scholarly journals Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension

2018 ◽  
Vol 8 (2) ◽  
pp. 204589401875924 ◽  
Author(s):  
Rachel K. Hopper ◽  
Yan Wang ◽  
Valerie DeMatteo ◽  
Ashley Santo ◽  
Steven M. Kawut ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Global Longitudinal Strain ◽  
Clinical Information ◽  
Pressure Change ◽  
Functional Class ◽  
Clinical Markers ◽  
Pulmonary Arterial ◽  
Rv Function ◽  
Prostacyclin Analogues

Pulmonary hypertension (PH) causes significant morbidity and mortality in children due to right ventricular (RV) failure. We sought to determine the effect of prostacyclin analogues on RV function assessed by echocardiography in children with PH. We conducted a retrospective cohort study of children with PH treated with a prostacyclin analogue (epoprostenol or treprostinil) between January 2001 and August 2015 at our center. Data were collected before initiation of treatment (baseline) and at 1–3 and 6–12 months after. Protocolized echocardiogram measurements including tricuspid annular plane systolic excursion (TAPSE) and RV global longitudinal strain were made with blinding to clinical information. Forty-nine individuals (65% female), aged 0–29 years at the time of prostacyclin initiation were included. Disease types included pulmonary arterial hypertension (idiopathic [35%], heritable [2%], and congenital heart disease-associated [18%]), developmental lung disease (43%), and chronic thromboembolic PH (2%). Participants received intravenous (IV) epoprostenol (14%) and IV/subcutaneous (SQ) (67%) or inhaled (18%) treprostinil. Over the study period, prostacyclin analogues were associated with improvement in TAPSE ( P = 0.007), RV strain ( P < 0.001), and qualitative RV function ( P = 0.037) by echocardiogram, and BNP ( P < 0.001), functional class ( P = 0.047) and 6-min walk distance ( P = 0.001). TAPSE and strain improved at early follow up ( P = 0.05 and P = 0.002, respectively) despite minimal RV pressure change. In children with PH, prostacyclin analogues are associated with an early and sustained improvement in RV function measured as TAPSE and strain as well as clinical markers of PH severity. RV strain may be a sensitive marker of RV function in this population.

scholarly journals CMR feature tracking cloud assess right ventricular functional reserve with pulmonary arterial hypertension

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
H Sato ◽  
Y Someya ◽  
M Nishiyama ◽  
W Satoh ◽  
K Kumasaka ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Feature Tracking ◽  
Global Longitudinal Strain ◽  
Early Career ◽  
Functional Reserve ◽  
Cine Mr ◽  
Pulmonary Arterial ◽  
Rv Function

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): JSPS KAKENHI, Grant-in-Aid for Early-Career Scientists. Background   Pulmonary arterial hypertension (PAH) remains a fatal disorder characterized by elevated pulmonary arterial pressure. Survival of the patients with PAH is determined from right ventricular (RV) function. CMR has become an attractive modality for following up and providing prognosis in such patients, and CMR feature tracking has been used as a newer useful parameter to assess RV function. However, it has not yet been determined whether CMR feature tracking can assess RV functional reserve in patients with PAH. Purpose We investigated whether CMR feature tracking can estimate RV functional reserve using a rat model with PAH. Methods  Rats were received injections with monocrotaline (MCT-rats, n = 19) or solvent (Ctr-rats, n = 5). Four weeks after the injections, we performed CMR on 7-T MRI scanner and imaged retrospective ECG-gated cine MR (16 phases/beat). RV ejection fraction (RVEF) and RV strain were analyzed before and after addition of 0.5∼3 nmol endothelin-1 (ET-1). After the measurements, we dissected trabeculae (length = 1.45 ± 0.07 mm, width = 334 ± 27 µm, thickness = 114 ± 6 µm) from the RVs of rat hearts. Trabeculae were electrically stimulated with 2-s intervals at extracellular Ca2+ of 0.7 and 2.0 mmol/L (24°C). Force and maximum dF/dt (dF/dtmax) were then measured using a silicon strain gauge in the absence and presence of 0.1 µM ET-1. Results  MCT-rats showed higher systolic RV pressure (RVP), lower RVEF, and lower RV global longitudinal strain (RVGLS) in CMR imaging and showed lower developed force and lower dF/dtmax in their trabeculae. Correlation between RVGLS and dF/dtmax was higher (r = 0.53, p &lt; 0.05) than that between RVEF and dF/dtmax (r = 0.24). In 5 MCT-rats with preserved RVEF (&gt;50%), RVGLS had already been reduced, suggesting that RVGLS is reduced earlier than RVEF. ET-1 increased developed force and dF/dtmax in trabeculae from MCT-rats (12.2 ± 5.7 to 17.4 ± 3.1 mN/mm2 and 0.08 ± 0.03 to 0.14 ± 0.06 mN/mm2/sec, respectively, n = 6), and ET-1 also increased RVP in MCT-rats and Ctr-rats (49.0 ± 19.3 to 59.7 ± 16.8 mmHg in MCT-rats, n = 6, 17.3 ± 7.5 to 20.4 ± 7.8 mmHg in Ctr-rats, n = 2). According to RV global circumferential strain (RVGCS) and RVEF, we could divide MCT-rats into three groups as follows: MCT-rats with reduced-RVGCS (&gt; -20%)/preserved-RVEF (&gt; 50%), MCT-rats with increased-RVGCS (&lt; -30%)/preserved-RVEF and MCT-rats with reduced-RVGCS/reduced-RVEF. ET-1 reduced RVGCS in MCT-rats with reduced-RVGCS/preserved-RVEF, while ET-1 did not change RVGCS in MCT-rats with increased-RVGCS/preserved-RVEF. MCT-rats with reduced-RVGCS/reduced-RVEF died after injection of ET-1.  In Ctr-rats, ET-1 did not change RVGCS and RVEF.  These results suggest that RVGCS can be useful to assess RV functional reserve. Conclusion  CMR feature tracking can estimate RV functional reserve earlier and more accurately than RVEF in rats with PAH.  RV strain may become an important parameter to assess RV functional reserve in patients with PAH.

