BACKGROUND: Pediatric pulmonary hypertension (PH) is characterized by a mean pulmonary arterial pressure exceeding 20 mmHg. There is limited research on the suitability of adult-based methods for estimating PH in pediatric populations. Using established formulas for adults, this study aimed to evaluate the correlation between echocardiographic estimates of systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures in children with congenital heart disease (CHD).
METHODS: A prospective study was conducted involving children with CHD undergoing cardiac catheterization without prior cardiac surgery. We used echocardiography to estimate pulmonary and right atrial pressures and compared these with invasively measured values. Four reliable regression equations were developed to estimate systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures. Cutoff values were determined to predict the occurrence of PH. Linear regression, Bland-Altman analysis, and receiver operating characteristic curve analysis were performed to assess the accuracy of echocardiography and establish diagnostic thresholds for PH.
RESULTS: The study involved 55 children (23 with normal pulmonary arterial pressure and 32 with PH) with acyanotic CHD aged 1 to 192 months. Four equations were developed to detect high pulmonary arterial pressures, with cutoff values of 32.9 for systolic pulmonary arterial pressure, 14.95 for diastolic pulmonary arterial pressure, and 20.7 for mean pulmonary arterial pressure. The results showed high sensitivity and moderate specificity but a tendency to underestimate systolic and mean pulmonary arterial pressures at higher pressures.
CONCLUSIONS: The study provides valuable insights into the use of adult-based echocardiographic formulas for estimating PH in pediatric patients with acyanotic CHD.

Infusion of fluids is one of the most common medical acts when resuscitating critically ill patients. However, fluids most often are given without consideration of how fluid infusion can actually improve tissue perfusion. Arthur Guyton\'s analysis of the circulation was based on how cardiac output is determined by the interaction of the factors determining the return of blood to the heart, i.e. venous return, and the factors that determine the output from the heart, i.e. pump function. His theoretical approach can be used to understand what fluids can and cannot do. In his graphical analysis, right atrial pressure (RAP) is at the center of this interaction and thus indicates the status of these two functions. Accordingly, trends in RAP and cardiac output (or a surrogate of cardiac output) can provide important guides for the cause of a hemodynamic deterioration, the potential role of fluids, the limits of their use, and when the fluid is given, the response to therapeutic interventions. Use of the trends in these values provide a physiologically grounded approach to clinical fluid management.

Endothelial dysfunction is important in the pathology of pulmonary hypertension, and circulating endothelial progenitor cells (EPCs) have been studied to evaluate endothelial dysfunction. In patients with chronic thromboembolic pulmonary hypertension (CTEPH), riociguat reportedly increases the number of circulating EPCs. However, the relationship between EPC numbers at baseline and changes in clinical parameters after riociguat administration has not been fully elucidated. Here, we evaluated 27 treatment-naïve patients with CTEPH and analyzed the relationships between EPC number at diagnosis and clinical variables (age, hemodynamics, atrial blood gas parameters, brain natriuretic peptide, and exercise tolerance) before and after riociguat initiation. EPCs were defined as CD45dim CD34+ CD133+ cells and measured by flow cytometry. A low number of circulating EPCs at diagnosis was significantly correlated with increased reductions in mean pulmonary arterial pressure (mPAP) (correlation coefficient = 0.535, P = 0.004) and right atrial pressure (correlation coefficient = 0.618, P = 0.001) upon riociguat treatment. We then divided the study population into two groups according to the mPAP change: a weak-response group (a decrease in mPAP of 4 mmHg or less) and a strong-response group (a decrease in mPAP of more than 4 mmHg). The number of EPCs at diagnosis was significantly lower in the strong-response group than in the weak-response group (P = 0.022), but there were no significant differences in other clinical variables or in medication profiles. In conclusion, circulating EPC numbers could be a potential predictor of the therapeutic effect of riociguat in CTEPH patients.

Discerning the etiology of acute kidney injury (AKI) in cirrhotic patients remains a formidable challenge due to diverse and overlapping causes. The conventional approach of empiric albumin administration for suspected volume depletion may inadvertently lead to fluid overload. In the recent past, point-of-care ultrasonography (POCUS) has emerged as a valuable adjunct to clinical assessment, offering advantages in terms of diagnostic accuracy, rapidity, cost-effectiveness, and patient satisfaction. This review provides insights into the strategic use of POCUS in evaluating cirrhotic patients with AKI. The review distinguishes basic and advanced POCUS, emphasizing a 5-point basic POCUS protocol for efficient assessment. This protocol includes evaluations of the kidneys and urinary bladder for obstructive nephropathy, lung ultrasound for detecting extravascular lung water, inferior vena cava (IVC) ultrasound for estimating right atrial pressure, internal jugular vein ultrasound as an alternative to IVC assessment, and focused cardiac ultrasound for assessing left ventricular (LV) systolic function and identifying potential causes of a plethoric IVC. Advanced POCUS delves into additional Doppler parameters, including stroke volume and cardiac output, LV filling pressures and venous congestion assessment to diagnose or prevent iatrogenic fluid overload. POCUS, when employed judiciously, enhances the diagnostic precision in evaluating AKI in cirrhotic patients, guiding appropriate therapeutic interventions, and minimizing the risk of fluid-related complications.

