Search

Introduction: The ratio between tricuspid annular plane systolic excursion (TAPSE) and estimated pulmonary artery systolic pressure (PASP) has been shown to be a reliable, non-invasive surrogate of the right ventricular-pulmonary artery (RV-PA) coupling. The present study analysed the association between TAPSE/PASP and response to cardiac resynchronization therapy (CRT) and the prognostic role of RV-PA in patients undergoing CRT implantation. The primary endpoints were: the association between baseline TAPSE/PASP and CRT response/cardiovascular and all-cause death., Methods and Results: All patients having undergone CRT implantation in our Center from 2016 to 2020 were included in our retrospective analysis. The RV-PA coupling was assessed by echocardiography at baseline and 1 year follow up in CRT recipients. The cut-off value of TAPSE/PASP resulted from ROC curve analysis (i.e.<0.33 mm/mmHg). A total of 229 patients (age 69.9 ± 10.1 years; 77.7% men) were included. During a mean follow-up of 44.2 ± 17.9 months, 40 (17.5%) patients died. The baseline value of TAPSE/PASP was not significantly associated with CRT response. Patients with a more impaired TAPSE/PASP ratio had significantly worse survival rates. On multivariate Cox regression, only TAPSE/PASP ratio and estimated glomerular filtration rate were independently associated with all-cause death. Finally, the TAPSE/PASP ratio significantly increased after CRT implantation in the group of "responders" whereas it did not change in 'non-responders'., Conclusions: The baseline value of TAPSE/PASP ratio was not associated with CRT response. However, the TAPSE/PASP ratio was a strong predictor of both all-cause and cardiovascular death in CRT recipients., Competing Interests: Declarations Competing interests The authors report no relationships that could be construed as a conflict of interest., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Pulmonary artery stenosis (PAS) often presents in children with congenital heart disease, altering blood flow and pressure during critical periods of growth and development. Variability in stenosis onset, duration, and severity result in variable growth and remodeling of the pulmonary vasculature. Computational fluid dynamics (CFD) models enable investigation into the hemodynamic impact and altered mechanics associated with PAS. In this study, a one-dimensional (1D) fluid dynamics model was used to simulate hemodynamics throughout the pulmonary arteries of individual animals. The geometry of the large pulmonary arteries was prescribed by animal-specific imaging, whereas the distal vasculature was simulated by a three-element Windkessel model at each terminal vessel outlet. Remodeling of the pulmonary vasculature, which cannot be measured in vivo, was estimated via model-fitted parameters. The large artery stiffness was significantly higher on the left side of the vasculature in the left pulmonary artery (LPA) stenosis group, but neither side differed from the sham group. The sham group exhibited a balanced distribution of total distal vascular resistance, whereas the left side was generally larger in the LPA stenosis group, with no significant differences between groups. In contrast, the peripheral compliance on the right side of the LPA stenosis group was significantly greater than the corresponding side of the sham group. Further analysis indicated the underperfused distal vasculature likely moderately decreased in radius with little change in stiffness given the increase in thickness observed with histology. Ultimately, our model enables greater understanding of pulmonary arterial adaptation due to LPA stenosis and has potential for use as a tool to noninvasively estimate remodeling of the pulmonary vasculature., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Right ventricular assist devices (RVADs) have been extensively used to provide hemodynamic support for patients with end-stage right heart (RV) failure. However, conventional in-parallel RVADs can lead to an elevation of pulmonary artery (PA) pressure, consequently increasing the right ventricular (RV) afterload, which is unfavorable for the relaxation of cardiac muscles and reduction of valve complications. The aim of this study is to investigate the hemodynamic effects of the pulsatile frequency of the RVAD on pulmonary artery. Firstly, a mathematical model incorporating heart, systemic circulation, pulmonary circulation, and RVAD is developed to simulate the cardiovascular system. Subsequently, the frequency characteristics of the pulmonary circulation system are analyzed, and the calculated results demonstrate that the pulsatile frequency of the RVAD has a substantive impact on the pulmonary artery pressure. Finally, to verify the analysis results, the hemodynamic effects of the pulsatile frequency of the RVAD on pulmonary artery are compared under diffident support modes. It is found that the pulmonary artery pressure decreases by approximately 6% when the pulsatile frequency changes from 1 to 3 Hz. The increased pulsatile frequency of RA-PA support mode may facilitate the opening of the pulmonary valve, while the RV-PA support mode can more effectively reduce the load of RV. This work provides a useful method to decrease the pulmonary artery pressure during the RVAD supports and may be beneficial for improving myocardial function in patients with end-stage right heart failure, especially those with pulmonary hypertension., Competing Interests: Declarations Conflict of interest The authors declare no competing interests., (© 2024. International Federation for Medical and Biological Engineering.)

Right ventricle-pulmonary artery (RV-PA) coupling has been linked to clinical outcomes in patients with severe aortic stenosis (AS) undergoing transcatheter valve implantation (TAVI). However, the best timing for prognostic assessment remains uncertain. Our aim was to determine the impact of RV longitudinal function parameters and RV-PA coupling on mortality in patients undergoing TAVI.  Retrospective, single center, analysis including patients with AS who underwent TAVI between 2007 and 2021. Echocardiographic evaluation was performed before, shortly after the procedure, and during follow-up. RV-PA uncoupling was defined as a TAPSE/PASP ratio<0.55 (severe RV uncoupling was defined as TAPSE/PASP ratio<0.32. The effect of RV parameters on all-cause mortality up to 12 months was assessed.  Among the 577 patients included, pre-procedural TAPSE/PASP ratio data were available for 205. RV-PA uncoupling was present in 113 patients (55.1%), with severe uncoupling observed in 31 (15.1%). Within the first 12 months after TAVI, 51 patients (9%) died. Severe RV-PA uncoupling was associated with mortality in univariable Cox regression; however, this association was lost after adjusting for EuroSCORE II. A significant association was found between the TAPSE/PASP ratio (per 0.1-unit increase) after the procedure and the primary endpoint (HR: 0.73; 95% CI: 0.56, 0.97; p=0.029). Higher postprocedural PASP (HR: 1.04; 95% CI: 1.02, 1.06; p<0.001 was also associated with all-cause mortality.  V-PA uncoupling and PASP after TAVI are associated with all-cause mortality in patients and may be valuable for patient selection and for planning post-procedural care., (© 2024. The Author(s).)

Purposr: This study created 3D CFD models of the Norwood procedure for hypoplastic left heart syndrome (HLHS) using standard angiography and echocardiogram data to investigate the impact of shunt characteristics on pulmonary artery (PA) hemodynamics. Leveraging routine clinical data offers advantages such as availability and cost-effectiveness without subjecting patients to additional invasive procedures., Methods: Patient-specific geometries of the intrathoracic arteries of two Norwood patients were generated from biplane cineangiograms. "Virtual surgery" was then performed to simulate the hemodynamics of alternative PA shunt configurations, including shunt type (modified Blalock-Thomas-Taussig shunt (mBTTS) vs. right ventricle-to-pulmonary artery shunt (RVPAS)), shunt diameter, and pulmonary artery anastomosis angle. Left-right pulmonary flow differential, Q p /Q s , time-averaged wall shear stress (TAWSS), and oscillatory shear index (OSI) were evaluated., Results: There was strong agreement between clinically measured data and CFD model output throughout the patient-specific models. Geometries with a RVPAS tended toward more balanced left-right pulmonary flow, lower Q p /Q s , and greater TAWSS and OSI than models with a mBTTS. For both shunt types, larger shunts resulted in a higher Q p /Q s and higher TAWSS, with minimal effect on OSI. Low TAWSS areas correlated with regions of low flow and changing the PA-shunt anastomosis angle to face toward low TAWSS regions increased TAWSS., Conclusion: Excellent correlation between clinically measured and CFD model data shows that 3D CFD models of HLHS Norwood can be developed using standard angiography and echocardiographic data. The CFD analysis also revealed consistent changes in PA TAWSS, flow differential, and OSI as a function of shunt characteristics., (© 2024. The Author(s) under exclusive licence to Biomedical Engineering Society.)