The Influence of Pulmonary Hemodynamics on Right Ventricular Function in Pulmonary Hypertension

2013 ◽  
Author(s):  
Vitaly O. Kheyfets ◽  
Lourdes Rios ◽  
Triston Smith ◽  
Theodore Schroeder ◽  
Jeffrey Mueller ◽  
...  
Keyword(s):  
Fluid Dynamics ◽  
Pulmonary Hypertension ◽  
Shear Stress ◽  
Right Ventricular ◽  
Pulmonary Arteries ◽  
Pulmonary Hemodynamics ◽  
Arterial Hemodynamics ◽  
Computational Fluid Dynamics Cfd ◽  
Pulmonary Arterial ◽  
Rv Function

Pulmonary arterial hypertension (PAH) is a degenerative disease that can lead to substantial morphometric remodeling of the pulmonary arteries. Previous studies have revealed coupling relationships between right ventricular (RV) function and pulmonary arterial hemodynamics. The objective of this study was to utilize computational fluid dynamics (CFD) to estimate spatially averaged Wall Shear Stress (WSS) for patients with PH and explore correlations between hemodynamics metrics and RV function.

Clinical Significance of Guanylate Cyclase Stimulator, Riociguat, on Right Ventricular Functional Improvement in Patients with Pulmonary Hypertension

Cardiology ◽  
2020 ◽  
pp. 1-7
Author(s):  
Mitsushige Murata ◽  
Takashi Kawakami ◽  
Masaharu Kataoka ◽  
Hidenori Moriyama ◽  
Takahiro Hiraide ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Speckle Tracking ◽  
Longitudinal Strain ◽  
Speckle Tracking Echocardiography ◽  
Pulmonary Arterial Pressure ◽  
Global Longitudinal Strain ◽  
Mean Pulmonary Arterial Pressure ◽  
Echocardiographic Parameters ◽  
Pulmonary Arterial ◽  
Rv Function

<b><i>Background:</i></b> Riociguat is a soluble guanylate cyclase stimulator that improves hemodynamics in patients with pulmonary hypertension (PH). Accumulating evidence implicates the additional effect of riociguat on the increase in cardiac output. However, its mechanisms have not been fully understood. This study aimed to investigate whether riociguat could ameliorate right ventricular (RV) contraction as well as hemodynamics. <b><i>Methods:</i></b> We studied 45 patients with pulmonary arterial hypertension (14) or chronic thromboembolic pulmonary hypertension (31) and evaluated hemodynamics, using right-sided heart catheterization, before and after the administration of riociguat. RV function was assessed by echocardiography, including speckle-tracking echocardiography. <b><i>Results:</i></b> Riociguat significantly improved the WHO functional class and reduced the mean pulmonary arterial pressure and vascular resistance. In addition, the cardiac index increased. RV remodeling was ameliorated after riociguat administration as assessed by the echocardiographic parameters, such as RV diameter and RV area index. RV function, including RV fractional area change and RV global longitudinal strain, also significantly improved, and their improvement was even observed in patients with mild PH after pulmonary endarterectomy or balloon pulmonary angioplasty. Furthermore, covariance analysis revealed that RV global longitudinal strain and RV fractional area change improved after riociguat administration, even with the same mean pulmonary arterial pressure, implicating the improvement of RV contractile function by riociguat, regardless of RV loading. <b><i>Conclusions:</i></b> Riociguat not only improved the hemodynamics of patients with PH but also ameliorated the echocardiographic parameters with RV function. RV strain could detect the subtle improvement in mild PH, and riociguat may have a benefit even after intervention, as assessed by speckle-tracking echocardiography.

