Right Ventricular Systolic Pressure Research Articles

The Role of Right Ventriculo-Arterial Coupling in Symptoms Presentation of Patients with Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy. The hallmark of HCM is myocardial fibrosis which contributes to heart failure, arrhythmias, and sudden cardiac death (SCD). To identify the factors implicated in heart failure symptoms and functional capacity of patients with HCM. In this cohort study, 43 patients with HCM were recruited. According to functional capacity and symptoms presentation, patients were categorized according to New York Heart Association (NYHA) classification, and echocardiographic measurements of left ventricle systolic and diastolic function were conducted. The echocardiographic assessment of right ventriculo-arterial coupling (RVAC) was made by calculating the tricuspid annular peak systolic tissue Doppler velocity (TASV)/estimated RV systolic pressure (RVSP) ratio. Almost half (51%) of our study population present symptoms of heart failure and were categorized as the symptomatic group-NYHA 2 or higher. Maximum LVOT gradient, RVSP, and the ratio of E/e' were higher in the symptomatic group compared with the asymptomatic group. TASV was lower in the symptomatic group compared with the asymptomatic group (11 ± 1 cm/s vs. 13 ± 2 cm/s, p = 0.04). However, there was no difference in other potentially influential factors, such as heart rate or systemic blood pressure. The SCD risk score does not differ between the two studied groups. The RVAC (estimated with the TASV/RVSP ratio) was lower in the symptomatic group compared with the asymptomatic group (0.32 ± 0.09 vs. 0.46 ± 0.11, p < 0.001). A low RVAC (as estimated with TASV/RVSP ratio) value could represent an echocardiographic marker of right ventricular-arterial uncoupling in patients with HCM and impaired functional status.

Comprehensive echocardiographic assessment of right ventricular function, pulmonary arterial elastic properties and ventricular-vascular coupling in adult patients with repaired tetralogy of fallot: clinical significance of 3D derived indices.

We aimed to comprehensively analyze by three-dimensional speckle-tracking echocardiography (3DSTE) and Doppler echocardiography right ventricular (RV) performance, pulmonary arterial (PA) elastic properties and right ventricular-pulmonary artery coupling (RVPAC) in patients with repaired tetralogy of Fallot (rTOF) and assess the feasibility and clinical utility of related echocardiographic indices. Twenty-four adult patients with rTOF and twenty-four controls were studied. RV end-diastolic volume(3D-RVEDV), RV end-systolic volume(3D-RVESV), RV ejection fraction(3D-RVEF), RV longitudinal strain(3D-RVLS) and RV area strain(3D-RVAS) were calculated by 3DSTE. RV end-systolic area (RVESA) was obtained by planimetry. Pulmonary regurgitation (PR) was assessed as trivial/mild or significant by cardiac magnetic resonance (CMR) and color-Doppler. Pulmonary artery (PA) elastic properties were determined using two-dimensional/Doppler echocardiography. RV systolic pressure (RVSP) was measured using standard Doppler methods. RVPAC was assessed using various 3DSTE-derived parameters (3DRVAS/RVSP, 3DRVLS/RVESA, 3DRVAS/RVESV). Overall, 3DRVEF and 3DRVAS were impaired in rTOF patients compared with controls. PA pulsatility and capacitance were reduced (p = 0.003) and PA elastance was higher (p = 0.0007) compared to controls. PA elastance had a positive correlation with 3DRVEDV (r = 0.64, p = 0.002) and 3DRVAS (r = 0.51, p = 0.02). By ROC (receiver operating characteristics) analysis, 3DRVAS/RVESV, 3DRVAS/RVSP and 3DRVLS/RVESA cutoff values of 0.31%/mmHg, 0.57%/mmHg and 0.86%/mmHg, respectively, had 91%, 88% and 88% sensitivity and 81%, 81% and 79% specificity in identifying exercise capacity impairment. In rTOF patients increased 3DSTE-derived RV volumes and impaired RV ejection fraction and strain are associated with reduced PA pulsatility and capacitance and increased PA elastance. 3DSTE-derived RVPAC parameters using different afterload-markers are accurate indices of exercise capacity.

Effect of Angiotensin Receptor-Neprilysin Inhibitor on Cardiac Remodeling in Heart Failure with Reduced Ejection Fraction in Kuwait

