Left Ventricular Eccentricity Index Research Articles

Ventricular interaction and clinical outcomes in patients undergoing transcatheter tricuspid valve repair

Abstract Background Excessive volume overload of the right ventricle (RV) due to tricuspid regurgitation (TR) can cause a leftward shift of the interventricular septum and changes left ventricular (LV) geometry, which leads to decreases LV distensibility and results in cardiac output. This unfavorable ventricular interaction might have prognostic impacts of patients undergoing transcatheter tricuspid valve repair (TTVr). Purpose We investigated the association between the ventricular interaction and clinical outcomes in patients undergoing TTVr. Methods We retrospectively assessed 304 patients who underwent TTVr. The ventricular interaction was assessed using LV eccentricity index which was defined as LV antero-posterior dimension / LV septo-lateral dimension at end-diastole in the parasternal short-axis view (Figure 1). Increased ventricular interaction was defined as LV eccentricity index ≥1.2. The primary outcome was a composite of all-cause mortality and heart failure hospitalization within one year after TTVr. Results The mean LV eccentricity index at baseline was 1.23 ± 0.20, and 168 of 304 patients (55.3%) had increased ventricular interaction. Patients with increased ventricular interaction were more likely to have massive or torrential TR, reduced RV function, larger RV areas, and lower cardiac index. Increased ventricular interaction at baseline was associated with an increased risk of the composite outcome (HR 2.14; 95%CI 1.36–3.37; p=0.001; Figure 2A), which was consistent after adjusting for age, male sex, renal function, LV ejection fraction, RV function, and residual TR. Echocardiography at baseline showed that 83 of 168 patients (49.4%) with increased ventricular interaction at baseline had an improvement in the ventricular interaction (i.e., LV eccentricity index ≥1.2 at baseline but <1.2 at discharge), which was associated with a lower risk of the composite outcome than persisted ventricular interaction (i.e., LV eccentricity index ≥1.2 at both baseline and discharge) (Figure 2B), irrespectively of baseline characteristics and residual TR. Smaller RV area and residual TR ≤2+ were related to the improved ventricular interaction. Conclusion Ventricular interaction, defined as LV eccentricity index ≥1.2, is associated with an increased risk of clinical outcomes after TTVr. However, the post-procedural improvement in ventricular interaction after TTVr may be linked to better prognosis.Figure 1Figure 2

CMR predictors of acute right heart failure after LVAD implantation

Abstract Background Acute right heart failure (RHF) is one of the most dangerous complications after placement of left ventricular assisted device (LVAD), having a significant impact on prognosis, and is estimated to be around 3 to 35%. Even though right ventricular evaluation with hemodynamical and echocardiographic measurements is a key aspect prior to LVAD implantation, prediction of RHF remains a challenge in daily clinical practice. Purpose To identify cardiac magnetic resonance (CMR) predictors of RHF after LVAD placement. Methods We retrospectively enrolled patients referred to our centre for implantation who also underwent CMR scan prior to LVAD implantation. Acute RHF was defined according to Mechanical Circulatory Support Academy Research Consortium (MRC ARC) criteria. Baseline characteristics, preoperative laboratory, echocardiographic, right heart catheterization (RHC) and CMR parameters were obtained. Patients were divided into two groups based on the development of acute RHF. The variables showing significant differences between the two groups were analysed with the receiver operating characteristic (ROC) curves to find the best cut-offs and then combined into a risk score. Results We enrolled 54 patients who underwent continuous-centrifugal flow LVAD implantation from 2013 to 2021 in whom a CMR within median time of 30 days pre-implantation was available. Four patients were excluded due to low quality images. Among the final cohort of 50 patients, 15 (28%) developed acute RHF. Patients who developed RHF showed worse INTERMACS class, worse renal and liver function, higher central venous pressure at RHC, worse TAPSE at echocardiography and overall smaller left ventricles at the CMR analysis when compared to those who had not acute RHF. No CMR derived right ventricle parameters nor moderate to severe tricuspid regurgitation at echocardiography were different among the two groups. We found that CMR-derived LV mass indexed < 65 g/m2, left ventricular end diastolic diameter (LVEDD) < 70 mm, and LV eccentricity index >1,12 were independent predictors of acute RHF. A CMR-derived score with these three parameters was built (1 point for each). This CMR score has shown to be an independent predictor of acute RHF at multivariate analysis with an odds ratio of 7,084 (CI 95%1.614-37.734) when corrected for other clinical and instrumental predictors of acute RHF. Conclusions Our study proposes a simple scoring system based on three CMR- derived parameters (LVEDD, LV mass indexed and LV eccentricity index) to screen for acute RHF prior LVAD implantation. This score shows promise to be an independent predictor of acute RHF, even when adjusted for clinical, echocardiographic, and haemodynamic parameters. Our model is built on CMR measurement of the LV, showing how patients with smaller ventricles and a left displacement of the interventricular septum (expressed with a higher LV eccentricity index) are at higher risk of developing acute RHF.ROC curves