Abstract 14298: Right Ventricular Strain Before and After Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Darrin Wong ◽  
Averie Tigges ◽  
Lawrence Ang ◽  
MITUL PATEL ◽  
Hyong Kim ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Pearson Correlation ◽  
Global Longitudinal Strain ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Ventricular Strain ◽  
Balloon Pulmonary Angioplasty ◽  
Before And After ◽  
Thromboembolic Pulmonary Hypertension ◽  
Rv Function

Background: Right ventricular (RV) function is impaired in chronic thromboembolic pulmonary hypertension (CTEPH). Balloon pulmonary angioplasty (BPA) may be an alternative treatment for patients (pts) who are not candidates for pulmonary thromboendarterectomy (PTE). We assessed the RV global and segmental strain patterns in pts who underwent BPA. Methods: Between 12/2016 and 5/2019, 22 pts had completed BPA treatment and had transthoracic echocardiograms (echos) before and after treatment. Of those 22 pts, 14 had echos with adequate imaging of the RV. Epsilon EchoInsight® was used to measure global and segmental RV strain. Tricuspid annular plane excursion (TAPSE) was also measured. Paired t-tests were used to determine mean differences for pre & post BPA, and Pearson correlation coefficient was used to determine association with hemodynamics. Results: RV global longitudinal strain (GLS) significantly improved after BPA (-11.1±3.4 to -16.2±2.9 % p<0.05). The apical and mid segmental strain also significantly improved after BPA (-5.0±9.1 to -12.5±7.5%, p=0.03; -13.3±6.2 to -17.5±5.4%, p<0.01 respectively). Basal strain did not significantly change (-15.2±6.3 to -18.5±9.2%, p=0.22). Furthermore, TAPSE did not change significantly (1.87±0.5 to 1.95±0.5 cm, p=0.55). Hemodynamically, mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) significantly decreased (40.3±10.5 to 34.0±10.3 mmHg, p=0.001 and 5.9±3.1 to 3.9±1.8 WU, p=0.01) while cardiac output remained unchanged (5.6±2.0 to 5.7±1.1 L/min, p=0.86). There was no correlation between improvement in RV GLS and decrease in mPAP and PVR. Conclusion: RV function as measured by RV GLS significantly improved after BPA, though only the mid and apical segments improved. TAPSE and RV basal strain did not change after BPA. Previous studies have shown that GLS did not improve significantly after PTE. Furthermore, TAPSE significantly decreases after PTE. The reasons for the difference in RV function after PTE vs BPA are not entirely clear, but may be due to operability and location of disease, as well as immediate post-operative RV stunning after PTE.

scholarly journals Exaggerated Right Ventricular Dysfunction in Females with Group 3 Pulmonary Hypertension

2021 ◽  
Author(s):  
Sasha Z Prisco ◽  
Felipe Kazmirczak ◽  
Kurt W Prins ◽  
Thenappan Thenappan
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Ventricular Dysfunction ◽  
Right Ventricular Dysfunction ◽  
Female Group ◽  
Biological Sex ◽  
Effects Of Ph ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Rv Function

Group 3 pulmonary hypertension (PH) patients have disproportionate right ventricular dysfunction (RVD) as compared to pulmonary arterial hypertension (PAH) patients, but the cause of the divergent RV phenotypes is unknown. One potential mechanism may be biological sex as females have better RV function than males. However, the combined effects of PH type and sex on RV function are unexplored. Therefore, we evaluated how sex and PH etiology modulated RVD in a single-center cohort study. Male sex was not associated with significant differences in RV function when comparing PH etiologies. However, female Group 3 patients had more pronounced RVD than female PAH patients. In particular, Group 3 females had marked reduction in RV function when pulmonary vascular resistance was matched. Group 3 females were older than PAH females, but the exaggerated RVD was still observed in postmenopausal (age≥55) Group 3 females. This suggests lung disease exacerbates RVD in Group 3 females.