Purpose: This study examines the hepatic extracellular volume fraction (ECV) disparity between the left and right lobes (ECV&#95;left and ECV&#95;right) in patients with chronic thromboembolic pulmonary hypertension (CTEPH), its association with right heart catheterization (RHC) metrics, and with intolerance to increased pulmonary hypertension (PH)-targeted medication dosages., Methods: We retrospectively analyzed 72 CTEPH-diagnosed patients who underwent equilibrium-phase abdominal dual-energy CT (DECT) and RHC. Hepatic ECVs, derived from DECT's iodine maps using circular regions of interest in the liver and aorta, were correlated with RHC parameters via Spearman's rank correlation and lobe differences through the Wilcoxon signed-rank test. Logistic regression assessed cases with ECV&#95;left exceeding ECV&#95;right by > 0.05, while receiver operating characteristic curve analysis gauged ECVs' predictive power for medication intolerance., Results: Of the 72 patients (57 females; median age 69), ECV&#95;total (0.24, IQR 0.20-0.27) moderately correlated with RHC parameters (r s  = 0.28, -0.24, 0.3 for mean pulmonary arterial pressure, cardiac index [CI], and pulmonary vascular resistance index, respectively). ECV&#95;left significantly surpassed ECV&#95;right (0.25 vs. 0.22, p < 0.001), with a greater ECV&#95;left by > 0.05 indicating notably lower CI (p < 0.001). In 27 patients on PH medication, ECV&#95;left effectively predicted medication intolerance (AUC = 0.84)., Conclusion: In CTEPH patients, hepatic ECV correlated with RHC metrics, where elevated left lobe ECV suggested reduced CI and potential medication intolerance., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: Dynamic chest radiography using X-ray fluoroscopic video analysis has shown potential for the diagnosis of pulmonary embolism (PE), but its diagnostic performance remains uncertain. We aimed to evaluate the diagnostic performance of fluoroscopic video analysis for diagnosing PE., Methods: A prospective single-center observational study was conducted between October 2020 and January 2022. Fifty consecutive adult patients, comprising definitive PE, pulmonary hypertension (PH), or suspected PH, were enrolled. The study population was classified into 23 PE and 27 non-PE cases by contrast-enhanced computed tomography, lung scintigraphy, right heart catheterization, and pulmonary angiography. Cineradiographic images of 10-second breath-holds were obtained and analyzed using a fluoroscopic video analysis workstation to generate pulmonary circulation images. Two blinded cardiologists qualitatively assessed the presence or absence of perfusion defects on the pulmonary circulation images. The diagnosis obtained from the fluoroscopic analysis was compared with the definitive diagnosis. The primary outcomes included sensitivity, specificity, positive and negative predictive values, and overall accuracy for diagnosing PE., Results: Perfusion defects were observed in 21 of 23 PE patients and 13 of 27 non-PE patients. The diagnostic performance of fluoroscopic video analysis for diagnosing PE showed a sensitivity of 91%, specificity of 52%, positive predictive value of 62%, negative predictive value of 88%, and overall accuracy of 70%., Conclusions: The high sensitivity of the fluoroscopic video analysis suggests its potential usefulness in ruling out PE without the need for contrast media or radionuclide; however, its specificity and overall accuracy remain limited., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

4D-flow MRI is a promising technique for assessing vessel hemodynamics. However, its utilization is currently limited by the lack of reference values, particularly for pulmonary vessels. In this work, we have analysed flow and velocity in the pulmonary trunk (PT), left and right pulmonary arteries (LPA and RPA, respectively) in Landrace pigs at both rest and stress through the software MEVISFlow. Nine healthy Landrace pigs were acutely instrumented closed-chest and transported to the CMR facility for evaluation. After rest measurements, dobutamine was administered to achieve a 25% increase in heart rate compared to rest. 4D-flow MRI images have been analysed through MEVISFlow by two independent observers. Inter- and intra-observer reproducibility was quantified using intraclass correlation coefficient. A significant difference between rest and stress regarding flow and velocity in all the pulmonary vessels was observed. Mean flow increased 55% in PT, 75% in LPA and 40% in RPA. Mean peak velocity increased 55% in PT, 75% in LPA and 66% in RPA. A good-to-excellent reproducibility was observed in rest and stress for flow measurements in all three arteries. An excellent reproducibility for velocity was found in PT at rest and stress, a good one for LPA and RPA at rest, while poor reproducibility was found at stress. The current study showed that pulmonary flow and velocity assessed through 4D-flow MRI follow the physiological alterations during cardiac cycle and after stress induced by dobutamine. A clinical translation to assess pulmonary diseases with 4D-flow MRI under stress conditions needs investigation., (© 2024. The Author(s).)

To test the hypothesis that cine MRI-derived radiomics features of the cardiac blood pool can represent hemodynamic characteristics of pulmonary hypertension-heart failure with preserved ejection fraction (PH-HFpEF). Nineteen PH-HFpEF patients (9 male, 57.8 ± 14.7 years) and 19 healthy controls (13 male, 50.3 ± 13.6 years) were enrolled. All participants underwent a cardiac MRI scan. One hundred and seven radiomics features (7 classes) of the blood pool in the left and right ventricles/atrium (LV/RV/LA/RA) were extracted from 4-chamber cine (2D images) at the stages of systole, rapid filling, diastasis, and atrial contraction within a cardiac cycle. For PH-HFpEF patients, features acquired from LV/LA were related to the pulmonary capillary wedge pressure (PCWP); features acquired from RV/RA were related to the mean pulmonary artery pressure (mPAP) using the Pearson correlation coefficient (r). Logistic regression, receiver operating characteristic (ROC) curve and the area under the curve (AUC) were used to test the capability of radiomics features in discriminating 2 subject groups. Features acquired from different chambers at various periods present diverse properties in representing hemodynamic indices of PH-HFpEF. Multiple radiomics features blood pool were significantly related to PCWP and/or mPAP (r: 0.4-0.679, p < 0.05). In addition, multiple features of blood pools acquired at various time points within a cardiac cycle can efficiently discriminate PH-HFpEF from controls (individual AUC: 0.7-0.864). Cine MRI-derived radiomics features of the cardiac blood pool have the potential to characterize hemodynamic abnormalities in the context of PH-HFpEF., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Natural hemodynamic changes after balloon pulmonary artery angioplasty (BPA) in a unified state without oxygen administration in patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains undetermined. This study aimed to clarify the delayed changes in the hemodynamics after BPA in patients with CTEPH. We analyzed 73 consecutive patients with CTEPH who underwent BPA between July 2014 and December 2022. We extracted and evaluated hemodynamic data of the right heart catheter without oxygen administration immediately before and after the first BPA; and immediately before the second BPA, as the "post-delayed changes" after BPA. BPA significantly improved the mean pulmonary artery pressure (mPAP, mmHg) and pulmonary vascular resistance (PVR, dyn-s/cm 5 ) from 36 (32-43) mmHg and 449 (312-627) dyn-s/cm 5 before the first BPA to 28 (23-32) mmHg and 275 (217-366) dyn-s/cm 5 immediately after BPA, and further significantly improved the values to 23 (20-28) mmHg and 225 (175-301) dyn-s/cm 5 post-delayed changes after BPA, respectively. Improvement observed on account of delayed changes was observed both with and without pulmonary hypertension drugs. The delayed changes were observed during a period of 5-180 days, which did not correlate with the changes in mPAP and PVR. Hemodynamic improvement owing to BPA was observed immediately after BPA; however, further improvement was observed as a result of delayed changes. mPAP improved by 3.4 ± 5.2 mmHg and PVR by 53 (10-99) dyn-s/cm 5 as delayed improvement. mPAP and PVR showed delayed improved by approximately 10% of their values before BPA., (© 2024. The Author(s) under exclusive licence to Japanese Association of Cardiovascular Intervention and Therapeutics.)

We studied changes of pulmonary microhemodynamics when modeling pulmonary artery thromboembolism on perfused isolated rabbit lungs after pretreatment with ranolazine and ivabradine. The increase in pulmonary artery pressure, pulmonary vascular resistance, and pre- and postcapillary resistance was less pronounced than in control animals, but was close to that in case of pulmonary thromboembolism after pretreatment with voltage-gated Na + channel blockers lidocaine and ropivacaine. The increase of capillary filtration coefficient inversely correlated with values of capillary hydrostatic pressure. Thus, ranolazine and ivabradine exhibit the properties of voltage-gated Na + channel blockers mainly in smooth muscles of pulmonary arterial vessels and promote the decrease in endothelial permeability., (© 2024. Springer Science+Business Media, LLC, part of Springer Nature.)