Abstract Background: Heart failure with reduced ejection fraction (HFrEF) is a growing concern in the Middle East and worldwide, despite advances in treatment. The introduction of angiotensin receptor-neprilysin inhibitor (ARNI) has shown promise in managing HFrEF by inhibiting the renin–angiotensin–aldosterone system. However, its effects on cardiac remodeling and outcomes in the Middle East are poorly understood. Objectives: To determine the effectiveness and safety of ARNI in improving outcomes for HFrEF patients in Kuwait. Methods: This observational study, conducted at Al Dabbous Cardiac Center in Kuwait, included 114 adult HFrEF patients treated with ARNI for 6 months. Data on patient characteristics, echocardiographic measurements, and clinical parameters were collected before and after treatment. Statistical analysis was performed using paired t-tests and nonparametric sign tests. Results: Following ARNI treatment, significant improvements were observed in left ventricular ejection fraction (LVEF), left ventricular end-diastolic diameter (LVEDD), right ventricular systolic pressure (RVSP), and New York Heart Association Functional Classification. LVEF increased from 29.54% to 32.22% (P &lt; 0.001). LVEDD and RVSP decreased significantly (P &lt; 0.001, P = 0.016, respectively), while systolic blood pressure decreased (P = 0.002). The study showed no significant changes in weight or potassium levels. Adverse effects were minimal. Conclusions: This study contributes to the growing body of evidence supporting ARNI’s effectiveness in improving outcomes for HFrEF patients, particularly in a Middle Eastern population. ARNI treatment resulted in significant improvements in cardiac remodeling and clinical parameters, emphasizing its potential as a valuable therapeutic option for HFrEF patients. Additional research is essential to optimize ARNI’s use and enhance patient outcomes.

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

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

Association Between Pulmonary Hypertension and Its Effect on 30-Day Mortality, Readmission, and Cost After Transcatheter Aortic Valve Replacement: A Multicenter Study.

Background Pulmonary hypertension (PH) is commonly seen in patients with severe aortic stenosis. Transcatheter aortic valve replacement (TAVR) has been shown to improve PH, however, its impact on clinical outcomes and cost remains unclear. Methods We did a multicenter, retrospective analysis of patients undergoing TAVR in our system between December 2012 to November 2020. The initial sample size was 1356. We excluded patients with prior history of heart failure, with a left ventricular ejection fraction of 40% or less, and patients who had active symptoms of heart failure within two weeks of the procedure. Patients were divided into four groups based on their pulmonary pressures, using right ventricular systolic pressure (RVSP) as a surrogate for PH. Groups included patients with normal pulmonary pressures (<35mmHg), mild (35-45mmHg) moderate (46-60mmHg), and severe PH (>60mmHg). Primary outcomes included 30-day mortality and readmission. Secondary outcomes included length of ICU stay and cost of admission. We used Chi-square and T-tests for demographic analysis of categorical continuous variables respectively. Adjusted regression was used for the reliability of correlation between variables. Multivariate analysis was used for final outcomes. Results Final sample size was 474. Average age was 78.9 years (SD: 8.2, 53% Male). Thirty-one percent (n=150) had normal pulmonary pressures, 33% (n=156) had mild PH, 25% (n=122) had moderate and 10% (n=46) had severe PH. Patients with history of hypertension (p-value<0.001), diabetes (p-value<0.001), chronic lung disease (p-value=0.006) and those on supplemental oxygen (p-value=0.046), had significantly higher proportion of patients with moderate and severe PH. We found significantly higher odds of 30-day mortality in patients with severe PH (OR: 6.77, CI: 1.09-41.98: p-value 0.04) compared with normal or mild PH. There was no significant difference in 30-day readmission (p-value=0.859) between the four groups. Cost did not change with severity of PH (Avg: $261,075: p-value=0.810). Patients with severe PH spent a significantly higher number of hours in ICU, compared with the other three groups (Mean: 18.2, p=value <0.001). Conclusions Severe pulmonary hypertension significantly increased the odds of 30-day mortality and ICU stay in TAVR patients. We did not see any significant difference in 30-day readmission and cost of admission, based on PH severity.

Combined use of right ventricular coupling and pulmonary arterial elastance as a comprehensive stratification approach for right ventricular function.

Right ventricular (RV)-pulmonary arterial uncoupling is the consequence of increased afterload and/or decreased RV contractility. However, the combination of arterial elastance (Ea) and end-systolic elastance (Ees)/Ea ratio to assess RV function is unclear. We hypothesized that the combination of both could comprehensively assess RV function and refine risk stratification. The median Ees/Ea ratio (0.80) and Ea (0.59mmHg/mL) were used to classify 124 advanced heart failure patients into four groups. RV systolic pressure differential was defined as end-systolic pressure (ESP) minus beginning-systolic pressure (BSP). Patients among different subsets showed dissimilar New York Heart Association functional class (V=0.303, P=0.010), distinct tricuspid annular plane systolic excursion/ pulmonary artery systolic pressure (mm/mmHg) (0.65 vs. 0.44 vs. 0.32 vs. 0.26, P<0.001), and diverse prevalence of pulmonary hypertension (33.3% vs. 35% vs. 90% vs. 97.6%, P<0.001). By multivariate analysis, Ees/Ea ratio (hazard ratio [HR] 0.225, P=0.004) and Ea (HR 2.194, P=0.003) were independently associated with event-free survival. Patients with Ees/Ea ratio >0.80 and Ea <0.59mmHg/mL had better outcomes (P<0.05). In patients with Ees/Ea ratio >0.80, those with Ea ≥0.59mmHg/mL had a higher adverse outcome risk (P<0.05). Ees/Ea ratio ≤0.80 was associated with adverse outcomes, even when Ea was <0.59mmHg/mL (P<0.05). Approximately 86% of patients with ESP-BSP >5mmHg had an Ees/Ea ratio ≤0.80 and/or an Ea ≥0.59mmHg/mL (V=0.336, P=0.001). Combined use of Ees/Ea ratio and Ea could be a comprehensive approach to assessing RV function and predicting outcomes. An exploratory analysis demonstrated that Ees/Ea ratio and Ea might be roughly estimated based on RV systolic pressure differential.