scholarly journals Right Ventricular Function and Its Coupling With Pulmonary Circulation in Precapillary Pulmonary Hypertension: A Three-Dimensional Echocardiographic Study

2021 ◽  
Vol 8 ◽  
Author(s):  
Yidan Li ◽  
Dichen Guo ◽  
Juanni Gong ◽  
Jianfeng Wang ◽  
Qiang Huang ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Clinical Outcomes ◽  
Predictive Value ◽  
Arterial Compliance ◽  
Systolic Pressure ◽  
Functional Class ◽  
Three Dimensions ◽  
World Health ◽  
Pulmonary Arterial

Objective: To assess right ventricular (RV) function and RV-pulmonary arterial (PA) coupling by three-dimensions echocardiography and investigate the ability of RV-PA coupling to predict adverse clinical outcomes in patients with precapillary pulmonary hypertension (PH).Methods: We retrospectively collected a longitudinal cohort of 203 consecutive precapillary PH patients. RV volume, RV ejection fraction (RVEF), and RV longitudinal strain (RVLS) were quantitatively determined offline by 3D echocardiography. RV-PA coupling parameters including the RVEF/PA systolic pressure (PASP) ratio, pulmonary arterial compliance (PAC), and total pulmonary resistance (TPR) were recorded.Results: Over a median follow-up period of 20.9 months (interquartile range, 0.1–67.4 months), 87 (42.9%) of 203 patients experienced adverse clinical outcomes. With increasing World Health Organization functional class (WHO-FC), significant trends were observed in increasing RV volume, decreasing RVEF, and worsening RVLS. RV arterial coupling (RVAC) and PAC were lower and TPR was higher for WHO-FC III+IV than WHO-FC I or II. The RVEF/PASP ratio showed a significant correlation with RVLS. RVAC had a stronger correlation with the RVEF/PASP ratio than other indices. Multivariate Cox proportional-hazard analysis identified a lower 3D RVEF and worse RVLS as strong predictors of adverse clinical events. RVAC, TPR, and PAC had varying degrees of predictive value, with optimal cutoff values of 0.74, 11.64, and 1.18, respectively.Conclusions: Precapillary-PH with RV-PA uncoupling as expressed by a RVEF/PASP ratio &lt;0.44 was associated with adverse clinical outcomes. PAC decreased and TPR increased with increasing WHO-FC, with TPR showing better independent predictive value.

scholarly journals Dynamic right ventricular–pulmonary arterial uncoupling during maximum incremental exercise in exercise pulmonary hypertension and pulmonary arterial hypertension

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401986243 ◽  
Author(s):  
Inderjit Singh ◽  
Farbod N. Rahaghi ◽  
Robert Naeije ◽  
Rudolf K.F. Oliveira ◽  
Rebecca R. Vanderpool ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Exercise Capacity ◽  
Incremental Exercise ◽  
Volume Index ◽  
Maximum Exercise ◽  
Pulmonary Arterial ◽  
Rv Function

Despite recent advances, the prognosis of pulmonary hypertension (PH) remains poor. While the initial insult in PH implicates the pulmonary vasculature, the functional state, exercise capacity, and survival of such patients are closely linked to right ventricular (RV) function. In the current study, we sought to investigate the effects of maximum incremental exercise on the matching of RV contractility and afterload (i.e. right ventricular–pulmonary arterial [RV–PA] coupling) in patients with exercise PH (ePH) and pulmonary arterial hypertension (PAH). End-systolic elastance (Ees), pulmonary arterial elastance (Ea), and RV–PA coupling (Ees/Ea) were determined using single-beat pressure-volume loop analysis in 40 patients that underwent maximum invasive cardiopulmonary exercise testing. Eleven patients had ePH, nine had PAH, and 20 were age-matched controls. During exercise, the impaired exertional contractile reserve in PAH was associated with blunted stroke volume index (SVI) augmentation and reduced peak oxygen consumption (peak VO2 %predicted). Compared to PAH, ePH demonstrated increased RV contractility in response to increasing RV afterload during exercise; however, this was insufficient and resulted in reduced peak RV–PA coupling. The dynamic RV–PA uncoupling in ePH was associated with similarly blunted SVI augmentation and peak VO2 as PAH. In conclusion, dynamic rest-to-peak exercise RV–PA uncoupling during maximum exercise blunts SV increase and reduces exercise capacity in exercise PH and PAH. In ePH, the insufficient increase in RV contractility to compensate for increasing RV afterload during maximum exercise leads to deterioration of RV–PA coupling. These data provide evidence that even in the early stages of PH, RV function is compromised.