Right ventricular (RV) diastolic stiffness is an independent predictor of survival and is strongly associated with disease severity in patients with precapillary pulmonary hypertension (PH). Therefore, a fully validated echocardiographic method for assessing RV diastolic stiffness needs to be established. This study aimed to compare echocardiography-derived RV diastolic stiffness and invasively measured pressure-volume loop-derived RV diastolic stiffness in patients with precapillary PH. We studied 50 consecutive patients with suspected or confirmed precapillary PH who underwent cardiac catheterization, magnetic resonance imaging, and echocardiography within a 1-week interval. Single-beat RV pressure-volume analysis was performed to determine the gold standard for RV diastolic stiffness. Elevated RV end-diastolic pressure (RVEDP) was defined as RVEDP ≥ 8 mmHg. Using continuous-wave Doppler and M-mode echocardiography, an echocardiographic index of RV diastolic stiffness was calculated as the ratio of the atrial-systolic descent of the pulmonary artery-RV pressure gradient derived from pulmonary regurgitant velocity (PRPGD AC ) to the tricuspid annular plane movement during atrial contraction (TAPM AC ). PRPGD AC /TAPM AC showed significant correlation with β (r = 0.54, p < 0.001) and RVEDP (r = 0.61, p < 0.001). A cut-off value of 0.74 mmHg/mm for PRPGD AC /TAPM AC showed 83% sensitivity and 93% specificity for identifying elevated RVEDP. Multivariate analyses indicated that PRPGD AC /TAPM AC was independently associated with disease severity in patients with precapillary PH, including substantial PH symptoms, stroke volume index, right atrial size, and pressure. PRPGD AC /TAPM AC , based on pulmonary regurgitation velocity waveform analysis, is useful for the noninvasive assessment of RV diastolic stiffness and is associated with prognostic risk factors in precapillary PH., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Pulmonary arterial hypertension (PAH) is a common complication of systemic lupus erythematosus (SLE), and PAH can cause right ventricle (RV) remodel and dyssynchrony. The aim of this study was to explore the value of RV dyssynchrony in predicting adverse clinical events in patients with systemic lupus erythematosus-aaociated pulmonary arterial hypertension (SLE-PAH) using two-dimensional speckle tracking echocardiography (2D-STE). A total of 53 patients with SLE-PAH were enrolled in this study. The dyssynchrony of the RV (RV-SD6) was evaluated by 2D-STE. The clinical data of all participants were collected, and routine cardiac function parameters were measured by two-dimensional echocardiography, and analyzed for their correlation with RV-SD6. The predictive value of RV-SD6 in clinical adverse event was evaluated. RV-SD6 was negatively correlated with RV-FLS, RV-FAC, and TAPSE (r = - 0.788, r = - 0.363 and r = - 0.325, respectively, all P < 0.01), while the correlation with RV-FLS was the strongest. linear regression analysis showed that RV-FLS was an independent risk factor for RV-SD6 (β = - 1.40, 95% CI - 1.65 ~ - 1.14, P < 0.001). Cox regression analysis showed that RV-SD6 was a predictor with clinical adverse events (HR = 1.03, 95% CI 1 ~ 1.06, P < 0.05). RV-SD6 was highly discriminative in predicting clinical adverse events (AUC = 0.764), at a cutoff of 51.10 ms with a sensitivity of 83.3% and specificity of 68.3%. RV-FLS was negatively correlated with RV-SD6 and was an independent risk factor for it. RV-SD6 can serve as an indicator for predicting the occurrence of adverse clinical events in SLE-PAH patients, with high sensitivity and specificity., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: Right heart catheterization (RHC) is the gold standard for the assessment of pulmonary artery systolic pressures (PASP). Despite high utilization of echocardiography for the non-invasive assessment of PASP, the data comparing real-time non-invasive echocardiographic PASP with invasive PASP is limited. Furthermore, evidence regarding the utility and diagnostic accuracy of ultrasound enhancing agents (UEA) for non-invasive PASP assessment is lacking. To evaluate the accuracy of non-invasive PASP assessment with real-time invasive measures and the incremental benefit of UEA in this setting., Methods: This was a prospective cohort study of 90 patients, undergoing clinically indicated RHC for hemodynamic assessment. All patients underwent a limited echocardiogram during RHC. Tricuspid regurgitant velocity (TRV) was measured on unenhanced echo, in the setting of centrally administrated agitated saline, then as either centrally administered or peripherally administered UEA., Results: Of the 90 patients enrolled in our study, 41% had pulmonary hypertension. The overall mean PASP measured by RHC was 32.8 mmHg (+/- 11.3 mmHg). Unenhanced echocardiograms had a moderate correlation with invasive PASP (r = 0.57; p = < 0.001) which improved to a strong correlation with administration of agitated saline (r = 0.75; p = < 0.001) or centrally administered UEA (r = 0.77; p = < 0.001), with the best correlation noted with peripherally administered UEA (r = 0.83; p = < 0.001). Against invasive PASP, agitated saline enhanced PASP had the lowest bias (0.12mmHg; -15.6 to 15.8mmHg) when compared with all other non-invasive measures of PASP., Conclusions: Unenhanced echocardiographic estimation of TRV was found to have a poorer correlation with invasively measured PASP when compared to agitated saline and centrally administered UEA. Agitated saline enhanced PASP demonstrated the lowest bias with invasive PASP when compared to other non-invasive measures of PASP., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Coronary artery anomalies are rare but potentially fatal abnormalities with occasional striking imaging findings radiologists should recognize., (© 2024. The Author(s).)

Untreated ventricular septal defects (VSDs) can lead to pulmonary arterial hypertension (PAH) characterized by elevated pulmonary artery (PA) pressure and vascular remodeling, known as PAH associated with congenital heart disease (PAH-CHD). Though previous studies have investigated hemodynamic effects on vascular mechanobiology in late-stage PAH, hemodynamics leading to PAH-CHD initiation have not been fully quantified. We hypothesize that abnormal hemodynamics from left-to-right shunting in early stage VSDs affects PA biomechanical properties leading to PAH initiation. To model PA hemodynamics in healthy, small, moderate, and large VSD conditions prior to the onset of vascular remodeling, computational fluid dynamics simulations were performed using a 3D finite element model of a healthy 1-year-old's proximal PAs and a body-surface-area-scaled 0D distal PA tree. VSD conditions were modeled with increased pulmonary blood flow to represent degrees of left-to-right shunting. In the proximal PAs, pressure, flow, strain, and wall shear stress (WSS) increased with increasing VSD size; oscillatory shear index decreased with increasing VSD size in the larger PA vessels. WSS was higher in smaller diameter vessels and increased with VSD size, with the large VSD condition exhibiting WSS >100 dyn/cm[Formula: see text], well above values typically used to study dysfunctional mechanotransduction pathways in PAH. This study is the first to estimate hemodynamic and biomechanical metrics in the entire pediatric PA tree with VSD severity at the stage leading to PAH initiation and has implications for future studies assessing effects of abnormal mechanical stimuli on endothelial cells and vascular wall mechanics that occur during PAH-CHD initiation and progression., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

We developed models of pulmonary artery (PA) size in Fontan patients as a function of age and body surface area (BSA) using linear regression and breakpoint analyses based on data from 43 Fontan patients divided into two groups: the extracardiac conduit (ECC) group (n = 24) and the non-ECC group (n = 19). Model predictions were compared against those of a non-Fontan control group (n = 18) and published literature. We observed strong positive correlations of the mean PA diameter with BSA (r = 0.9, p < 0.05) and age (r = 0.88, p < 0.05) in the ECC group. The absolute percentage differences between our BSA and age model predictions against published literature were less than 16% and 20%, respectively. Predicted PA size for Fontan patients was consistently smaller than the control group. These models may serve as useful references for clinicians and be utilized to construct 3D anatomic models that correspond to patient body size or age., (© 2020. Springer Science+Business Media, LLC, part of Springer Nature.)

The combination of early trans-mitral inflow and mitral annular tissue Doppler velocities (E/e' ratio) is widely applied to noninvasively estimate left ventricular (LV) filling pressures. However, when E/e' is between 8 and 14 its accuracy decreases substantially. Left atrial (LA) deformation analysis by speckle tracking echocardiography was recently proposed as an alternative approach to estimate LV filling pressures, but its role when E/e' is between 8 and 14 has been under-investigated. We aimed to assess whether LA strain could help to identify elevated filling pressures in patients with E/e' between 8 and 14. Among consecutive non-selected patients who underwent a comprehensive echocardiographic evaluation, we enrolled those with E/e' ratio > 8 and ≤ 14. Exclusion criteria were: organic mitral valve disease or mitral surgery; presence of mitral regurgitation greater than moderate in severity; diseases associated with pre-capillary pulmonary hypertension; and undetectable systolic pulmonary artery pressure (PAP-S). Peak LA longitudinal (PALS) and contraction strain (PACS) values was obtained by averaging all segments, and by separately averaging segments measured in the 4-chamber and 2-chamber views. Seventy-six patients had E/e' > 8 and ≤ 14 and formed the study cohort. Mean age 69 ± 12 years, LV ejection fraction (LVEF) 54.5 ± 11.2%, mean E/e' 11.2 ± 1.9, PAP-S 33 ± 7 mmHg, PALS 31.6 ± 11.7%. PALS was significantly associated to PAP-S after adjustment for LVEF, E/e', septal LV longitudinal shortening velocity (s'), LA volume indexed (p = 0.002) and also for ASE/EACVI diastolic dysfunction classification (p = 0.0002). Furthermore, PALS but not ASE/EACVI diastolic dysfunction grading, resulted independently associated to New York Heart Association (NYHA) class (p = 0.0004). PALS is able to predict increased intra-cardiac pressure and NYHA class in patients characterized by E/e' between 8 and 14. Therefore, we propose that PALS might be incorporated in a simplified diagnostic algorithm based on E/e' classes.

