Successful Management and Risk Stratification by Exercise Right Heart Catheterization Before Pregnancy in a Patient With Pulmonary Arterial Hypertension
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SUBMITTER: Yanagisawa Y
PROVIDER: S-EPMC10140741 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
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Project description: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.
| S-EPMC6643191 | biostudies-literature
Project description:BackgroundPrognosis in pulmonary arterial hypertension (PAH) is closely related to indexes of right ventricular function. A better understanding of their relationship may provide important implications for risk stratification in PAH.Research questionCan clinical network graphs inform risk stratification in PAH?Study design and methodsThe study cohort consisted of 231 patients with PAH followed up for a median of 7.1 years. An undirected, correlation network was used to visualize the relationship between clinical features in PAH. This network was enriched for right heart parameters and included N-terminal pro-hormone B-type natriuretic peptide (NT-proBNP), comprehensive echocardiographic parameters, and hemodynamics, as well as 6-min walk distance (6MWD), vital signs, laboratory data, and diffusing capacity for carbon monoxide (Dlco). Connectivity was assessed by using eigenvector and betweenness centrality to reflect global and regional connectivity, respectively. Cox proportional hazards regression was used to model event-free survival for the combined end point of death or lung transplantation.ResultsA network of closely intertwined features centered around NT-proBNP with 6MWD emerging as a secondary hub were identified. Less connected nodes included Dlco, systolic BP, albumin, and sodium. Over the follow-up period, death or transplantation occurred in 92 patients (39.8%). A strong prognostic model was achieved with a Harrell's C-index of 0.81 (0.77-0.85) when combining central right heart features (NT-proBNP and right ventricular end-systolic remodeling index) with 6MWD and less connected nodes (Dlco, systolic BP, albumin, sodium, sex, connective tissue disease etiology, and prostanoid therapy). When added to the baseline risk model, serial change in NT-proBNP significantly improved outcome prediction at 5 years (increase in C-statistic of 0.071 ± 0.024; P = .003).InterpretationNT-proBNP emerged as a central hub in the intertwined PAH network. Connectivity analysis provides explainability for feature selection and combination in outcome models.
| S-EPMC9131051 | biostudies-literature
Project description:BackgroundSeveral cardiopulmonary exercise test (CPET) parameters (peak VO2, PetCO2 and VE/VCO2) emerged as tools for the prediction of pulmonary arterial hypertension (PAH). Less is known on ventilatory power (VP) in patients with suspect PAH.AimTo ascertain possible correlations between VP derived at CPET and hemodynamic parameters at right heart catheterization (RHC) indicative of PH.MethodsForty-seven consecutive outpatients with suspect of PAH were assessed by CPET and RHC; VP was defined as peak SBP divided by the minute ventilation-CO2 production slope at CPET and Diastolic Pressure Gradient (DPG), Trans-pulmonary Pressure Gradient (TPG), mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) at RHC were also assessed and compared with VP.ResultsVP values were inversely related to mPAP (r -0.427, p 0.003), DPG (r -0.36, p 0.019), TPG (r: -0.43, p 0.004), and PVR (r -0.52, p 0.001). Correlations remained significant even after correction at multivariate analysis for age and gender. VP values below median identified subjects with mPAP ≥ 25 mmHg with an odds ratio of 4.5 (95% confidence interval 1.05-19.36, p < 0.05), an accuracy of 0.712 at ROC curve analysis (95% confidence interval 0.534-0.852, p < 0.05) and a positive predictive power 82%.ConclusionsIn patients with suspected PAH, VP assessed at CPET might provide further information in predicting PAH at RHC. Correlations with PVR and DPG may be helpful in differentiating patients with isolated post-capillary PH from those with combined post-capillary and pre-capillary.
| S-EPMC7178492 | biostudies-literature
Project description:Elevated right atrial (RA) pressure is an established prognostic measure in pulmonary arterial hypertension (PAH). However, little is known about perturbations in RA function in PAH.Reservoir (RA longitudinal strain [RA LS]), conduit (RA early LS rate), and active (RA late LS rate) phases were assessed by 2D speckle tracking in 65 patients with PAH, 6-minute walk distance ?450 m, and a pulmonary vascular resistance >800 dynes·s/cm(5), despite therapy with at least 2 PAH-specific medications enrolled in the Imatinib in Pulmonary Arterial Hypertension, a Randomized Efficacy Study (IMPRES) trial and were compared with 30 healthy controls of similar age and sex. We studied the association of RA functional measures with invasive pulmonary hemodynamics, cardiac structure and function, and N-terminal pro brain natriuretic peptide. RA LS and early LS rate were reduced in PAH patients compared with controls (27.1±11.6 versus 56.9±12.7, adjusted P<0.001 and -0.6±0.5 versus -1.5±0.5, adjusted P<0.001, respectively) even after adjusting for RA area and invasive RA pressure, whereas RA late LS rate was similar between groups (-1.4±0.7 versus -1.5±0.4, P=0.42). Among PAH patients, worse RA LS correlated with greater RA size (r=-0.50, P<0.0001) and pressure (r=-0.37, P=0.002), but not pulmonary artery pressure (r=-0.07, P=0.58). Worse RA LS was also associated with right ventricular enlargement and dysfunction and higher N-terminal pro brain natriuretic peptide independent of RA size and pressure.RA reservoir and passive conduit functions are impaired in PAH, independent of RA size and pressure, and likely reflect right ventricular failure and overload.URL: http://www.clinicaltrials.gov. Unique identifier: NCT00902174.