Ventricular Dysfunction in Patients With Congenital Diaphragmatic Hernia Who Die After Repair

BackgroundQuantitative echocardiographic (echo) measures of ventricular function predict mortality in pediatric pulmonary hypertension (PH), but studies in congenital diaphragmatic hernia (CDH)-related PH are limited. Few studies report quantitative echo data beyond the first week of life in CDH non-survivors. MethodsA single-center retrospective, cross-sectional, cohort study included CDH patients born between January 2013 and April 2022 who survived to surgical repair but died during the neonatal hospitalization. Quantitative measures of right (RV) and left ventricular (LV) size and function including tricuspid annular plane systolic excursion Z-score (TAPSEZ), RV fractional area change (FAC), RV/LV ratio, LV eccentricity index, LV M-mode dimensions, and RV/LV systolic strain were performed offline on the last echocardiogram before death. Data were compared between patients who died ≤30 days after repair (“early”) vs. >30 days after repair (“late”) using the Wilcoxon rank sum test. ResultsTwenty-five (11 early, 14 late) deceased patients had echo images available for analysis. LV size by end-diastolic dimension Z-score was smaller in patients who died early vs. late after repair [-3.03 (−3.93, −2.51) vs. −0.24 (−2.11, 0.53), p = 0.021]. There were trends toward worse RV function (TAPSEZ, RVFAC, RV global and free wall strain) and LV function (apical 4 chamber strain) in patients who died early vs. late after repair. ConclusionThese preliminary findings support future study of the impact of ventricular hypoplasia and dysfunction on mortality and opportunities for risk stratification based on quantitative echo findings in CDH. Level of EvidenceCohort study, 4.

Dysphagia, dyspnoea, dizziness, and ‘inverted D’: Abnormal LV eccentricity secondary to extrinsic compression by oesophageal achalasia

AbstractExtrinsic compression of the left atrium (LA) due to oesophageal achalasia though uncommon has been reported in literature. This case report describes a middle‐aged man who presented with sudden onset dyspnoea and atrial fibrillation, but was later diagnosed to have achalasia cardia and megaoesophagus, resulting in extrinsic compression of the LA as well as posterobasal wall of the left ventricle (LV). The patient's early complaints of dysphagia and weight loss prompted a thorough study, with echocardiography helping to identify extrinsic compression of heart chambers caused by the dilated oesophagus. The left ventricular eccentricity index was increased secondary to compression by volume‐loaded megaoesophagus, which was one of the most notable findings. Through this case, we describe probably the first case of LV compression by achalasia cardia, in addition to LA and we want to highlight the fact that D‐shaped LV can also occur secondary to extrinsic etiologies particularly compression from posterior mediastinal structures.

Refractory cor pulmonale under extracorporeal membrane oxygenation for acute respiratory distress syndrome: the role of conversion to veno-pulmonary arterial assist-a case series.

Pulmonary vascular dysfunction during severe acute respiratory distress syndrome (ARDS) may lead to right ventricle (RV) dysfunction and acute cor pulmonale (ACP). The occurrence/persistence of ACP despite conventional extracorporeal membrane oxygenation (ECMO) is a challenging situation. We explored the usefulness of a specific dual-lumen cannula that bypasses the RV, and on which a veno-pulmonary arterial assist (V-P ECMO) was mounted, in ARDS patients. We report a case-series of ARDS patients put on conventional veno-arterial or veno-venous ECMO and presented refractory ACP as an indication for a reconfiguration to V-P ECMO using the ProtekDuo cannula. The primary endpoint was the mitigation of RV and pulmonary vascular dysfunction as assessed by the change in end-diastolic RV/left ventricle (LV) surface ratio. Six patients had their conventional ECMO reconfigured to V-P ECMO to treat refractory ACP. There was a decrease in end-diastolic RV/LV surface ratio, as well as end-systolic LV eccentricity index, and lactatemia immediately after V-P ECMO initiation. The resolution of refractory ACP was immediately achieved in four of our six (66%) patients. The V-P ECMO was weaned after a median of 26 [8-93] days after implantation. All but one patient were discharged home. We detected one case of severe hemolysis with V-P ECMO and two suspected cases of right-sided infective endocarditis. V-P ECMO is useful to mitigate RV overload and to improve hemodynamics in case of refractory ACP despite conventional ECMO.

Serial tissue Doppler imaging in the evaluation of bronchopulmonary dysplasia-associated pulmonary hypertension among extremely preterm infants: a prospective observational study.