Higher incidence of heart failure and atrial fibrillation in valvular heart disease post-COVID-19

Abstract Funding Acknowledgements Type of funding sources: None. Patients with cardiovascular diseases are at higher risk of Coronavirus disease 2019 (COVID-19) complications. The data if post-COVID-19 patients are more susceptible to postoperative cardiac surgery complications are scarce. There are limited pieces of information on the possible consequences for patients with severe valvular disease undergoing valvular cardiac surgery. The study aimed to assess the impact of COVID-19 on patients with severe valvular disease and its postoperative course and outcomes. Methods We retrospectively identified 34 severe valvular disease patients indicated for surgery that underwent cardiac valvular surgery post-COVID-19. Adverse cardiovascular events in post-COVID-19 (heart failure (HF) and paroxysmal atrial fibrillation (AF) were recorded. All patients underwent control echocardiography preoperatively to assess changes in ejection fraction (EF), right ventricular systolic pressure (RVSP), and diastolic filling (E/e’). The patients were also followed for the postoperative course and hospital outcomes. All these data were compared to a historical cohort of 94 non-COVID-19 patients. Results There were no differences in mean age in post-COVID 65.2±11.6 vs. non-COVID-19 group 63.9±11.7, p = 0.57 or sex 74.6% vs. 70.7% of males, p = 0.38 or BMI 28.9± 4.5 vs. 28.2 ± 3.9, p = 0.42, with borderline significance in Euroscore 3 ± 4 vs. 1.6 ± 1.8, p = 0.05. retrospectively. Post-COVID-19 patients had a significantly higher prevalence of symptomatic HF 43.3% vs. 21.4%, p = 0.03, and AF 37.1% vs. 17%, p = 0.02 on admission. There were no significant differences between post-COVID-19 and COVID-19 in EF 56.9 ± 10.2 vs. 53.5 ± 12.6, p = 0.2, RVSP 38.1 ± 13.1 vs. 44.9 ± 12.8, p = 0.1 or E/e’ 13.8 ± 4.4 vs. 56.9 ± 10.2, p = 0.9. There were no significant differences in postoperative outcomes when post-COVID-19 group was compared to the non-COVID-19 group, the incidence of pneumonia was 8.6 vs. 6.9, p = 0.7, sepsis 2.9 vs. 4.3, p = 0.58, stroke 0% vs. 6.4%, p = 0.14, acute kidney failure 2.9 vs. 1.1, p = 0.47, postoperative acute MI 2.9% vs. 0%, p = 0.1, and mortality 0% vs. 1.1%, p = 0.5, retrospectively. There was a significantly higher incidence of transfusion in the post-COVID-19 group 77.1% vs. 41.5%, p &amp;lt; 0.01. Hospital stay for post-COVID-19 patients was 8.8 ± 4.4, vs. 8.7 ± 4 days, p = 0.94, total hospital stay 13 ± 7.4, vs. 11.4 ± 4.5, p = 0.2 and ICU stay 31.4 ± 37.8, vs. 44.4 ± 32.4 hours, p = 0.13. Conclusions Patients with severe valvular heart disease after COVID-19 are prone to develop heart failure and atrial fibrillation. The post-COVID-19 period following cardiac surgery is not associated with increased postoperative complications.

Effective echocardiographic parameters for predicting cardiac tamponade in patients with large amount of pericardial effusion

Abstract Funding Acknowledgements Type of funding sources: None. Background Cardiac tamponade is life-threatening medical condition that needs rapid diagnosis and pericardiocentesis. For diagnosing cardiac tamponade, comprehensive and complex doppler echocardiographic evaluation is necessary. The aim of this study was to evaluate the echocardiographic parameters for differentiating cardiac tamponade in patients with large amount of pericardial effusion. Methods From March 2003 to January 2020, 392 patients with large amount of PE with TTE evaluation were enrolled. We divided the patients into two groups; group A: patients performed subsequent drainage (n=197), group B: patients who are not performed drainage (n=195). Quantitative echocardiographic parameters were compared between two groups and analyzed which parameters were related with cardiac tamponade. Results The patients in group A showed low left ventricular end-diastolic diameter/body surface area (LVEDD/BSA) (25.44 ± 7.75 vs. 28.86 ± 6.10 mm/m2, p&amp;lt;0.001), left atrium volume index (LAVI) (24.08 ± 13.53 vs. 42.69 ± 28.69 ml/m2, p&amp;lt;0.001), early diastolic mitral inflow velocity (E velocity) (0.63 ± 0.18 vs. 0.88 ± 0.31 m/s, p&amp;lt;0.001), right ventricular systolic pressure (RVSP) (31.23 ± 9.47 vs. 39.16 ± 16.49 mmHg, p&amp;lt;0.001), early diastolic mitral inflow velocity/early diastolic mitral annular tissue velocity (E/e’) (11.62 ± 5.07 vs. 16.56 ± 7.75, p&amp;lt;0.001) compared with group B. In the multivariable analysis, E velocity was the most statistically significant parameter (Odds ratio=0.034, 95% CI=0.003–0.470, p = 0.012) and LAVI was the second one (Odds ratio=0.952, 95% CI=0.913–0.993, p = 0.022) related with cardiac tamponade. The ROC curve showed the cutoff value of E velocity was 0.678 m/s (sensitivity=0.675, specificity=0.673) and that of LAVI was 26.62 ml/m2 (sensitivity=0.681, specificity=0.679) for no need of pericardial drainage. Conclusion In patients with large amount of PE, E velocity and LAVI can be simple and useful echocardiographic parameters to differentiate the patients with cardiac tamponade who need pericardial drainage.

