLUSION: The best method for the evaluation of RV function in HTx recipients is 3D echocardiography. Besides, the subclinical impact of residual PH on RV function can be best determined by RVFW-LS and 3D-RVEF in these patients.

Bio-Humoral and Non-Invasive Haemodynamic Correlates of Renal Venous Flow Patterns across the Heart Failure Spectrum.

Background: We evaluated the bio-humoral and non-invasive haemodynamic correlates of renal congestion evaluated by Doppler renal venous flow (RVF) across the heart failure (HF) spectrum, from asymptomatic subjects with cardiovascular risk factors (Stage A) and structural heart disease (Stage B) to patients with clinically overt HF (Stage C). Methods: Ultrasound evaluation, including echocardiography, lung ultrasound and RVF, along with blood and urine sampling, was performed in 304 patients. Results: Continuous RVF was observed in 230 patients (76%), while discontinuous RVF (dRVF) was observed in 74 (24%): 39 patients had pulsatile RVF, 18 had biphasic RVF and 17 had monophasic RVF. Stage C HF was significantly more common among patients with dRVF. Monophasic RVF was associated with worse renal function and a higher urinary albumin-to-creatinine ratio (uACR). After adjusting for hypertension, diabetes mellitus, the presence of Stage C HF and serum creatinine levels, worsening RVF patterns were associated with higher NT-proBNP levels, worse right ventricular-arterial coupling, larger inferior vena cava and higher echo-derived pulmonary artery wedge pressure. This trend was confirmed when only patients with HF Stage C were analysed after adjusting for the left ventricle ejection fraction (LVEF). Conclusion: Abnormal RVF is common across the HF spectrum. Worsening RVF patterns are independently associated with increased congestion, worse non-invasive haemodynamics and impaired RV-arterial coupling. RVF evaluation could refine prognostic stratification across the HF spectrum, irrespective of LVEF.

[Management of postoperative arrhythmias in the tetralogy of Fallot: a literature review]. / Gestione delle aritmie cardiache dopo correzione chirurgica della tetralogia di Fallot: revisione della letteratura.

Tetralogy of Fallot (ToF) occurs in about 4 births/1000/year and represents about one tenth of all congenital heart diseases. Nowadays 86% of patients reach adulthood with corrective surgery. Before the 1980s, these patients were treated only with "surgical palliation", which consisted in the creation of a systemic to pulmonary artery shunt or a pulmonary valvulotomy, whereas after the introduction of extracorporeal circulation, corrective surgery is performed electively between 3 and 6 months of life. After repair patients during their life may develop hemodynamic lesions, including right ventricular outflow tract dysfunction, and arrhythmias which can occur in over 30% of cases. It is estimated that these patients present a risk of sudden death of 0.2%/year. Therefore, for the prevention and treatment of arrhythmic events, a periodic follow-up in specialized centres for adult congenital heart disease is mandatory, because most often arrhythmias are triggered by the presence of hemodynamic lesions, first of all pulmonary regurgitation.