To evaluate serial tissue Doppler cardiac imaging (TDI) in the evolution of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) among extremely preterm infants. Prospective observational study. Single-center, tertiary-level neonatal intensive care unit. Infant born <28 weeks gestation. Utility of TDI in the early diagnosis and prediction of BPD-PH and optimal timing for screening of BPD-PH. A total of 79 infants were included. Of them, 17 (23%) had BPD-PH. The mean gestational age was 25.9 ± 1.1 weeks, and mean birth weight was 830 ± 174 g. The BPD-PH group had a high incidence of hemodynamically significant patent ductus arteriosus (83% vs. 56%, p < 0.018), longer oxygen days (96.16 ± 68.09 vs. 59.35 ± 52.1, p < 0.008), and prolonged hospital stay (133.8 ± 45.9 vs. 106.5 ± 37.9 days, p < 0.005). The left ventricular eccentricity index (0.99 ± 0.1 vs. 1.1 ± 0.7, p < 0.01) and the ratio of acceleration time to right ventricular ejection time showed a statistically significant trend from 33 weeks (0.24 ± 0.05 vs. 0.28 ± 0.05, p < 0.05). At 33 weeks, the BPD-PH group showed prolonged isovolumetric contraction time (27.84 ± 5.5 vs. 22.77 ± 4, p < 0.001), prolonged isovolumetric relaxation time (40.3 ± 7.1 vs. 34.9 ± 5.3, p < 0.003), and abnormal myocardial performance index (0.39 ± 0.05 vs. 0.32 ± 0.03, p < 0.001). These differences persisted at 36 weeks after conceptional gestational age. TDI parameters are sensitive in the early evolution of BPD-PH. Diagnostic accuracy can be increased by combining the TDI parameters with conventional echocardiographic parameters. BPD-PH can be recognizable as early as 33-34 weeks of gestation.

Acute changes in cardiac dimensions, function, and longitudinal mechanics in healthy individuals with and without high-altitude induced pulmonary hypertension at 4559m.

High-altitude pulmonary hypertension (HAPH) has a prevalence of approximately 10%. Changes in cardiac morphology and function at high altitude, compared to a population that does not develop HAPH are scarce. Four hundred twenty-one subjects were screened in a hypoxic chamber inspiring a FiO2 =12% for 2h. In 33 subjects an exaggerated increase in systolic pulmonary artery pressure (sPAP) could be confirmed in two independent measurements. Twenty nine of these, and further 24 matched subjects without sPAP increase were examined at 4559m by Doppler echocardiography including global longitudinal strain (GLS). SPAP increase was higher in HAPH subjects (∆=10.2vs. ∆=32.0mm Hg, p<.001). LV eccentricity index (∆=.15vs. ∆=.31, p=.009) increased more in HAPH. D-shaped LV (0 [0%] vs. 30 [93.8%], p=.00001) could be observed only in the HAPH group, and only in those with a sPAP ≥50mm Hg. LV-EF (∆=4.5vs. ∆=6.7%, p=.24) increased in both groups. LV-GLS (∆=1.2vs. ∆=1.1 -%, p=.60) increased slightly. RV end-diastolic (∆=2.20vs. ∆=2.7cm2 , p=.36) and end-systolic area (∆=2.1vs. ∆=2.7cm2 , p=.39), as well as RA end-systolic area index (∆=-.9vs. ∆=.3cm2 /m2 , p=.01) increased, RV-FAC (∆=-2.9vs. ∆=-4.7%, p=.43) decreased, this was more pronounced in HAPH, RV-GLS (∆=1.6vs. ∆=-.7 -%, p=.17) showed marginal changes. LV and LA dimensions decrease and left ventricular function increases at high-altitude in subjects with and without HAPH. RV and RA dimensions increase, and RV longitudinal strain increases or remains unchanged in subjects with HAPH. Changes are negligible in those without HAPH.

The effect of bariatric surgery type on cardiac reverse remodelling.