TAPSE/systolic pulmonary artery pressure in patients with repaired TOF: Does it correlate with cardiac magnetic resonance indices?

Abstract Funding Acknowledgements Type of funding sources: None. Background and Purpose Potential benefits and optimal timing of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) are still questionable because of high morbidity due to the need for reinterventions. Cardiac magnetic resonance (CMR) is a valuable tool for the detection of irreversible right ventricular (RV) remodelling. We sought to evaluate the relation between CMR derived volumes and peak tricuspid annular systolic excursion (TAPSE)/invasively measured systolic pulmonary artery pressure (sPAP) ratio in patients who underwent right heart catheterization. Methods One hundred and forty-eight patients with rTOF were evaluated; 26 of the patients either had right ventricular outflow tract obstruction or had corrective surgery with a pulmonary conduit. Patients with severe pulmonary regurgitation underwent CMR to quantify RV volumes and EF either before PVR or as part of the follow-up protocol including those who had previous PVR and recurrent pulmonary regurgitation. Echocardiographic indices of RV function were measured according to the EACVI 2015 recommendations for chamber quantification. In a subset of patients who underwent right heart catheterization, systolic RV pressure, sPAP were recorded and TAPSE/invasively measured sPAP ratio was calculated. CMR derived volumes and EF were compared with TAPSE/sPAP. Results Forty-six patients with matching studies for echo, CMR and right heart catheterization were analyzed. Mean age of the patients were 28.0 ± 7.3 years. Mean tricuspid systolic annular velocity (S’), TAPSE, and fractional area change were 11.4 ± 2.9 cm/sec, 16.8 ± 2.9 cm, and 31.7 ± 4.2 %, respectively. CMR derived end-diastolic volume index, end-systolic volume index and EF were 154.6 ± 38.5 ml/m2, 97.4 ± 26.5 ml/m2and 40.0 ± 7.6 %, respectively. There was a negative correlation between TAPSE/sPAP and CMR derived end-diastolic volume index (r= −0.44, p = 0.049) and end-systolic volume index (r= −0.50, p = 0.024) but not with RV EF. Conclusion In patient with rTOF and significant pulmonary regurgitation, TAPSE/invasively measured sPAP is more strongly correlated to CMR derived end-systolic volume index as opposed to end-diastolic volume index. Our findings support the use of CMR derived end-systolic volume index preferentially in the decision making for PVR.

Right Ventricle Myocardial Work in ToF-repair survivors: no longer behind the scenes

Abstract Funding Acknowledgements Type of funding sources: None. Background Tetralogy of Fallot is the most common cyanotic CHD and survivors after ToF repair are growing rapidly. Children and, more frequently, young adults spToF develop late complications: life-threatening ventricular arrhythmias and SCD remain a great concern in these patients. This study has the aim to analyse advanced Right Ventricle echocardiographic and CMR features sp-ToF in the long-term follow-up, finding non-invasive predictors of adverse prognosis. Methods This is a retrospective cohort study. 240 patients sp-ToF were included (mean age 13 ± 11 years). The electrocardiographic, echocardiographic, CMR and CPET data were reviewed retrospectively. Non-invasive RV Global myocardial Work Index (RVGWI) was calculated as the area of the RV pressure-strain loops. RV Global Constructive Work (RVGCW), RV Global Wasted Work (RVGWW) and RV Global Work Efficiency (RVGWE) were estimated. Patients were screened according to age at surgery, time since the radical repair at the follow-up, increased estimated RV systolic pressure, differences in instrumental findings after PVrep. A composite end-point of MACEs (syncope, sustained and non-sustained ventricular tachycardia, sudden cardiac death) was used. Results Older patients at surgery (≥12 months) have significantly reduced VO2 max at CPET, larger RA and LA areas and larger RV basal diameter. CPET values are not significantly different in patients with more than 25 years since the corrective surgery but echo and CMR-LVEF measured are slightly but significantly reduced and RVGWE is significantly lower. Patients with RVSP ≥ 60 mmHg have significantly higher RVGWW, worst RV FWLS, lower RVGCW and RVGWE. Patients who have undergone PVrep show improvement in RV GLS, RV FWLS and RVGWE and a RVGWW reduction. As reguards the CMR RV volume evaluation sp-ToF, patients with RVESVi ≥80ml/m2 have lower RVGWE and show an inverse correlation between RVEDVi and RVGWE. Sp-ToF patients with borderline/impaired predicted VO2 max at CPET have a reduced RV FWLS, higher RVGWW and an inverse correlation between METs and RVGWW. Patients who have suffered MACEs (2 NS-TV, 3 S-TV, 7 syncopes, 8 sudden deaths) hadsignificant impairment of RV systolic function (FAC, RV FWLS, CMR-RVFE), significantly higher RVGWW, lower RVGWE and RVGWI. Conclusion Long-term survival and clinical condition sp-ToF in infancy are generally good and late functional status in survivors could be excellent up to 25 years after radical correction. However, older patients at surgery have relative reduced functional status compared with those undergoing early repair. The advanced echocardiographic and CMR evaluation of RV function sp-ToF are usually related to worse prognosis, VT and reduced functional status. RV myocardial work evaluation is feasible in patients with repaired ToF, it may be a more sensitive indicator of RV fuctional impairment compared with standard echocardiographic parameters and is able to predict exercise capacity.