OBJECTIVE: Blood flow in the hepatic veins and superior vena cava (SVC) reflects right heart filling; however, their Doppler profiles are often not identical, and no studies have compared their diagnostic efficacies. We aimed to determine which venous Doppler profile is reliable for detecting elevated right atrial pressure (RAP).
METHODS: In 193 patients with cardiovascular diseases who underwent cardiac catheterization within 2 d of echocardiography, the hepatic vein systolic filling fraction (HV-SFF) and the ratio of the peak systolic to diastolic forward velocities of the SVC (SVC-S/D) were measured. HV-SFF < 55% and SVC-S/D < 1.9 were regarded as elevated RAP. We also calculated the fibrosis 4 index (FIB-4) as a serum liver fibrosis marker.
RESULTS: HV-SFF and SVC-S/D were feasible in 177 (92%) and 173 (90%) patients, respectively. In the 161 patients in whom both venous Doppler waveforms could be measured, HV-SFF and SVC-S/D were inversely correlated with RAP (r = -0.350, p < 0.001; r = -0.430, p < 0.001, respectively). SVC-S/D > 1.9 showed a significantly higher diagnostic accuracy of RAP elevation compared with HV-SFF < 55% (area under the curve, 0.842 vs. 0.614, p < 0.001). Multivariate analyses showed that both FIB-4 (β = -0.211, p = 0.013) and mean RAP (β = -0.319, p < 0.001) were independent determinants of HV-SFF. In contrast, not FIB-4 but mean RAP (β = -0.471, p < 0.001) was an independent determinant of SVC-S/D. The diagnostic accuracy remained unchanged when HV-SFF < 55% was considered in conjunction with the estimated RAP based on the inferior vena cava morphology. Conversely, SVC-S/D showed an incremental diagnostic value over the estimated RAP.
CONCLUSIONS: SVC-S/D enabled a more accurate diagnosis of RAP elevation than HV-SFF.

Impaired respiratory variation of right atrial pressure (RAP) in severe pulmonary hypertension (PH) suggests difficulty tolerating increased preload during inspiration. Our study explores whether this impairment links to specific factors: right ventricular (RV) diastolic function, elevated RV afterload, systolic RV function, or RV-pulmonary arterial (PA) coupling. We retrospectively evaluated respiratory RAP variation in all participants enrolled in the EXERTION study. Impaired respiratory variation was defined as end-expiratory RAP - end-inspiratory RAP ≤ 2 mm Hg. RV function and afterload were evaluated using conductance catheterization. Impaired diastolic RV function was defined as end-diastolic elastance (Eed) ≥ median (0.19 mm Hg/mL). Seventy-five patients were included; PH was diagnosed in 57 patients and invasively excluded in 18 patients. Of the 75 patients, 31 (41%) had impaired RAP variation, which was linked with impaired RV systolic function and RV-PA coupling and increased tricuspid regurgitation and Eed as compared to patients with preserved RAP variation. In backward regression, RAP variation associated only with Eed. RAP variation but not simple RAP identified impaired diastolic RV function (area under the receiver operating characteristic curve [95% confidence interval]: 0.712 [0.592, 0.832] and 0.496 [0.358, 0.634], respectively). During exercise, patients with impaired RAP variation experienced greater RV dilatation and reduced diastolic reserve and cardiac output/index compared with patients with preserved RAP variation. Preserved RAP variation was associated with a better prognosis than impaired RAP variation based on the 2022 European Society of Cardiology/European Respiratory Society risk score (chi-square P = 0.025) and survival free from clinical worsening (91% vs 71% at 1 year and 79% vs 50% at 2 years [log-rank P = 0.020]; hazard ratio: 0.397 [95% confidence interval: 0.178, 0.884]). Subgroup analyses in patients with group 1 and group 4 PH demonstrated consistent findings with those observed in the overall study cohort. Respiratory RAP variations reflect RV diastolic function, are independent of RV-PA coupling or tricuspid regurgitation, are associated with exercise-induced haemodynamic changes, and are prognostic in PH.Trial registration. NCT04663217.

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.
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.

In the absence of vascular obstruction, central venous pressure (CVP) is a hydrostatic pressure in the cranial and caudal vena cava, providing valuable information about cardiac function and intravascular volume status. It is also a component in evaluating volume resuscitation in patients with septic shock and monitoring patients with right heart disease, pericardial disease, or volume depletion. Central venous pressure is calculated in dogs by invasive central venous catheterization, which is considered high-risk and impractical in critically ill patients. This study aimed to investigate the feasibility of using echocardiographic tricuspid E/E\' as a noninvasive method to estimate CVP in anesthetized healthy dogs under controlled hypovolemic conditions. Ten male mixed-breed dogs were included in the study after a thorough health assessment. For hypovolemia induction, blood withdrawal was performed, and echocardiographic factors of the tricuspid valve, including peak E and E\' velocities, were measured during CVP reduction. Repeated measures analysis of variance and Bonferroni post hoc tests were employed to compare the average difference between measured echocardiographic indices and CVP values derived from catheterization and intermittent measurement methods. Spearman\'s ρ correlation coefficient was used to evaluate the correlation between echocardiographic indices and CVP. E peak velocity had a significant negative correlation with venous blood pressure phases (r = -0.44, P = .001), indicating a decrease in peak E velocity with progressive CVP reduction. However, tricuspid valve E\' peak velocity and E/E\' did not correlate with CVP, suggesting that these parameters are not reliable for CVP estimation in dogs.

Point-of-care ultrasound (POCUS) of the internal jugular vein (IJV) offers a non-invasive means of estimating right atrial pressure (RAP), especially in cases where the inferior vena cava is inaccessible or unreliable due to conditions such as liver disease or abdominal surgery. While many clinicians are familiar with visually assessing jugular venous pressure through the internal jugular vein, this method lacks sensitivity. The utilization of POCUS significantly enhances the visualization of the vein, leading to a more accurate identification. It has been demonstrated that combining IJV POCUS with physical examination enhances the specificity of RAP estimation. This review aims to provide a comprehensive summary of the various sonographic techniques available for estimating RAP from the internal jugular vein, drawing upon existing data.

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