Bariatric surgery is effective in reversing adverse cardiac remodelling in obesity. However, it is unclear whether the three commonly performed operations; Roux-en-Y Gastric Bypass (RYGB), Laparoscopic Sleeve Gastrectomy (LSG) and Laparoscopic Adjustable Gastric Band (LAGB) are equal in their ability to reverse remodelling. Fifty-eight patients underwent CMR to assess left ventricular mass (LVM), LV mass:volume ratio (LVMVR) and LV eccentricity index (LVei) before and after bariatric surgery (26 RYGB, 22 LSG and 10 LAGB), including 46 with short-term (median 251-273 days) and 43 with longer-term (median 983-1027 days) follow-up. Abdominal visceral adipose tissue (VAT) and epicardial adipose tissue (EAT) were also assessed. All three procedures resulted in significant decreases in excess body weight (48-70%). Percentage change in VAT and EAT was significantly greater following RYGB and LSG compared to LAGB at both timepoints (VAT:RYGB -47% and -57%, LSG -47% and -54%, LAGB -31% and -25%; EAT:RYGB -13% and -14%, LSG -16% and -19%, LAGB -5% and -5%). Patients undergoing LAGB, whilst having reduced LVM (-1% and -4%), had a smaller decrease at both short (RYGB: -8%, p < 0.005; LSG: -11%, p < 0.0001) and long (RYGB: -12%, p = 0.009; LSG: -13%, p < 0.0001) term timepoints. There was a significant decrease in LVMVR at the long-term timepoint following both RYGB (-7%, p = 0.006) and LSG (-7%, p = 0.021), but not LAGB (-2%, p = 0.912). LVei appeared to decrease at the long-term timepoint in those undergoing RYGB (-3%, p = 0.063) and LSG (-4%, p = 0.015), but not in those undergoing LAGB (1%, p = 0.857). In all patients, the change in LVM correlated with change in VAT (r = 0.338, p = 0.0134), while the change in LVei correlated with change in EAT (r = 0.437, p = 0.001). RYGB and LSG appear to result in greater decreases in visceral adiposity, and greater reverse LV remodelling with larger reductions in LVM, concentric remodelling and pericardial restraint than LAGB.

Left ventricular eccentricity index to assess precapillary pulmonary hypertension in dogs

IntroductionInterventricular septal flattening, frequently present in pulmonary hypertension (PH) can be quantified by the left ventricular eccentricity index (EI) measured at end-diastole (EId), end-systole (EIs) and at maximal septal flattening (EIm). In humans, EI correlates with invasive pulmonary arterial pressure.The aim of this study was to evaluate if EI correlates with parameters of right heart remodeling (RHR) and if EI is a quantitative marker of PH in dogs. Materials and methodsLeft ventricular eccentricity indices were retrospectively measured in four groups (no, mild, moderate and severe PH) with interpretable tricuspid and/or pulmonary regurgitation. ResultsNinety-seven dogs were included, with no (n = 29), mild (n = 13), moderate (n = 25) and severe (n = 30) PH. The intra- and inter-observer variability for EI measurements ranged from 2 % to 11 %. All EI were significantly elevated in severe compared to no, mild and moderate PH (P < 0.0005). In the moderate group, EIs and EIm were higher compared to the no PH group (P < 0.01). Tricuspid and pulmonary regurgitation pressure gradients and RHR parameters correlated with EId, EIs and EIm in all groups. Optimal cut-off values discriminating moderate and severe PH from no and mild PH were 1.24 (Sensitivity (Se) 60 %; Specificity (Sp) 90 %) for EId, 1.34 (Se 67 %; Sp 95 %) for EIs and 1.37 (Se 76 %; Sp 83 %) for EIm. ConclusionsLeft ventricular eccentricity indices are reproducible echocardiographic variables increasing with severity of PH. Dogs with moderate and severe PH can be discriminated from dogs with no or mild PH using EIs and EIm.

Prevalence of pulmonary hypertension in obstructive sleep apnea and its relation to disease severity.

The purpose of this study is to examine the prevalence of pulmonary hypertension (PHTN) in children with obstructive sleep apnea (OSA) using echocardiographic (ECHO) parameters and to examine ECHO findings as they relate to severity of OSA. A retrospective cohort study of patients with OSA undergoing polysomnogram and ECHO within 30 days of each other, between January 1, 2015, and December 31, 2020, was performed, excluding cardiac disease. ECHO evidence of PHTN was defined as ≥ 2 of the following: tricuspid regurgitation velocity > 3.0 m/s, pulmonary acceleration/ejection time ratio < 0.3, left ventricular eccentricity index > 1.5, and right ventricular dysfunction or abnormal geometry. ECHO parameters were compared to OSA severity using obstructive apnea-hypopnea index, percent of time with oxygen saturation < 90%, and percent of time with end-tidal carbon dioxide > 50 mmHg. Odds ratios were calculated for each comorbidity to evaluate for risk factors. Of 509 patients, 4.3% were found to have echocardiographic evidence of PHTN. Neither obstructive apnea-hypopnea index severity nor worsening hypoxemia or hypercarbia correlated with worsening ECHO parameters. Comorbidities including bronchopulmonary dysplasia (odds ratio, 5.22, 2.01-13.53), prematurity (odds ratio, 3.10, 1.28-7.47), and autism (odds ratio, 3.69, 1.01-13.49) were associated with increased odds of PHTN. Significant echocardiographic evidence of PHTN was seen in 4.3% of children with OSA. ECHO findings of PHTN did not correlate with polysomnogram parameters. Comorbidities, particularly bronchopulmonary dysplasia, prematurity, and autism, appear to be a risk factor for developing PHTN in patients with OSA. Milligan I, Shaw AK, Leu R, Kanaan U, Michelfelder E. Prevalence of pulmonary hypertension in obstructive sleep apnea and its relation to disease severity. J Clin Sleep Med. 2024;20(5):675-680.