HIV status and survival of patients with pulmonary hypertension due to left heart disease: the Pan African Pulmonary Hypertension Cohort

In sub-Saharan Africa, little is known about pulmonary hypertension in left heart disease (PH-LHD). We used multivariate logistic and cox-hazard proportional regression models to examine factors associated with increased right ventricular systolic pressure (RVSP) and the effect of real-world HIV status scenarios on 6-month survival rate in the Pan African Pulmonary Hypertension Cohort (PAPUCO) study, a prospective cohort from four African countries. Exposure to biomass fuel smoke (aOR, 95%CI 3.07, 1.02–9.28), moderate to severe NYHA/FC III/IV (aOR, 95%CI 4.18, 1.01–17.38), and unknown HIV status (aOR, 95%CI 2.73, 0.96–7.73) predicted moderate to severe RVSP at the time of presentation. Six months later, HIV infection, moderate-to-severe NYHA/FC, and alcohol consumption were associated with decreased survival probabilities. Upon adjusting for HIV infection, it was observed that an incremental rise in RVSP (1 mmHg) and inter-ventricular septal thickness (1 mm) resulted in an 8% (aHR, 95%CI 1.08, 1.02–1.13) and 20% (aHR, 95%CI 1.2, 1.00–1.43) increase in the probability of mortality due to PH-LHD. In contrast, the risk of death from PH-LHD was reduced by 23% for each additional unit of BMI. (aHR, 95%CI 0.77, 0.59–1.00). In conclusion, the present study offers insights into the determinants that are notably linked to unfavorable survival outcomes in patients with pulmonary hypertension due to left heart disease. Certain factors identified in this study are readily evaluable and amenable to modification, even in settings with limited resources.

Relationship Between Transthoracic Echocardiography and Transesophageal Echocardiography in the Anatomic and Hemodynamic Assessment of Secundum Atrial Septal Defects in Children and Adults

Objective: To determine the relationship between transthoracic and transesophageal echocardiography in the anatomic and hemodynamic assessment of secundum atrial septal defects in children and adults.&#x0D; Study Design: Cross-sectional study.&#x0D; Place and Duration of Study: Department of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan, from Jul 2020 to Jun 2021.&#x0D; Methodology: Seventy-seven patients above three years of age with secundum atrial septal defects were included. Linear regression was used to determine any statistically significant difference between transthoracic and transesophageal echocardiography approaches.&#x0D; Results: Of 77 patients, there were 37(48.1%) male and 40(51.9%) female. No significant difference was noted in terms of any of the clinical parameters during transthoracic echocardiography and transesophageal echocardiography. As per transthoracic echocardiography, 35(45.5%) were noted to be deviceable, while in transesophageal echocardiography, 36(46.8%) patients were found to be deviceable (p=0.872). No emergency exploration or deaths occurred. Linear regression showed no statistically significant difference between transthoracic echocardiogram and transesophageal echocardiography findings in terms of atrial septal defects size (p=0.226), total septal length (p=0.930), RV systolic pressure (p=0.440), RV functions and TAPSE (0.748) except LV ejection fraction function findings (p&lt;0.001).&#x0D; Conclusion: Anatomic and hemodynamic assessment of secundum atrial septal defects in children and adults correlated well with transthoracic and transesophageal echocardiography.