Accurate pulmonary artery (PA) imaging is necessary for management of patients with complex congenital heart disease (CHD). The ability of newer imaging modalities such as 3D rotational angiography (3DRA) or phase-contrast magnetic resonance angiography (PC-MRA) to measure PA diameters has not been compared to established angiography techniques. Measurements of PA diameters (including PA stenosis and PA stents) from 3DRA and non-contrast-enhanced PC-MRA were compared to 2D catheter angiography (CA) and multi-slice computed tomography (MSCT) in a swine CHD model (n = 18). For all PA segments 3DRA had excellent agreement with CA and MSCT (ICC = 0.94[0.91-0.95] and 0.92[0.89-0.94]). 3DRA PA stenosis measures were similar to CA and MSCT and 3DRA was on average within 5% of 10.8 ± 1.3 mm PA stent diameters from CA and MSCT. For compliant PA segments, 3DRA was on average 3-12% less than CA (p < 0.05) and MSCT (p < 0.01) for 6-14 mm vessels. PC-MRA could not reliably visualize stents and distal PA vessels and only identified 34% of all assigned measurement sites. For measured PA segments, PC-MRA had good agreement to CA and MSCT (ICC = 0.87[0.77-0.92] and 0.83[0.72-0.90]) but PC-MRA overestimated stenosis diameters and underestimated compliant PA diameters. Excellent CA-MSCT PA diameter agreement (ICC = 0.95[0.93-0.96]) confirmed previous data in CHD patients. There was little bias in PA measurements between 3DRA, CA and MSCT in stenotic and stented PAs but 3DRA underestimates measurements of compliant PA regions. Accurate PC-MRA imaging was limited to unstented proximal PA anatomy.

The sacubitril/valsartan combination is an important agent used in the treatment of heart failure with reduced ejection fraction (HFrEF). Pulmonary artery stiffness (PAS) is an index developed to evaluate the pulmonary vascular bed. Changes in pulmonary vascular structures in HFrEF patients can affect PAS. In this study, we aimed to investigate the effect of sacubitril/valsartan on PAS in HFrEF patients. One hundred fifty HFrEF patients, who received sacubitril/valsartan therapy and continued for at least 6 months without interruption, were examined retrospectively. N-terminal pro-B-type natriuretic peptide levels (NT-proBNP), NYHA classes, Minnesota Living with Heart Failure Questionnaire (MLWHFQ) scores, New York Heart Association (NYHA) functional classes and echocardiograpic parameters such as left ventricular ejection fraction (LVEF), mean pulmonary artery pressure (mPAP), right ventricle myocardial performance index (RV-MPI), Tricuspid annular plane systolic excursion (TAPSE), right ventricular fractional area change (RV-FAC) and PAS changes were evaluated before and 6 months after sacubitril/valsartan treatment. PAS was calculated by using the maximal frequency shift and acceleration time of the pulmonary artery flow trace measured in the echocardiogram. PAS values were significantly reduced (23.8 ± 2.8 vs 19.1 ± 3.1 kHz/ms, p < 0.001) after the sacubitril/valsartan treatment. Sacubitril/valsartan treatment was associated with significant improvements in NYHA class and MLWHFQ scores; significant reductions in the NT-proBNP levels, mPAP, and RV-MPI, and significant increases in LVEF, TAPSE, and RV-FAC (p < 0.05). The significant reduction in the PAS value was significantly correlated with the improvements in the MLWFQ scores, NT-proBNP levels, mPAP, RV-MPI, TAPSE and RV-FAC. In HFrEF patients, switching from angiotensin-converting enzyme inhibitor/angiotensin II receptor blocker therapy to sacubitril/valsartan may result in reduction in PAS.

The manuscript discusses the application of CT pulmonary angiography, ventilation-perfusion scan, and magnetic resonance angiography to detect acute pulmonary embolism and to plan endovascular therapy. CT pulmonary angiography offers high accuracy, speed of acquisition, and widespread availability when applied to acute pulmonary embolism detection. This imaging modality also aids the planning of endovascular therapy by visualizing the number and distribution of emboli, determining ideal intra-procedural catheter position for treatment, and signs of right heart strain. Ventilation-perfusion scan and magnetic resonance angiography with and without contrast enhancement can also aid in the detection and pre-procedural planning of endovascular therapy in patients who are not candidates for CT pulmonary angiography.

Pulmonary hypertension (PH) presents unusual hemodynamic states characterized by abnormal high blood pressure in pulmonary artery. The objective of this study is to simulate how the hemodynamics develops in typical PH cases without treatment. A lumped-parameter circuit platform of human circulation system is set up to simulate hemodynamic abnormalities of PH in different etiologies and pathogenesis. Four typical cases are considered, which are distal pulmonary artery stenosis, left ventricular diastolic dysfunction, ventricular septal defect, and mitral stenosis. The authors propose regulation laws for chambers and vessels to adapt the abnormal hemodynamic conditions for each PH case. The occurrence and development of each PH case are simulated over time using the lumped-parameter circuit platform. The blood pressure, blood flow, pressure-volume relations for chambers and vessels are numerically calculated for each case of PH progression. The model results could be a quite helpful to understand the hemodynamic mechanism of typical PHs. Graphical Abstract.

Purpose: In echocardiography the severity of aortic stenosis (AS) is defined by effective orifice area (EOA), mean pressure gradient (mPG AV ) and transvalvular flow velocity (maxV AV ). The hypothesis of the present study was to confirm the pathophysiological presence of combined left ventricular hypertrophy (LVH), diastolic dysfunction (DD) and pulmonary artery hypertension (PAH) in patients with "pure" severe AS., Methods and Results: Patients (n = 306) with asymptomatic (n = 133) and symptomatic (n = 173) "pure" severe AS (mean age 78 ± 9.5 years) defined by indexed EOA < 0.6 cm 2 were enrolled between 2014 and 2016. AS patients were divided into 4 subgroups according to mPG AV and indexed left ventricular stroke volume: low flow (LF) low gradient (LG)-AS (n = 133), normal flow (NF) LG-AS (n = 91), LF high gradient (HG)-AS (n = 21) and NFHG-AS (n = 61). Patients with "pure" severe AS showed mean mPG AV of 31.7 ± 9.1 mmHg and mean maxV AV of 3.8 ± 0.6 m/s. Only 131 of 306 patients (43%) exhibited mPG AV  > 40 mmHg and maxV AV  > 4 m/s documenting incongruencies of the AS severity assessment by Doppler echocardiography. LVH was documented in 81%, DD in 76% and PAH in 80% of AS patients. 54% of "pure" AS patients exhibited all three alterations. Ranges of mPG AV and maxV AV were higher in patients with all three alterations compared to patients with less than three. 224 (73%) patients presented LG-conditions and 82 (27%) HG-conditions. LVH was predominant in NF-AS (p = 0.014) and PAH in LFHG-AS (p = 0.014). Patients' treatment was retrospectively assessed (surgery: n = 100, TAVI: n = 48, optimal medical treatment: n = 156)., Conclusion: In patients with "pure" AS according to current guidelines the presence of combined LVH, DD and PAH as accepted pathophysiological sequelae of severe AS cannot be confirmed. Probably, the detection of these secondary cardiac alterations might improve the diagnostic algorithm to avoid overestimation of AS severity.