Assessment of Myocardial Function in Infants of Mothers with Gestational Diabetes Mellitus Using Deformation Imaging over the First Year of Age

To assess serial myocardial performance and pulmonary vascular resistance (PVR) in infants of mothers with gestational diabetes mellitus (GDM) over the first year of life. This was a prospective, observational study. Echocardiography was performed at birth, 6months, and 1year of age. Pulmonary artery acceleration time and left ventricular (LV) eccentricity index provided surrogate measurements of PVR. Biventricular function was assessed by tissue Doppler imaging and deformation analysis. Fifty infants of mothers with GDM were compared with 50 controls with no difference in gestation (38.9±0.8 weeks vs 39.3±0.9weeks; P=.05) or birthweight (3.55±0.49 kg vs 3.56±0.41kg; P=.95). At 1 year of age, the pulmonary artery acceleration time was lower (70±11 vs 79±10; P=.01) in the GDM group. LV global longitudinal strain (24.7±1.9 vs 28.8±1.8 %; P<.01), LV systolic strain rate (1.8±0.2 vs 2.1±0.3 1/s; P<.01), and RV free wall strain (31.1±4.8 vs 34.6±3.9 %; P<.01) were lower in the GDM cohort at 1year of age (all P values adjusted for gestation, mode of delivery, and maternal body mass index). Our findings demonstrate higher indices of PVR and lower biventricular function in infants ofmothers with GDM compared with controls at each time point assessed in this study over the first year of life.

Quantitative Measures of Right Ventricular Size and Function by Echocardiogram Correlate with Cardiac Catheterization Hemodynamics in Congenital Diaphragmatic Hernia

To evaluate associations between cardiac catheterization (cath) hemodynamics, quantitative measures of right ventricular (RV) function by echocardiogram, and survival in patients with congenital diaphragmatic hernia (CDH). This single-center retrospective cohort study enrolled patients with CDH who underwent index cath from 2003 to 2022. Tricuspid annular plane systolic excursion z score, RV fractional area change, RV free wall and global longitudinal strain, left ventricular (LV) eccentricity index, RV/LV ratio, and pulmonary artery acceleration time were measured from preprocedure echocardiograms. Associations between hemodynamic values, echocardiographic measures, and survival were evaluated by Spearman correlation and Wilcoxon rank sum test, respectively. Fifty-three patients (68% left-sided, 74% liver herniation, 57% extracorporeal membrane oxygenation, 93% survival) underwent cath (39 during index hospitalization, 14 later) including device closure of a patent ductus arteriosus in 5. Most patients (n=31, 58%) were on pulmonary hypertension treatment at cath, most commonly sildenafil (n=24, 45%) and/or intravenous treprostinil (n=16, 30%). Overall, hemodynamics were consistent with precapillary pulmonary hypertension. Pulmonary capillary wedge pressure was >15mm Hg in 2 patients (4%). Lower fractional area change and worse ventricular strain were associated with higher pulmonary artery pressure while higher LV eccentricity index and higher RV/LV ratio were associated with both higher pulmonary artery pressure and higher pulmonary vascular resistance. Hemodynamics did not differ based on survival status. Worse RV dilation and dysfunction by echocardiogram correlate with higher pulmonary artery pressure and pulmonary vascular resistance on cath in this CDH cohort. These measures may represent novel, noninvasive clinical trial targets in this population.

Cardiac Magnetic Resonance Derived Left Ventricular Eccentricity Index and Right Ventricular Mass Measurements Predict Outcome in Children with Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is associated with increased right ventricular (RV) afterload, affecting RV remodeling and RV performance, a major determinant of outcome in PAH-patients. In children with PAH, treatment strategy is guided by risk stratification where noninvasive prognosticators are highly needed. The prognostic value of RV characteristics derived by cardiac magnetic resonance (CMR) has been scarcely studied in pediatric PAH. We aimed to identify CMR-derived morphometric and functional RV characteristics prognostic for outcome in children with PAH. From the Dutch National cohort, thirty-eight children with either idiopathic/heritable PAH (IPAH/HPAH) or PAH associated with congenital heart disease (PAH-CHD), who underwent CMR, were included (median (interquartile range) [IQR] age 13.0 years (10.8-15.0), 66% females). Patients had severe PAH, characterized by their World Health Organization Functional Class, increased N-terminal pro-B-type natriuretic peptide and high pulmonary arterial pressure and pulmonary vascular resistance index at time of CMR. RV-ejection fraction (RVEF), indexed RV-mass (RVMi), the ratio between RV and LV mass (RVM/LVM-ratio) and left ventricular eccentricity index (LVEI) all correlated with transplant-free survival from time of CMR. These correlations could not be confirmed in the PAH-CHD group. This study shows that CMR-derived measures reflecting RV function and remodeling (LVEI, RVMi, RVM/LVM-ratio, RVEF) predict transplant-free survival in children with IPAH/HPAH and may be included in risk stratification scores in pediatric PAH.