#4358 INCREASED RV PRESSURE AS A PREDICTOR OF ACUTE DECOMPENSATED HF IN ESRD PATIENTS ON MAINTENANCE HD

Abstract Background and Aims Many patients with end-stage renal disease (ESRD) on hemodialysis (HD) have reduced vascular compliance and are likely to develop heart failure (HF). This study aimed to determine the factors associated with acute decompensation events among ESRD patients undergoing HD. Methods We retrospectively investigated ESRD patients on HD using a medical record review. We divided the patients into those admitted to hospital due to acute decompensated heart failure (ADHF) and those who were not. We compared the medical histories, electrocardiograms, and echocardiographic and laboratory data between the two groups. The cut-off value of the TR jet velocity for predicting an ADHF with corresponding sensitivity and specificity was estimated using a receiving operator characteristic (ROC) curve analysis. A p value &amp;lt; 0.05 was considered to indicate statistical significance. Results Of the 188 ESRD patients on HD, 87 were excluded, and 101 were enrolled (mean age: 63.7 years; 52.1% male). Thirty patients (29.7%) were admitted due to ADHF. These patients exhibited similar left ventricular ejection fraction (LVEF), left ventricular (LV) mass index, and E/E′ values compared to the non-ADHF group. However, the ADHF group exhibited significantly higher tricuspid regurgitation (TR) jet velocity (2.9±0.6 vs. 2.5±0.4 m/s; p = 0.004) and right ventricular systolic pressure (RVSP) (43.5±17.2 vs. 34.2±9.9 mmHg; p = 0.009) than the non-ADHF group, respectively. A multivariate logistic regression analysis demonstrated that the TR jet velocity (odds ratio, 8.356; 95% confidence interval, 1.806–38.658; p = 0.007) was an independent predictor of ADHF after adjusting for age and sex, while the LVEF and E/E′ were not. Conclusions Our data showed that an increased TR jet velocity was an independent predictor of ADHF events in ESRD patients on HD, but the LVEF and E/E′ were not.

Cardiometabolic Risk Factors Associated With Right Ventricular Function and Compensation in Patients Referred for Echocardiography.

Background Pulmonary hypertension and right ventricular (RV) dysfunction are drivers of adverse outcomes; however, modifiable risk factors for RV dysfunction are not well described. We investigated the association between clinical markers of metabolic syndrome and echocardiographic RV function in a large referral population. Methods and Results Using electronic health record data, we performed a retrospective cohort study of patients aged ≥18 years referred for transthoracic echocardiography between 2010 and 2020 with RV systolic pressure (RVSP) or tricuspid annular plane systolic excursion (TAPSE) values. Pulmonary hypertension was defined by RVSP >33 mm Hg and RV dysfunction by TAPSE ≤1.8 cm. Our sample included 37 203 patients of whom 19 495 (52%) were women, 29 752 (83%) were White, with a median age of 63 years (interquartile range, 51-73). Median (interquartile range) RVSP was 30.0 mm Hg (24.0-38.7), and median TAPSE was 2.1 cm (1.7-2.4). Within our sample, 40% had recorded RVSP >33 mm Hg, and 32% with TAPSE <1.8 cm. Increase in RVSP from normal (<33 mm Hg) to mildly elevated (33-39 mm Hg) or elevated (>39 mm Hg) was associated with lower low-density lipoprotein and high-density lipoprotein, and higher hemoglobin A1c and body mass index (P<0.001). A decrease in TAPSE between groups of TAPSE >1.8 cm, TAPSE 1.5-1.8 cm, and TAPSE <1.5 cm was associated with increased triglyceride:high-density lipoprotein ratio and hemoglobin A1c, and decreased body mass index, low-density lipoprotein, high-density lipoprotein, and systolic blood pressure (P<0.001). Most associations between cardiometabolic predictors and RVSP and TAPSE were nonlinear with clear inflection points associated with higher pulmonary pressure and lower RV function. Conclusions Clinical measures of cardiometabolic function were highly associated with echocardiographic measures of right ventricular function and pressure.

Establishment and Confirmation of a Postnatal Right Ventricular Volume Overload Mouse Model.

Right ventricular (RV) volume overload (VO) is common in children with congenital heart disease. In view of distinct developmental stages,the RV myocardium may respond differently to VO in children compared to adults. The present study aims to establish a postnatal RV VO model in mice using a modified abdominal arteriovenous fistula. To confirm the creation of VO and the following morphological and hemodynamic changes of the RV, abdominal ultrasound, echocardiography, and histochemical staining were performed for 3 months. As a result, the procedure in postnatal mice showed an acceptable survival and fistula success rate. In VO mice, the RV cavity was enlarged with a thickened free wall, and the stroke volume was increased by about 30%-40% within 2 months after surgery. Thereafter, the RV systolic pressure increased, corresponding pulmonary valve regurgitation was observed, and small pulmonary artery remodeling appeared. In conclusion, modified arteriovenous fistula (AVF) surgery is feasible to establish the RV VO model in postnatal mice. Considering the probability of fistula closure and elevated pulmonary artery resistance, abdominal ultrasound and echocardiography must be performed to confirm the model status before application.