To compare intravenous contrast material (CM) injection protocols for dual-energy CT pulmonary angiography (CTPA) in patients with suspected acute pulmonary embolism with regard to image quality and pulmonary perfused blood volume (PBV) values. A total of 198 studies performed with four CM injection protocols varying in CM volume and iodine delivery rates (IDR) were retrospectively included: (A) 60 ml at 5 ml/s (IDR = 1.75gI/s), (B) 50 ml at 5 ml/s (IDR = 1.75gI/s), (C) 50 ml at 4 ml/s (IDR = 1.40gI/s), (D) 40 ml at 3 ml/s (IDR = 1.05gI/s). Image quality and PBV values at different resolution settings were compared. Pulmonary arterial tract attenuation was highest for protocol A (397 ± 110 HU; p vs. B = 0.13; vs. C = 0.02; vs. D < 0.001). CTPA image quality of protocol A was rated superior compared to protocols B and D by reader 1 (p = 0.01; < 0.001), and superior to protocols B, C and D by reader 2 (p < 0.001; 0.02; < 0.001). Otherwise, there were no significant differences in CTPA quality ratings. Subjective iodine map ratings did not vary significantly between protocols A, B, and C. Both readers rated protocol D inferior to all other protocols (p < 0.05). PBV values did not vary significantly between protocols A and B at resolution settings of 1, 4 and 10 (p = 0.10; 0.10; 0.09), while otherwise PBV values displayed a decreasing trend from protocol A to D (p < 0.05). Higher CM volume and IDR are associated with superior CTPA and iodine map quality and higher absolute PBV values.

Noninvasive estimation of systolic pulmonary artery pressure (SPAP) during exercise stress echocardiography (ESE) is recommended for pulmonary hemodynamics evaluation but remains flow-dependent. Our aim was to assess the feasibility of pulmonary vascular reserve index (PVRI) estimation during ESE combining SPAP with cardiac output (CO) or exercise-time and compare its value in three group of patients: with invasively confirmed pulmonary hypertension (PH), at risk of PH development (PH risk) mainly with systemic sclerosis and in controls (C) without clinical risk factors for PH, age-matched with PH risk patients. We performed semisupine ESE in 171 subjects: 31 PH, 61 PH at risk and 50 controls as well as in 29 young, healthy normals. Rest and stress assessment included: tricuspid regurgitant flow velocity (TRV), pulmonary acceleration time (ACT), CO (Doppler-estimated). SPAP was calculated from TRV or ACT when TRV was not available. We estimated PVRI based on CO (peak CO/SPAP*0.1) or exercise-time (ESE time/SPAP*0.1). During stress, TRV was measurable in 44% patients ACT in 77%, either one in 95%. PVRI was feasible in 65% subjects with CO and 95% with exercise-time (p < 0.0001). PVRI was lower in PH compared to controls both for CO-based PVRI (group 1 = 1.0 ± 0.95 vs group 3 = 4.28 ± 2.3, p < 0.0001) or time-based PVRI estimation (0.66 ± 0.39 vs 3.95 ± 2.26, p < 0.0001). The proposed criteria for PH detection were for CO-based PVRI ≤ 1.29 and ESE-time based PVRI ≤ 1.0 and for PH risk ≤ 1.9 and ≤ 1.7 respectively. Noninvasive estimation of PVRI can be obtained in near all patients during ESE, without contrast administration, integrating TRV with ACT for SPAP assessment and using exercise time as a proxy of CO. These indices allow for comparison of pulmonary vascular dynamics in patients with varied exercise tolerance and clinical status.

Pulse wave analysis enables stroke volume to be estimated from an arterial blood pressure waveform. Multi-beat analysis is a novel pulse wave analysis method. We aimed to investigate cardiac output (CO) estimations using multi-beat analysis of the radial arterial blood pressure waveform in patients undergoing off-pump coronary artery bypass surgery (OPCAB) using intermittent pulmonary artery thermodilution (PATD) as the reference method. This was a prospective clinical method comparison study. In 58 patients, we measured CO using PATD (PATD-CO; reference method) and simultaneously recorded the radial arterial blood pressure waveform that we used for off-line estimation of CO based on multi-beat analysis (MBA-CO; test method) using the Argos CO monitor (Retia Medical; Valhalla, NY, USA). The final analysis was performed using 572 paired CO measurements. We performed Bland-Altman analysis accounting for multiple observations per patient. To describe the ability of the test method to track changes in CO over time we computed four-quadrant plots using a central exclusion zone of 15% and calculated the concordance rate. Mean PATD-CO was 4.13 ± 1.26 L/min and mean MBA-CO was 4.31 ± 1.25 L/min. The mean of the differences between PATD-CO and MBA-CO was - 0.20 L/min with a standard deviation of ± 1.14 L/min and 95% limits of agreement of - 2.48 to + 2.08 L/min. The concordance rate for CO changes between PATD-CO and MBA-CO was 89%. CO estimations using multi-beat analysis (Argos monitor) show reasonable agreement and trending ability compared with PATD-CO as the reference method in adult patients during OPCAB.

Cardiac output (CO) is a key hemodynamic variable that can be minimally invasively estimated by pulse wave analysis. Multi-beat analysis is a novel pulse wave analysis method. In this prospective observational clinical method comparison study, we compared CO estimations by multi-beat analysis with CO measured by intermittent pulmonary artery thermodilution (PATD) in adult patients treated in the intensive care unit (ICU) after off-pump coronary artery bypass surgery (OPCAB). We included patients after planned admission to the ICU after elective OPCAB who were monitored with a radial arterial catheter and a pulmonary artery catheter. At seven time points, we determined CO using intermittent PATD (PATD-CO; reference method) and simultaneously recorded the radial arterial blood pressure waveform that we later used to estimate CO using multi-beat analysis (MBA-CO; test method) with the Argos monitor (Retia Medical; Valhalla, NY, USA). Blood pressure waveforms impaired by inappropriate damping properties or artifacts were excluded. We compared PATD-CO and MBA-CO using Bland-Altman analysis accounting for repeated measurements, the percentage error, and the concordance rate derived from four-quadrant plot analysis (15% exclusion zone). We analyzed 167 CO values of 31 patients. Mean PATD-CO was 5.30 ± 1.22 L/min and mean MBA-CO was 5.55 ± 1.82 L/min. The mean of the differences between PATD-CO and MBA-CO was 0.08 ± 1.10 L/min (95% limits of agreement: - 2.13 L/min to + 2.29 L/min). The percentage error was 40.7%. The four-quadrant plot-derived concordance rate was 88%. CO estimation by multi-beat analysis of the radial arterial blood pressure waveform (Argos monitor) shows reasonable agreement compared with CO measured by intermittent PATD in adult patients treated in the ICU after OPCAB.

Pulmonary hypertension (PH) is a well-recognised complication of sarcoidosis. Non-invasive diagnosis is challenging due to limited accuracy of echocardiography in interstitial lung disease. This study evaluates the value of echocardiographic PH probability for diagnosing PH in pulmonary sarcoidosis. All consecutive patients between August 2015 and November 2018 were prospectively screened for PH, and classified as low, intermediate or high PH probability. Patients with intermediate or high PH probability were referred for right heart catheterisation. PH was defined as a mean pulmonary artery pressure of ≥ 25 mm Hg. Additional data on pulmonary function and chest-CT was collected. Of all 479 patients, PH was present in 17 and absent in 19 patients. Six patients refused right heart catheterisation. PH was present in 33% and 75% of patients with intermediate and high PH probability respectively (n = 36). TRV max was measurable in 46% of all patients. Measurability did not correlate with FVC% predicted or presence of significant fibrosis. In intermediate and high PH probability, TRV max < 2.9 m/s successfully ruled out PH whereas a TRV max > 3.4 confirmed PH in all patients. If TRV max was absent or in between 2.9 and 3.4, secondary echocardiographic signs were not able to improve the diagnostic accuracy. PH is unlikely in patients with a TRV max < 2.9 m/s on echocardiography, whereas PH is highly suspected in a TRV max > 3.4 m/s. Discrimination is challenging if the TRV max is between 2.9-3.4 m/s or absent. Additional secondary signs do not improve discrimination. Decision making for further investigations should be made by an expert team.