Treprostinil in Neonates with Congenital Diaphragmatic Hernia-Related Pulmonary Hypertension

To describe our experience with treprostinil, evaluate correlations with cardiac function, and assess for adverse effects in neonates with congenital diaphragmatic hernia-related pulmonary hypertension (CDH-PH). A retrospective review of a single-center prospective registry at a quaternary care children's hospital. Patients included in the study had CDH-PH treated with treprostinil between April 2013 and September 2021. Assessed outcomes were brain-type natriuretic peptide levels and quantitative echocardiographic parameters collected at baseline, 1week, 2weeks, and 1month after treprostinil initiation. Right ventricular (RV) function was assessed by tricuspid annular plane systolic excursion Z-score and speckle tracking echocardiography (global longitudinal and free wall strain). Septal position and left ventricular (LV) compression were assessed by eccentricity index and M-mode Z-scores. Fifty-one patients were included, with an average expected/observed lung-to-head ratio of 28.4±9.0%. Most patients required extra-corporeal membrane oxygenation (n=45, 88%). Survival to hospital discharge was 31/49 (63%). Treprostinil was initiated at a median age of 19days with a median effective dose of 34ng/kg/minute. Median baseline brain-type natriuretic peptide level decreased from 416.9 pg/mL to 120.5 pg/mL after 1month. Treprostinil was associated with improved tricuspid annular plane systolic excursion Z-score, RV global longitudinal strain, RV free wall strain, LV eccentricity index, and LV diastolic and systolic dimensions, reflecting less compression by the RV, regardless of ultimate patient survival. No serious adverse effects were recorded. In neonates with CDH-PH, treprostinil administration is well tolerated and is associated with improved RV size and function.

Prognostic Value of Transthoracic Echocardiography in Children With Pulmonary Arterial Hypertension.

Background Transthoracic echocardiography is part of the regular follow-up protocol at most pediatric pulmonary arterial hypertension (PAH) centers. We aimed to develop a comprehensive and simple echocardiographic risk stratification for children with PAH. Methods and Results We included 63 children with PAH and a biventricular cardiac anatomy without relevant shunt lesions (60% female patients; mean age, 9.0years; 42 idiopathic PAH and 21 associated PAH) undergoing a standardized transthoracic echocardiographic assessment. The prognostic value of echocardiographic parameters was assessed using Cox proportional hazards survival analysis and recursive partitioning for classification tree methods. Over a median follow-up period of 4.0years, 17 patients died and 4 underwent lung transplantation. Various echocardiographic parameters were associated with the combined endpoint of death and transplantation on univariate analysis. On further analysis, right atrial area (z score) and left ventricular diastolic eccentricity index (LVEId) emerged as robust and independent predictors of transplant-free survival. Considering mortality alone as an end point, a combination of right atrial area, left ventricular diastolic eccentricity index, and tricuspid annular plane systolic excursion were identified as independent predictors of outcome. Based on these parameters, we propose simple risk scores that can be applied at the bedside without computer assistance. CONCLUSIONS Echocardiographic parameters predict prognosis in children with pulmonary hypertension. A combination of widely available parameters including right atrial area, left ventricular eccentricity index, and tricuspid annular plane systolic excursion emerged as risk stratifiers that await external validation but may assist clinicians determining the prognosis of children with PAH.

Changes in epicardial and visceral adipose tissue depots following bariatric surgery and their effect on cardiac geometry.