Right Ventricular Systolic Pressure Trajectory as a Predictor of Hospitalization and Mortality in Patients With Chronic Heart Failure

BackgroundPulmonary hypertension is common among patients with heart failure (HF). Right ventricular systolic pressure (RVSP) is frequently used to assess its presence and severity. Although RVSP has been associated with adverse outcomes, the importance of serial measurements has not been studied. We evaluated associations between serial RVSP measurements and cardiovascular events in patients with HF. MethodsPatients with HF and 2 echocardiograms performed ≥ 6 months apart were included. RVSP was categorized, using the second echocardiogram, as follows: normal (< 40 mm Hg); severely elevated (≥ 60 mm Hg); moderately elevated (50-59 mm Hg); or mildly elevated (40-49 mm Hg). Patients also were classified according to change in RVSP categories between echocardiograms. The primary outcome was time to HF hospitalization (HFH) or all-cause mortality (ACM) after the second echocardiogram. ResultsIn total, 4319 patients were included (median age: 78 years; 52.1% female). During a median follow-up period of 19.4 months, HFH/ACM occurred in 2714 patients (62.8%). In multivariable analysis, baseline RSVP that was mildly elevated (1069 patients, hazard ratio [HR] 1.31, 95% confidence interval [CI] 1.12-1.54), moderately elevated (797 patients, HR 1.54, 95% CI 1.30-1.82), or severely elevated (837 patients, HR 1.92, 95% CI 1.60-2.31) was independently associated with HFH/ACM. Additionally, improving RVSP was associated with increased HFH/ACM in both categorical (HR 1.16, 95% CI 1.01-1.33) and continuous analyses. ConclusionsRVSP measurements identify patients at increased risk who may require more-aggressive monitoring and medical therapy. Our study raises the hypothesis that, in addition to the absolute value of RVSP, improving RVSP category may identify higher-risk patients, but further study is needed to elucidate the underlying reasons.

Macitentan in the Young-Mid-term Outcomes of Patients with Pulmonary Hypertensive Vascular Diseasetreated in a Pediatric Tertiary Care Center.

Pulmonary hypertension (PH) is a severe hemodynamic condition with high morbidity and mortality. Approved targeted therapies are limited for pediatric subjects, and treatments are widely adopted from adult algorithms. Macitentan is a safe and effective drug used for adult PH, but data on pediatric patients are limited. In this prospective single-center study, we investigated mid- and long-term effects of macitentan in children with advanced pulmonary hypertensive vascular disease. Twenty-four patients were enrolled in the study for treatment with macitentan. Efficacy was determined by echo parameters and brain natriuretic peptide levels (BNP) at 3months and 1year. For detailed analysis, the entire cohort was subgrouped into patients with congenital heart disease-related PH (CHD-PH) and non-CHD-PH patients, respectively. Mean age of the patients was 10.7±7.6 years; median observation period was 36months. Twenty of 24 patients were on additional sildenafil and/or prostacyclins. Two of 24 patients discontinued because of peripheral edema. Within the entire cohort, BNP levels and all echo measures such as right ventricular systolic pressure (RVSP), right ventricular end-diastolic diameter (RVED), tricuspid annular plane systolic excursion (TAPSE), pulmonary velocity time integral (VTI), and pulmonary artery acceleration time (PAAT) improved significantly after 3months (p ≤ 0.01), whereas in the long term significant improvement persisted for BNP levels (-16%), VTI (+14%) and PAAT (+11%) (p<0.05). By subgroup analysis, non-CHD PH patients showed significant improvements in BNP levels (-57%) and all echo measures (TAPSE +21%, VTI +13%, PAAT +37%, RVSP -24%, RVED -12%) at 3months (p≤0.01), whereas at 12months, improvements persisted (p<0.05) except for RVSP and RVED (nonsignificant). In CHD-PH patients, none of the measures changed (nonsignificant). 6-MWD (distance walked in 6 minutes)slightly increased but was not statistically evaluated. Data presented herein account for the largest cohort of severely affected pediatric patients receiving macitentan. Overall, macitentan was safe and associated with significant beneficial effects and sustained positive signals after 1year, albeit in the long term disease progression remains a major concern. Our data suggest limited efficacy in CHD-related PH, whereas favorable outcomes were mainly driven by improvements in patients with PH not related to CHD. Larger studies are needed to verify these preliminary results and to prove efficacy of this drug in different pediatric PH entities.

CHANGES ON RIGHT VENTRICULAR DIMENSIONS, FUNCTION, AND PULMONARY CIRCULATION LOADING ACCORDING TO THE DEGREE OF INTERDIALYTIC WEIGHT GAIN IN MAINTENANCE HEMODIALYSIS PATIENTS