Purpose: Additional pulmonary blood flow (APBF) can provide better pulsating blood flow and systemic arterial oxygen saturation, while low blood pulsation and low oxygen saturation are defects of the Fontan and Glenn procedure. Studying the hemodynamic effect of APBF is beneficial for clinical decisions. This study aimed to explore the effect on particle washout, as well as the differences among the sensitivities of both different hemodynamic parameters and different procedures to APBF., Methods: The patient-specific clinical datasets of a patient who underwent bilateral bidirectional Glenn (BBDG) with APBF were enrolled in this study, and using these datasets, Glenn- and Fontan-type artery models were reconstructed. A series of parameters, including the total caval flow pulsatility index (TCPI), indexed energy loss (iPL), wall shear stress (WSS), systemic arterial oxygen saturation (Sat art ), particle washout time (WOT), pressure in the right superior vena cava (P RSVC ), pulmonary flow distribution (PFD) and hepatic flow distribution (HFD), were computed from computational fluid dynamic (CFD) simulation to evaluate the hemodynamic effect of APBF., Results: The result showed that APBF led to better iPL and Sat art but worse P RSVC and heart load accompanied by a great impact on HFD, making hepatic flow easier to perfuse the side without MPA and APBF. The increase in the APBF rate also effectively results in larger flow pulsation, region velocity, and wall shear stress and lower WOT, and this effect may be more effective for patients with persistent left superior vena cava (PLSVC). However, APBF might have little effect on PFD. Furthermore, APBF might affect WOT, iPL and HFD more significantly than P RSVC and has a greater improvement effect in patients with poorer iPL and WOT., Conclusions: Moderate APBF is not only a measure to promote pulmonary artery growth and systemic arterial oxygen saturation but also an effective method against endothelial dysfunction and thrombosis. However, moderate APBF is patient-specific and should be determined based on hemodynamic preference that leads to desired patient outcomes, and care should be taken to prevent P RSVC and heart load from being too high as well as an imbalance in HFD.

Remodeling in the right-sided heart plays an important role in the management of pulmonary hypertension (PH) patients. However, the effect of balloon pulmonary angioplasty (BPA) on right ventricular (RV) and right atrial (RA) morphology of patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains uncertain. This study involved 45 CTEPH patients who underwent BPA with mean pulmonary artery pressure (mPAP) of 37.0 mmHg (all ≥ 25 mmHg). All patients underwent echocardiography and right-heart catheterization at baseline and 3 months after BPA. RV and RA remodeling was assessed as RV and the RA area, and RV systolic function was calculated by averaging peak speckle-tracking longitudinal strain of the RV free-wall (RV free-wall strain). Significant reverse remodeling in the right-sided heart was observed after BPA, resulting in improvement of mPAP and pulmonary vascular resistance (RV area: from 15.0 ± 5.3 to 9.6 ± 3.0 cm 2 , p < 0.0001; RA area: from 17.3 ± 6.6 to 13.4 ± 3.8 cm 2 , p = 0.0002; RV free-wall strain: from 15.9 ± 5.6 to 21.2 ± 4.9%, p < 0.0001). Furthermore, multiple regression analysis showed that the baseline RV area was an independent predictor of post-BPA normalization of RV systolic function defined as RV free-wall strain ≥ 20% (odds ratio = 1.16, p = 0.0305). Interestingly, significant RV reverse remodeling was also observed after additional BPA even in 18 CTEPH patients with residual pulmonary arterial stenosis, whose mPAP was normalized after BPA (RV area: from 11.5 ± 3.8 to 9.2 ± 3.8 cm 2 , p = 0.0045; RV free-wall strain: from 17.2 ± 4.8 to 22.8 ± 7.4%, p = 0.0216). Significant reverse remodeling in the right-sided heart, as well as hemodynamic improvement, was observed in CTEPH patients after BPA.

This study examined the relationship between global longitudinal strain (GLS) and pulmonary function tests (PFT) in patients with systemic sclerosis (SS) and normal ejection fraction (EF) and pulmonary artery pressure (PAP) and healthy controls. Sixty patients in two groups underwent extensive screening, including echocardiography, physical examination, the modified Rodnan Skin Score, and pulmonary function tests. Pulmonary interstitial disease was diagnosed by the pulmonary function test and by CT scan in case of indication. GLS score was computed as the mean peak systolic strain for 17 segments. The mean GLS score was - 18.36 ± 2.1 in the case group and - 20.66 ± 1.6 in the control group (P value < 0.001). GLS scores had a significant inverse relationship with the forced expiratory volume in one second (FEV1) to forced vital capacity (FVC) ratio (P value = 0.049) and both FEV and FVC in patients younger than 35 years old (P = 0.046 and 0.049, respectively). GLS scores had no significant relationship with time elapsed since the onset of skin manifestations, and Raynaud phenomenon, Rodnan score, EF, systolic PAP, or the six-minute walk test results. The patients' six-minute walk test had a significant positive relationship with FVC and right ventricular end diastolic diameter (P value = 0.018 and 0.047, respectively). According to our findings, GLS is significantly lower in patients with SS (with normal EF & PAP) than in healthy individuals. It is also related with certain pulmonary function indices including FEV1/FVC. The reduction in GLS is associated with reduced pulmonary function strength.

Purpose: For the relief of right ventricular outflow tract obstruction in operative treatment of tetralogy of Fallot and other complex congenital heart diseases, transannular monocusp patch operations are often necessary to prevent right ventricular pressure overload and reduce pulmonary regurgitation. However, long-term durability of a monocusp leaflet is unsatisfactory, its failure is believed to be related to mechanical stress, whose distribution is primarily affected by geometric configurations. Therefore, the influence of several geometrical parameters on stress distribution of leaflet is investigated., Methods: Five parameters affecting leaflet configuration were established: angle between free edge of the leaflet and vessel wall, angle formed by the two end points of free edge, length of the free edge of the leaflet, height of the leaflet, and shape of elliptic conical surface constituting the leaflet surface. The first four parameters were fixed, and two factors were defined to describe the last parameter. Seven models with different values of these factors were analyzed using finite element method at the pressure of the pulmonary artery loaded on the leaflet., Results: The peak stresses of all models occurred at end points of the free edge of the leaflet (tear high-risk regions). The middle of leaflet had the greatest stress gradient and produced tissue wrinkling; this area could be the risk region of calcification. Both factors were noted to influence the stress distribution, and one of the factors could also relieve the wrinkling., Conclusions: The leaflet of model (1.2&#95;min) had the most even stress distribution and lowest peak principal stress, which was the optimal choice among all the models.

To explore the value of right ventricular (RV) parameters detected by three-dimensional echocardiography (3DE) in risk stratification in pulmonary arterial hypertension (PAH) patients. We prospectively recruited 130 pulmonary hypertension patients from National Center for Cardiovascular Diseases, Fuwai Hospital. Each participant was performed a transthoracic echocardiography and 3DE parameters were measured using an off-line software (4D RV Function 2.0, TomTec). Patients were classified into low, intermediate-high risk group based on 2015 ESC Guidelines. A total of 91 PAH patients (34 ± 12 years old, 25 males) were enrolled, among which, 42 were classified into low risk group, while 49 were intermediate-high risk group. Compared with low-risk patients, those with intermediate-high risk had significantly larger 3DE-RV volumes, worse ejection fraction (EF) and tricuspid annular plane systolic excursion, and decreased longitudinal strain (LS). Receive operating characteristic curves illustrated all the 3DE parameters were able to predict intermediate-high risk stratification, especially 3D-RVEF (area under curve, 0.82, 95% CI 0.73-0.91, P < 0.001). And 3D-RVEF < 26.39% had a 81.6% sensibility and 73.8% specificity to predict intermediate-high risk stratification. Univariate and multivariate Logistic regression analyses identified 3D-RV end-diastolic (OR 1.02, 95% CI 1.01-1.03, P = 0.002) and end-systolic (OR 1.03, 95% CI 1.01-1.04, P < 0.001) volumes, 3D-RVEF (OR 0.82, 95% CI 0.75-0.90, P < 0.001) and LS of free wall (OR 1.17, 95% CI 1.05-1.31, P = 0.005) as independent predictors of intermediate-high risk stratification. In conclusion, RV volumes, EF and free wall strain detected by 3DE were independent predictors of intermediate-high risk stratification in PAH patients, among which, RVEF showed the best predictive capacity.

Tricuspid annular (TA) size, assessed by 2D transthoracic echocardiography (TTE), has a well-established prognostic value in patients undergoing mitral valve surgery, with TA dilatation triggering simultaneous tricuspid annuloplasty. While TA dilatation is common in patients with dilated atria secondary to atrial fibrillation, little is known about the mechanisms of TA dilatation in patients with sinus rhythm (SR). This study aimed to identify echocardiographic parameters most closely related to the TA size as a potential tool for identification of patients prone to developing TA enlargement. 120 patients with SR underwent clinically indicated TTE, including 30 patients with normal hearts and 90 patients diagnosed with at least one right heart abnormality, defined as: right ventricular (RV) or right atrial (RA) dilatation, ≥ moderate tricuspid regurgitation (TR) and elevated systolic pulmonary artery pressure (sPAP). RA and RV end-diastolic and end-systolic volumes (EDV, ESV) and function were measured using commercial 3D software (TomTec). 3D RV long and short axes were used as surrogate indices of RV shape. Degrees of TR and sPAP were estimated by 2D TTE. 3D TA sizing was performed at end-diastole using 3D custom software. Linear regression analysis was used to identify variables best correlated with TA size, followed by multivariate analysis to identify independent associations. The highest correlations were found between TA area and: RA ESV (r = 0.73; p < 0.01), RV EDV (r = 0.58; p < 0.01), RV end-diastolic long and short axes (r = 0.53, 0.42; both p < 0.01), TR degree (r = 0.40; p < 0.01) and sPAP (r = 0.32; p < 0.01). Multivariate analysis revealed that RA ESV was the only parameter independently associated with TA area (p < 0.05, r = 0.85). In conclusion, RA volume plays an important role in TA dilatation even in patients with normal SR. Understanding of annular remodeling mechanisms could aid in identifying patients at higher risk for TA dilatation, especially those scheduled for mitral valve surgery.