Obesity affects cardiac geometry, causing both eccentric (due to increased cardiac output) and concentric (due to insulin resistance) remodelling. Following bariatric surgery, reversal of both processes should occur. Furthermore, epicardial adipose tissue loss following bariatric surgery may reduce pericardial restraint, allowing further chamber expansion. We investigated these changes in a serial imaging study of adipose depots and cardiac geometry following bariatric surgery. 62 patients underwent cardiac magnetic resonance (CMR) before and after bariatric surgery, including 36 with short-term (median 212 days), 37 medium-term (median 428 days) and 32 long-term (median 1030 days) follow-up. CMR was used to assess cardiac geometry (left atrial volume (LAV) and left ventricular end-diastolic volume (LVEDV)), LV mass (LVM) and LV eccentricity index (LVei - a marker of pericardial restraint). Abdominal visceral (VAT) and epicardial (EAT) adipose tissue were also measured. Patients on average had lost 21kg (38.9% excess weight loss, EWL) at 212 days and 36kg (64.7% EWL) at 1030 days following bariatric surgery. Most VAT and EAT loss (43% and 14%, p<0.0001) occurred within the first 212 days, with non-significant reductions thereafter. In the short-term LVM (7.4%), LVEDV (8.6%) and LAV (13%) all decreased (all p<0.0001), with change in cardiac output correlated with LVEDV (r=0.35,p=0.03) and LAV change (r=0.37,p=0.03). Whereas LVM continued to decrease with time (12% decrease relative to baseline at 1030 days, p<0.0001), both LAV and LVEDV had returned to baseline by 1030 days. LV mass:volume ratio (a marker of concentric hypertrophy) reached its nadir at the longest timepoint (p<0.001). At baseline, LVei correlated with baseline EAT (r=0.37,p=0.0040), and decreased significantly from 1.09 at baseline to a low of 1.04 at 428 days (p<0.0001). Furthermore, change in EAT following bariatric surgery correlated with change in LVei (r=0.43,p=0.0007). Cardiac volumes show a biphasic response to weight loss, initially becoming smaller and then returning to pre-operative sizes by 1030 days. We propose this is due to an initial reversal of eccentric remodelling followed by reversal of concentric remodelling. Furthermore, we provide evidence for a role of EAT contributing to pericardial restraint, with EAT loss improving markers of pericardial restraint.

Pericardial fat is adversely related to cardio-mechanical interaction in heart failure with preserved ejection fraction: implications for exercise intolerance

Abstract Background Exercise intolerance is the primary manifestation in heart failure with preserved ejection fraction (HFpEF); however, the pathophysiologic mechanism(s) remains incompletely understood. Excess cardiac adiposity may physically constrain the myocardium, resulting in adverse Cardio-Mechanical Interaction (i.e., greater left ventricular eccentricity); a phenomenon only expected to worsen during exercise, with increased respiratory excursion and hemodynamic load. Evidence for this hypothesis, however, remains limited to a small number of observations, from a single group, made only under resting conditions using transthoracic echocardiography. Purpose To evaluate the relationship between pericardial fat and cardio-mechanical interaction in HFpEF at rest and during exercise using high resolution cardiac magnetic resonance imaging (cMRI). Methods We performed real-time (ungated), free-breathing cinematic imaging of the left ventricular (LV) short axis in 11 individuals with HFpEF (4M/7F, BMI: 36±6, age: 69±4 years). Imaging was performed at rest and during dynamic leg exercise (30 Watts) using an MRI-compatible ergometer (Ergospect, Austria). Epicardial and paracardial fat area were measured using high resolution cine images in the horizontal long axis imaging plane, with epicardial fat defined as the adipose tissue within the pericardium and paracardial fat defined as the adipose tissue outside of the pericardium (Figure 1A); the sum of which defined pericardial fat area. The LV eccentricity index was calculated as the ratio of LV short axis diameter parallel to the septum (anteroposterior dimension, AP) to the LV short axis diameter perpendicular to the septum (septolateral dimension, SL, Figure 1A) at mid-ventricular level, during inspiration at end-diastole. Results At rest, adverse cardio-mechanical interaction (i.e., AP/SL &amp;gt;1.0) was observed in 5 of the 11 cases. In those with adverse cardio-mechanical interaction both epicardial and paracardial fat area were significantly higher, resulting in greater pericardial fat area (Figure 1B). While epicardial fat area was not related to LV eccentricity index (R2=0.19, P=0.18), we observed a strong correlation between paracardial fat area and LV eccentricity index (R2=0.93, P&amp;lt;0.01), and pericardial fat area and LV eccentricity index (R2=0.86, P&amp;lt;0.01, Figure 1C). In contrast to our hypothesis, however, supine exercise did not exacerbate cardio-mechanical interaction, with LV eccentricity index remaining elevated in 4 of the 5 original cases, improving the fifth case (1.1 to 0.9, rest to exercise). Conclusions Taken together, these data extend prior reports of adverse cardio-mechanical interaction in HFpEF, showing greater contribution from paracardial fat versus epicardial fat. Future studies are needed to examine cardio-mechanical interaction during upright exercise in HFpEF patients. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): National Institutes of Health

A framework of deep learning networks provides expert-level accuracy for the detection and prognostication of pulmonary arterial hypertension