Abstract 14221: Right Ventricular Adaptation to Pressure Overload: Differences Between Chronic Thromboembolic Pulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Natalia J Braams ◽  
Joost W van Leeuwen ◽  
anton vonk noordegraaf ◽  
Harm Jan Bogaard ◽  
Lilian J Meijboom ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Diastolic Stiffness ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Rv Function

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (iPAH)) are both associated with right ventricular (RV) failure and death. Although both conditions develop in the pre-capillary pulmonary vasculature, patient characteristics are different. CTEPH patients are older, predominantly male and more often have a history of venous thromboembolism. Therefore, the RV might be affected differently in CTEPH compared to iPAH. We aimed to compare RV adaptation in CTEPH and iPAH. Methods: Between 2000 and 2019 all treatment naive iPAH and CTEPH patients diagnosed in the Amsterdam UMC were included if a right heart catheterization and cardiac magnetic resonance imaging (CMR) were performed at the time of diagnosis. RV volumes, mass and function were assessed with CMR. RV contractility, afterload, RV-pulmonary artery (RV-PA) coupling and diastolic stiffness (Eed) were obtained using single beat pressure-volume loop analysis. Differences in RV phenotypes between iPAH and CTEPH were analyzed using multiple linear regression with interaction testing after correcting for confounders. Results: A total of 235 patients were included, 116 with CTEPH and 119 with iPAH. CTEPH patients were older, predominantly male, had a higher systemic blood pressure and a lower pulmonary vascular resistance at the time of diagnosis. After correcting for these confounders, RV function and RV-PA coupling were similar in both groups. However, CTEPH patients had a higher RV end-diastolic volume index (87±27 ml/m2 vs. 82±25 ml/m2), and a lower RV wall thickness (0,6±0,1 g/ml vs. 0,7±0,2 g/ml; figure 1A). The increase in afterload in CTEPH was associated with a disproportionally larger increase in diastolic stiffness compared to iPAH, independent of RV wall thickness (figure 1B). Conclusions: Despite a similar RV function, the RV in CTEPH is more dilated and stiffer than the RV in iPAH, independent of age, sex and afterload.

Right ventricular pressure-volume loop shape and systolic pressure change in pulmonary hypertension

2021 ◽  
Author(s):  
Manuel Jonas Richter ◽  
Steven Hsu ◽  
Athiththan Yogeswaran ◽  
Faeq Husain-Syed ◽  
István Vadász ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Pulse Pressure ◽  
Augmentation Index ◽  
Systolic Pressure ◽  
Pressure Differential ◽  
Stroke Work ◽  
Loop Shape ◽  
Pulmonary Arterial ◽  
Rv Function

Right ventricular (RV) function determines outcome in pulmonary arterial hypertension (PAH). RV pressure-volume loops - the gold standard for measuring RV function - are difficult to analyze. Our aim was to investigate whether simple assessments of RV pressure-volume loop morphology and RV systolic pressure differential reflect PAH severity and RV function. We analyzed multi-beat RV pressure-volume loops (obtained by conductance catheterization with preload reduction) in 77 patients with PAH and 15 patients without pulmonary hypertension in two centers. Patients were categorized according to their pressure-volume loop shape (triangular, quadratic, trapezoid, or notched). RV systolic pressure differential was defined as end-systolic minus beginning-systolic pressure (ESP−BSP); augmentation index as ESP−BSP/pulse pressure; pulmonary arterial capacitance (PAC) as stroke volume/pulse pressure; and RV-arterial coupling as end-systolic/arterial elastance (Ees/Ea). Trapezoid and notched pressure-volume loops were associated with the highest afterload (Ea), augmentation index, pulmonary vascular resistance (PVR), mean pulmonary arterial pressure, stroke work, and B-type natriuretic peptide, and the lowest Ees/Ea and PAC. Multivariate linear regression identified Ea, PVR, and stroke work as the main determinants of ESP−BSP. ESP−BSP also significantly correlated with multi-beat Ees/Ea (Spearman's rho: −0.518, P < 0.001). A separate retrospective analysis of 113 patients with PAH showed that ESP−BSP obtained by routine right heart catheterization significantly correlated with a non-invasive surrogate of RV-arterial coupling (tricuspid annular plane systolic excursion/pulmonary arterial systolic pressure ratio; rho: −0.376, P < 0.001). In conclusion, pressure-volume loop shape and RV systolic pressure differential predominately depend on afterload and PAH severity and reflect RV-arterial coupling in PAH.

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