Objective: The right ventricle (RV) is a thin-walled cardiac chamber, whose sizing and pressure characteristics are susceptible to altered loading conditions. This study aimed to investigate changes in RV's function, right heart's dimensions, and pulmonary pressures in relation to the degree of fluid accumulation between hemodialysis sessions, evaluated according to the recommended threshold of interdialytic-weight-gain corrected for dry weight (IDWG%). Design and method: Forty-one patients receiving standard thrice-weekly hemodialysis were divided into a higher (&gt; 4.5%) and a lower ( &lt; 4.5%) IDWG% group for the present analysis, according to the recommended higher threshold of IDWG%. All participants underwent a 2-dimensional echocardiographic assessment at the start and the end of the 3-day and the 2-day interdialytic interval in a crossover design. Results: RV systolic pressure (RVSP) increments were more pronounced in the higher compared to the lower IDWG% group (16.43 ± 5.37 vs 14.11 ± 13.38 mmHg respectively, p = 0.015) over the 3-day interval, while changes in RV filling pressures, assessed by RV E/Em did not differ significantly between the two groups (p = 0.84). Significant right heart chambers’ enlargement was observed in both groups and during both intervals, but a more prominent dilatation of inferior vena cava was noted in patients with higher IDWG% (0.44 ± 0.25 vs 0.2 ± 0.31cm, p = 0.014) over the 2-day interval. Conclusions: During the 3-day interdialytic interval, pulmonary circulation is particularly overloaded in patients with fluid accumulation higher that the recommended thresholds, as evidenced by higher RVSP elevations. This finding may add another plausible mechanism for increased cardiovascular morbidity and mortality during the long interval.

Right ventricular–pulmonary arterial coupling in patients with implanted left ventricular assist devices

ObjectiveBoth the right ventricular (RV) contractile function and pulmonary arterial (PA) pressure influence clinical outcomes in patients supported with left ventricular assist devices (LVADs), but the impact of RV-PA coupling is unknown. This study aimed to determine the prognostic impact of RV-PA coupling in patients with implanted LVADs. MethodsPatients with implanted third-generation LVADs were retrospectively enrolled. The RV-PA coupling was assessed preoperatively by the ratio of RV free wall strain (RVFWS) derived from speckle-tracking echocardiography and noninvasively measured peak RV systolic pressure (RVSP). The primary end point was a composite of all-cause mortality or right heart failure (RHF) hospitalization. Secondary end points consisted of all-cause mortality at a 12-month follow-up and RHF hospitalization. ResultsA total of 103 patients were screened, and 72 with good RV myocardial imaging were included. The median age was 57 years; 67 patients (93.1%) were men, and 41 (56,9%) had dilated cardiomyopathy. A receiver-operating characteristic analysis (AUC 0.703, 51.5% sensitivity, 94.9% specificity) was used to identify the optimal cutoff point (0.28%/mmHg) for the RVFWS/TAPSE threshold. Nineteen subjects (26.4%) had advanced RV-PA uncoupling. Event rates were estimated using the Kaplan-Meier method showing a strong association with an increased risk for the primary end point of death or RHF hospitalization (89.47% vs. 30.19%, p < 0.001). A similar observation applied to all-cause mortality (47.37% vs. 13.21%, p = 0.003) and RHF hospitalization (80.43% vs. 20%, p < 0.001). ConclusionsAn advanced RV dysfunction assessed by RV-PA coupling may serve as a predictor of adverse outcomes in patients with implanted LVADs.

0797 Correlation between Obstructive Sleep Apnea and Echocardiography Parameters in Infants and Preschool Children with Down Syndrome

Abstract Introduction Down syndrome (DS) is one of the most common genetic disorders caused by the Trisomy of chromosome 21. Children with DS secondary to the unique airway anatomy and hypotonia, are at risk for obstructive sleep apnea (OSA). Untreated OSA in children can lead to serious complications because of chronic hypoxia leading to Pulmonary hypertension (PHTN) and Cor pulmonale. An overnight polysomnogram (PSG), the gold standard for diagnosing OSA in children, should be performed within 4 years per American Academy of Pediatrics recommendations. However, OSA in DS has been observed at a much younger age. Methods In this single-center retrospective study, data was collected by reviewing the medical records and PSG REDCap database from 2019-2021. Patients with ICD code 10 diagnosis of DS between 0 to 4 years of age who underwent PSG and echocardiography were included. Our primary aim was to assess the severity of OSA in preschool children and describe echocardiogram parameters in DS patients with OSA. We hypothesized that there will be differences in polysomnogram parameters of DS children with and without PHTN. Results 61 patients met the criteria for the study with the mean age being 11.3 months. 3 patients (5.4%) did not have OSA (apnea hypopnea index (AHI)&amp;lt; 1/hr), 11 patients (19.6%) had mild OSA (AHI 1-4.9/hr), 12 patients (21.4%) had moderate OSA (AHI 5-10/hr), 30 patients had severe OSA (AHI &amp;gt;10/hr). Five patients with missing AHI values were excluded for subsequent analyses. Echocardiogram parameters were compared using the described AHI cut offs to define severity of OSA. OSA was observed to be significantly higher in the group with elevated RV systolic pressure. Conclusion Although our study did not find any difference in PSG findings in young children with DS with and without PHTN, obstructive apnea index was significantly elevated in young children with elevated RV systolic pressure. OSA is a significant contributor to the development of PHTN in children with DS. Children with DS are at higher risk for PHTN due to their unique pulmonary vasculature. As PHTN is a significant comorbidity of DS, it is important to perform PSGs early during childhood to detect and treat OSA. Support (if any)