The use of 3D printed models of the right ventricular outflow tract (RVOT) for surgical and interventional planning is growing and often requires image segmentation of cardiac magnetic resonance (CMR) images. Segmentation results may vary based on contrast, image sequence, signal threshold chosen by the operator, and manual post-processing. The purpose of this study was to determine potential biases and post-processing errors in image segmentation to enable informed decisions. Models of the RVOT and pulmonary arteries from twelve patients who had contrast enhanced CMR angiography with gadopentetate dimeglumine (GPD), gadofosveset trisodium (GFT), and a post-GFT inversion-recovery (IR) whole heart sequence were segmented, trimmed, and aligned by three operators. Geometric agreement and minimal RVOT diameters were compared between sequences and operators. To determine the contribution of threshold, interoperator variability was compared between models created by the same two operators using the same versus different thresholds. Geometric agreement by Dice between objects was high (intraoperator: 0.89-0.95; interoperator: 0.95-0.97), without differences between sequences. Minimal RVOT diameters differed on average by - 1.9 to - 1.3 mm (intraoperator) and by 0.4 to 1.4 mm (interoperator). The contribution of threshold to interoperator geometric agreement was not significant (same threshold: 0.96 ± 0.06, different threshold: 0.93 ± 0.05; p = 0.181), but minimal RVOT diameters were more variable with different versus constant thresholds (- 9.12% vs. 2.42%; p < 0.05). Thresholding does not significantly change interoperator variability for geometric agreement, but does for minimal RVOT diameter. Minimal RVOT diameters showed clinically relevant variation within and between operators.

This experimental study describes the adaptive processes of the right ventricular (RV) myocardium after pulmonary artery banding (PAB) evaluated by cine cardiac magnetic resonance (CMR), phase-contrast CMR (PC-CMR), and conductance catheter. Seven sheep were subjected to CMR 3 months after PAB. Conductance catheter measurements were performed before and 3 months after PAB. Four nonoperated, healthy, age-matched animals served as controls. Higher RV masses (p < 0.01), elevated RV end-systolic volumes (p < 0.05), and lower RV ejection fraction (p < 0.01) were observed in the operated group. The time-to-peak pulmonary artery flow was longer in the banding group (p < 0.01). RV maximal pressure and RV end-diastolic pressure correlated with the time-to-peak flow in the pulmonary artery (r = - 0.70 and - 0.69, respectively). In summary, PAB caused RV hypertrophy, increased myocardial contractility, and decreased RV-EF and cardiac output. The time-to-peak pulmonary artery flow correlated with RV pressures.

Early detection of mild pulmonary arterial hypertension (PAH) based on clinical evaluation and echocardiography remains quite challenging. In addition to enhanced right ventricular (RV) assessment, cardiac magnetic resonance (CMR) imaging may accurately reflect deleterious remodeling and increased stiffness of the central pulmonary arteries based on pulsatility, or percent change of the PA during the cardiac cycle. The purpose of this study is to assess the utility of measuring PA pulsatility by CMR as a potential early maker in PAH. We hypothesize that pulsatility may help discriminate mild PAH from normal control subjects. Consecutive patients with PAH (n = 51) were prospectively enrolled to receive same day CMR and right heart catheterization (RHC). PA stiffness indices including pulsatility, distensibility, compliance, and capacitance were calculated. Comparisons were made between patients with varying severities of PAH and normal controls (n = 18). Of the 51 subjects, 20 had mild PAH, and 31 moderate-severe based on hemodynamic criteria. PA pulsatility demonstrated a progressive decline from normal controls (53%), mild PAH (22%), to moderate-severe PAH (17%; p < 0.001). There was no difference in RV size, function or mass between mild PAH and normal controls. PA pulsatility below 40% had an excellent ability to discriminate between mild PAH and normal controls with a sensitivity of 95% and specificity of 94%. CMR assessment of PA stiffness may noninvasively detect adverse pulmonary vascular remodeling and mild PAH, and thus be a valuable tool for early detection of PAH. Trial Registration: ClinicalTrials.gov Identifier: NCT01451255; https://clinicaltrials.gov/ct2/show/NCT01451255 .

A growing body of evidence has demonstrated that pulmonary arterial capacitance (PAC) is the strongest hemodynamic predictor of clinical outcomes across a wide spectrum of cardiovascular disease, including pulmonary hypertension and heart failure. We hypothesized that a ratio of right ventricular stroke volume (RVOT VTI) to the associated peak arterial systolic pressure (PASP) could function as a reliable non-invasive surrogate for PAC. We performed a prospective study of patients undergoing simultaneous transthoracic echocardiography and right heart catheterization (RHC) for various clinical indications. Measurements of the RVOT VTI/PASP ratio from echocardiographic measurements were compared against PAC calculated from RHC measurements. Correlation coefficients and Bland-Altman analysis compared the RVOT VTI/PASP ratio with PAC. Forty-five subjects were enrolled, 38% were female and mean age was 54 years (SD 13 years). The reason for referral to RHC was most commonly post-heart transplant surveillance (40%), followed by heart failure (22%), and pulmonary hypertension (18%). Pre-capillary pulmonary hypertension was present in 18%, isolated post-capillary pulmonary hypertension was present in 13%, and combined pre-and post-capillary pulmonary hypertension was present in 29%. The RVOT VTI/PASP ratio was obtainable in the majority of patients (78%), and Pearson's correlation demonstrated moderately-strong association between PAC and the RVOT VTI/PASP ratio, r = 0.75 (P < 0.001). Bland-Altman analysis demonstrated good agreement between measurements without suggestion of systematic bias and a mean difference in standardized units of - 0.133. In a diverse population of patients and hemodynamic profiles, we validated that the ratio of RVOT VTI/PASP to be a reliably-obtained non-invasive marker associated with PAC.

Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of connective tissue disease (CTD). Right atrial (RA) function is essential to maintaining adequate total right heart function in PAH. However, little is known about prognostic utility of RA function in CTD-PAH. RA longitudinal strain (LS) and strain rate (LSR) were evaluated in 53 consecutive patients (51 female, mean age 42 ± 15 years) with CTD-PAH, including systemic lupus erythematosus (SLE) (33.7%), mixed connective tissue disease (MCTD) (32.1%), primary Sjögren's syndrome (pSS) (26.4%), and systemic sclerosis (SSc) (3.8%). At a mean follow-up of 19.3 ± 10.9 months, 20 patients (37.7%) were clinically worse. The group with clinical events had worse clinical conditions and poorer RA function at baseline compared with the group that had no clinical events. RA LS independently reflected World Health Organization functional class (WHO FC) after adjusting for RA area (RAA), tricuspid regurgitation (TR) grade, right ventricular (RV) global longitudinal strain (GLS), and pulmonary vascular resistance (PVR) (P = 0.006). Receiver operator characteristic (ROC) curve analysis indicated that RA LS < 22.9% was predictive of clinical worsening during follow-up (sensitivity = 80%; specificity = 87.9%; area under the curve (AUC) = 0.858), and the Kaplan-Meier curve confirmed that RA LS ≥ 22.9% was associated with more favorable long-term outcomes compared to RA LS < 22.9% (log-rank P < 0.01). On univariate Cox proportional hazards analysis, PVR, RVGLS, RAA, and RA LS were associated with long-term outcome, while RA LS was the only independent predictor in the multivariate analysis. Our findings suggest that RA LS measurements by speckle-tracking echocardiography (STE) can independently reflect the extent of right heart failure and predict clinical outcomes in patients with CTD-PAH. RA LS < 22.9% is associated with a higher risk of clinical worsening.