To test the hypothesis that deep learning (DL) networks reliably detect pulmonary arterial hypertension (PAH) and provide prognostic information. Consecutive patients with PAH, right ventricular (RV) dilation (without PAH), and normal controls were included. An ensemble of deep convolutional networks incorporating echocardiographic views and estimated RV systolic pressure (RVSP) was trained to detect (invasively confirmed) PAH. In addition, DL-networks were trained to segment cardiac chambers and extracted geometric information throughout the cardiac cycle. The ability of DL parameters to predict all-cause mortality was assessed using Cox-proportional hazard analyses. Overall, 450 PAH patients, 308 patients with RV dilatation (201 with tetralogy of Fallot and 107 with atrial septal defects) and 67 normal controls were included. The DL algorithm achieved an accuracy and sensitivity of detecting PAH on a per patient basis of 97.6 and 100%, respectively. On univariable analysis, automatically determined right atrial area, RV area, RV fractional area change, RV inflow diameter and left ventricular eccentricity index (P &lt; 0.001 for all) were significantly related to mortality. On multivariable analysis DL-based RV fractional area change (P &lt; 0.001) and right atrial area (P = 0.003) emerged as independent predictors of outcome. Statistically, DL parameters were non-inferior to measures obtained manually by expert echocardiographers in predicting prognosis. The study highlights the utility of DL algorithms in detecting PAH on routine echocardiograms irrespective of RV dilatation. The algorithms outperform conventional echocardiographic evaluation and provide prognostic information at expert-level. Therefore, DL methods may allow for improved screening and optimized management of PAH.

Serial Assessment of Cardiac Function and Pulmonary Hemodynamics in Infants With Down Syndrome

There is a dearth of longitudinal data describing the evolution of cardiopulmonary hemodynamics in infants with Down syndrome (DS) beyond infancy. We hypothesized that babies with DS, independent of the presence of congenital heart disease (CHD), demonstrate biventricular systolic and diastolic impairment and sustained elevation of pulmonary pressures compared with controls over the first 2years of age. This was a prospective observational cohort study of 70 infants with DS (48 with CHD and 22 without CHD) and 60 controls carried out in 3 tertiary neonatal intensive care units in Dublin, Ireland. Infants with DS with and without CHD and non-DS controls underwent serial echocardiograms at birth, 6months, 1year, and 2years of age to assess biventricular systolic and diastolic function using deformation analysis. Pulmonary vascular resistance was assessed using pulmonary artery acceleration time and left ventricular (LV) eccentricity index. Infants with DS exhibited smaller LV (birth: 27±4 vs 31±2mm, P&lt;.01; 2years: 43±5 vs 48±4mm, P&lt;.01) and right ventricular (birth: 28±3 vs 31±2mm, P&lt;.01; 2years: 40±4 vs 44±3mm, P&lt;.01) lengths and lower LV (birth: -19%±3% vs -22%±2%, P&lt;.01; 2years: -24%±2% vs -26%±2%, P&lt;.01) and right ventricular (birth: -19%±4% vs -22%±3%, P&lt;.01; 2years: -29%±6% vs -33%±4%, P&lt;.01) systolic strain over the 2-year period. Pulmonary artery acceleration time was lower in the DS group throughout the study period (birth: 44±10 vs 62±14ms, P&lt;.01; 2years 71±12 vs 83±11ms, P&lt;.01). No differences were observed between DS infants with and without CHD (all P&gt;.05). Infants with DS exhibit impaired maturational changes in myocardial function and pulmonary vascular resistance. Such novel findings provide valuable insights into the pathophysiology affecting cardiorespiratory morbidity in this population.

Exploring the failing right ventricle in pulmonary hypertension by cardiac magnetic resonance: An in vivo study utilizing Macitentan.

Cardiac magnetic resonance (CMR) imaging is used to assess the right ventricle (RV) of pulmonary hypertensive (PH) patients and more recently to track changes in response to therapy. We wished to investigate if repeat CMRs could be used to assess ventricular changes in the Sugen 5416 hypoxic (Su/Hx) rat model of PH treated with the dual endothelin receptor antagonist Macitentan. Male Sprague Dawley Su/Hx rats were dosed for 3 weeks with either vehicle or Macitentan (30 mg/kg) daily, control rats received only vehicle. All rats underwent three CMR scans; before treatment, 2 weeks into treatment, and end of the study. A separate group of Su/Hx and control rats, treated as above, underwent terminal hemodynamic measurements. Using terminal and CMR measurements, Macitentan was found to lower RV systolic pressure pulmonary artery remodeling and increase RV ejection fraction but not change RV hypertrophy (RVH). Repeat CMRs determined that Su/Hx rats treated with Macitentan had significantly reversed RVH via reducing RV mass as well as reducing elevated left ventricular eccentricity index; reductions in RV mass were also observed in Su/Hx vehicle rats exposed to normoxic conditions. We have demonstrated that repeat CMRs can be used to assess the volume and structural changes in the ventricles of the Su/Hx rat model. Using repeat CMRs has allowed us to build a more complete picture of the response of the RV and the left ventricle to treatment. It is unknown if these effects are a consequence of direct action on the RV or secondary to improvements in the lung vasculature.