Pulmonary hypertension (PH) is a pathophysiological disorder defined by an increase in pulmonary arterial pressure which can occur in multiple clinical conditions. Irrespective of etiology, PH entails a negative impact on exercise capacity and quality of life, and is associated with high mortality particularly in pulmonary arterial hypertension. Noninvasive imaging techniques play an important role in suggesting the presence of PH, providing noninvasive pulmonary pressure measurements, classifying the group of PH, identifying a possibly underlying disease, providing prognostic information and assessing response to treatment. While echocardiography, computed tomography (CT) and ventilation/perfusion scans are an integral part of routine work-up of patients with suspected PH according to current guidelines and across centers, innovative new techniques and applications in the field of PH such as 3D echocardiography, dual-energy CT, 4D flow magnetic resonance imaging (MRI), T1 and extracellular volume fraction mapping, non-contrast-enhanced MRI sequences for perfusion and ventilation assessment, and molecular-targeted positron emission tomography are emerging. This review discusses advanced and emerging imaging techniques in diagnosis, prognostic evaluation and follow-up of patients with PH.

Pulmonary arteriovenous malformation, a condition most commonly associated with hereditary hemorrhagic telangiectasia, is an abnormal communication between the pulmonary artery and pulmonary vein without an intervening capillary communication. Although asymptomatic in ~ 50% individuals, it can present with the dreaded complications of stroke or intracranial abscess in high-risk individuals including pregnant women, if untreated. The mainstay of treatment is now endovascular embolization of the feeding artery which can alleviate the symptoms and prevent these complications. In this review, we describe the pathophysiology, methods of screening, diagnostic workup and treatment of these vascular lesions with a particular focus on the currently used embolization techniques and their outcomes.

Background: Periodic echo-based screening to detect early stages of a rare complication of dasatinib, pulmonary arterial hypertension (PAH), is inefficient and weakens the potential benefit of dasatinib as a potent drug for chronic myelogenous leukemia (CML). This study aimed to identify the predisposing factors of DASA-PAH to stratify high-risk patients for dasatinib-induced PAH (DASA-PAH)., Methods: Sixty consecutive adult patients who received dasatinib were enrolled in this case-control study. We defined DASA-PAH when at least one of the following four criteria was met: (1) recent electrocardiographic changes indicating right ventricular pressure overload, (2) estimated systolic pulmonary arterial pressure > 40 mmHg measured by Doppler echocardiography; (3) computed tomography (CT)-measured pulmonary artery to aorta diameter (PaD/AoD) ratio > 1; and (4) mean pulmonary arterial pressure > 25 mmHg and pulmonary artery wedge pressure < 15 mmHg measured by right heart catheterization., Results: We identified 13 patients with DASA-PAH among 59 patients analyzed. Baseline PaD/AoD ratios of patients who developed DASA-PAH (PH group) were significantly larger than those who did not (NPH group). A dramatic rise in PaD/AoD ratio after dasatinib treatment was observed. Interestingly, the EUTOS score and spleen size were significantly smaller in the PH than in the NPH group., Conclusion: High baseline PaD/AoD ratio and low EUTOS score were associated with DASA-PAH development. The spleen might play a protective role against DASA-PAH.

The purpose of this study was to assess in pediatric pulmonary artery (PA) reconstruction candidates the feasibility and added utility of preoperative chest computed tomography angiography (CTA) using dual-energy technique, from which perfused blood volume (PBV)/iodine maps can be generated as a surrogate of pulmonary perfusion. Pediatric PA reconstruction patients were prospectively recruited for a new dose-neutral dual-energy CTA protocol. For each case, the severity of anatomic PA obstruction was graded by two pediatric cardiovascular radiologists in consensus using a modified Qanadli index. PBV maps were qualitatively reviewed and auto-segmented using Siemens syngo.via software. Associations between Qanadli scores and PBV were assessed with Spearman correlation (r) and ROC analysis. Effective radiation doses were estimated from dose-length product and ICRP 103 k-factors, using cubic Hermite spline interpolation. 19 patients were recruited with mean (SD) age of 6.0 (5.1), 11 (57.9%) female, 11 (73.7%) anesthetized. Higher QS correlated with lower PBV, both on a whole lung (r =  - 0.54, p < 0.001) and lobar (r =  - 0.50, p < 0.001) basis. The lung with lowest absolute PBV was predictive of the lung with highest Qanadli score, with AUC of 0.70 (95% CI 0.47-0.93). Qualitatively, PBV maps were heterogeneous, corresponding to multifocal PA stenoses, with decreased iodine content in areas of most severe obstruction. In conclusion, dual-energy chest CTA is feasible for pediatric PA reconstruction candidates. PBV maps show deficits in regions of more severe anatomic obstruction and may serve as a novel biomarker in this population.

Acute pulmonary embolism (PE) is a major public health problem and accounts for 100,000-180,000 deaths per year in the United States. Current prognostic stratification separates acute PE into massive, submassive, and low-risk by the presence or absence of sustained hypotension, RV dysfunction, and myocardial necrosis. Massive, submassive and low-risk PE have mortality rates of 25-65%, 3%, and < 1%, respectively. In this review we will focus on therapies currently available to manage acute massive and submassive PE.

Congenital pulmonary artery (PA) anomalies comprise a rare and heterogeneous spectrum of disease, ranging from abnormal origins to complete atresia. They may present in early infancy or more insidiously in adulthood, often in association with congenital heart disease such as tetralogy of Fallot or other syndromes. In recent years, cross-sectional imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), has become widely utilized for the noninvasive assessment of congenital PA diseases, supplementing echocardiography and at times supplanting invasive angiography. In this article, modern CT and MRI techniques for imaging congenital PA disorders are summarized. The key clinical features, cross-sectional imaging findings, and treatment options for the most commonly encountered entities are then reviewed. Emphasis is placed on the ever-growing role of cross-sectional imaging options in facilitating early and accurate diagnosis and tailored treatment.

Magnetic resonance imaging (MRI) plays an increasingly important role in the non-invasive evaluation of the pulmonary vasculature. MR angiographic (MRA) techniques provide morphological information, while MR perfusion techniques provide functional information of the pulmonary vasculature. Contrast-enhanced MRA can be performed at high spatial resolution using 3D T1-weighted spoiled gradient echo sequence or at high temporal resolution using time-resolved techniques. Non-contrast MRA can be performed using 3D steady state free precession, double inversion fast spin echo, time of flight or phase contrast sequences. MR perfusion can be done using dynamic contrast-enhanced technique or using non-contrast techniques such as arterial spin labelling and time-resolved imaging of lungs during free breathing with Fourier decomposition analysis. MRI is used in the evaluation of acute and chronic pulmonary embolism, pulmonary hypertension and other vascular abnormalities, congenital anomalies and neoplasms. In this article, we review the different MR techniques used in the evaluation of pulmonary vasculature and its clinical applications.

Multi-energy computed tomography (MECT) refers to acquisition of CT data at multiple energy levels (typically two levels) using different technologies such as dual-source, dual-layer and rapid tube voltage switching. In addition to conventional/routine diagnostic images, MECT provides additional image sets including iodine maps, virtual non-contrast images, and virtual monoenergetic images. These image sets provide tissue/material characterization beyond what is possible with conventional CT. MECT provides invaluable additional information in the evaluation of pulmonary vasculature, primarily by the assessment of pulmonary perfusion. This functional information provided by the MECT is complementary to the morphological information from a conventional CT angiography. In this article, we review the technique and applications of MECT in the evaluation of pulmonary vasculature.

An accurate distinction between isolated post-capillary pulmonary hypertension (Ipc-PH) and combined post- and pre-capillary pulmonary hypertension (Cpc-PH) is integral to therapy and prognosis in heart failure (HF). This study aimed to compare the ability of four previously validated Doppler estimates of pulmonary vascular resistance (PVR Doppler ) to distinguish Ipc-PH from Cpc-PH in a well-defined HF population. Consecutive subjects referred for HF assessment underwent standard echocardiography immediately followed by right heart catheterization (RHC). Subjects with atrial fibrillation, acute coronary syndrome, significant valvular disease or poor image quality were excluded. PVR Doppler estimates were correlated with invasive PVR and agreement was studied using Bland-Altman analysis. Receiver operating characteristics analyses were performed to determine the ability of PVR Doppler methods to identify PVR > 3WU. 55 HF subjects (58 ± 16 years, 55% Ipc-PH) were analyzed. PVR Doppler estimates demonstrated weak to modest associations with invasive PVR. The Doppler method proposed by Abbas et al. demonstrated relatively strong discriminatory ability to distinguish Ipc-PH from Cpc-PH (AUC = 0.79; 95% CI 0.63-0.96; p = 0.001). However, Bland-Altman analysis revealed wide limits of agreement (bias = 0; SD = 1.83WU) and greater variability at higher mean PVR. Conclusions: PVR Doppler estimates demonstrate reasonable ability to distinguish Ipc-PH from Cpc-PH but may not be reliable independent PH distinguishers in HF.