| S-EPMC4629509 | biostudies-literature
Project description:Pulmonary arterial hypertension (PAH) is a right heart failure syndrome. In early-stage PAH, the right ventricle tends to remain adapted to afterload with increased contractility and little or no increase in right heart chamber dimensions. However, less than optimal right ventricular (RV)-arterial coupling may already cause a decreased aerobic exercise capacity by limiting maximum cardiac output. In more advanced stages, RV systolic function cannot remain matched to afterload and dilatation of the right heart chamber progressively develops. In addition, diastolic dysfunction occurs due to myocardial fibrosis and sarcomeric stiffening. All these changes lead to limitation of RV flow output, increased right-sided filling pressures and under-filling of the left ventricle, with eventual decrease in systemic blood pressure and altered systolic ventricular interaction. These pathophysiological changes account for exertional dyspnoea and systemic venous congestion typical of PAH. Complete evaluation of RV failure requires echocardiographic or magnetic resonance imaging, and right heart catheterisation measurements. Treatment of RV failure in PAH relies on: decreasing afterload with drugs targeting pulmonary circulation; fluid management to optimise ventricular diastolic interactions; and inotropic interventions to reverse cardiogenic shock. To date, there has been no report of the efficacy of drug treatments that specifically target the right ventricle.
| S-EPMC9487395 | biostudies-literature
Project description:Right ventricular function critically affects the prognosis of patients with pulmonary arterial hypertension. We aimed to analyze the prognostic value of right ventricular indices calculated using magnetic resonance imaging and right heart catheterization metrics in pulmonary arterial hypertension. We retrospectively collected data from 57 Japanese patients with pulmonary arterial hypertension and 18 controls and calculated six indices of right ventricular function: two indices of contractility (end-systolic elastance calculated with right ventricular maximum pressure and with magnetic resonance imaging metrics); two indices of right ventricular-pulmonary arterial coupling (end-systolic elastance/arterial elastance calculated with the pressure method (end-systolic elastance/arterial elastance (P)) and with the volume method (end-systolic elastance/arterial elastance (V)); and two indices of right ventricular diastolic function (stiffness (β) and end-diastolic elastance). We compared the indices between controls and patients with pulmonary arterial hypertension and examined their prognostic role. In patients with pulmonary arterial hypertension, end-systolic elastance (right ventricular maximum pressure) was higher (pulmonary arterial hypertension 0.94 (median) vs control 0.42 (mmHg/mL), p < 0.001), end-systolic elastance/arterial elastance (V) was lower (pulmonary arterial hypertension 0.72 vs control 1.69, p < 0.001), and β and end-diastolic elastance were significantly higher than those in the controls. According to the log-rank test, end-systolic elastance/arterial elastance (P) and end-diastolic elastance were significantly associated with the composite event rate. According to the multivariate Cox regression analysis, decreased end-systolic elastance/arterial elastance (P) was associated with a higher composite event rate (hazard ratio 11.510, 95% confidence interval: 1.954-67.808). In conclusion, an increased right ventricular contractility, diastolic dysfunction, and a trend of impaired right ventricular-pulmonary arterial coupling were observed in our pulmonary arterial hypertension cohort. According to the multivariate outcome analysis, a decreased end-systolic elastance/arterial elastance (P), suggestive of impaired right ventricular-pulmonary arterial coupling, best predicted the pulmonary arterial hypertension-related event.
| S-EPMC7543138 | biostudies-literature
Project description: Not available
| S-EPMC7528786 | biostudies-literature
Project description: Not available
| S-EPMC9271414 | biostudies-literature
Project description:BackgroundRight ventricular (RV) functional reserve affects functional capacity and prognosis in patients with pulmonary arterial hypertension (PAH). PAH associated with systemic sclerosis (SSc-PAH) has a substantially worse prognosis than idiopathic PAH (IPAH), even though many measures of resting RV function and pulmonary vascular load are similar. We therefore tested the hypothesis that RV functional reserve is depressed in SSc-PAH patients.Methods and resultsRV pressure-volume relations were prospectively measured in IPAH (n=9) and SSc-PAH (n=15) patients at rest and during incremental atrial pacing or supine bicycle ergometry. Systolic and lusitropic function increased at faster heart rates in IPAH patients, but were markedly blunted in SSc-PAH. The recirculation fraction, which indexes intracellular calcium recycling, was also depressed in SSc-PAH (0.32±0.05 versus 0.50±0.05; P=0.039). At matched exercise (25 W), SSc-PAH patients did not augment contractility (end-systolic elastance) whereas IPAH did (P<0.001). RV afterload assessed by effective arterial elastance rose similarly in both groups; thus, ventricular-vascular coupling declined in SSc-PAH. Both end-systolic and end-diastolic RV volumes increased in SSc-PAH patients to offset contractile deficits, whereas chamber dilation was absent in IPAH (+37±10% versus +1±8%, P=0.004, and +19±4% versus -1±6%, P<0.001, respectively). Exercise-associated RV dilation also strongly correlated with resting ventricular-vascular coupling in a larger cohort.ConclusionsRV contractile reserve is depressed in SSc-PAH versus IPAH subjects, associated with reduced calcium recycling. During exercise, this results in ventricular-pulmonary vascular uncoupling and acute RV dilation. RV dilation during exercise can predict adverse ventricular-vascular coupling in PAH patients.
| S-EPMC4907868 | biostudies-literature
Project description:BACKGROUND:Pulmonary arterial hypertension (PAH) is a chronic debilitating illness. The effects of vigorous aerobic exercise training (AET) on heart function in PAH are poorly understood. METHODS:Eighteen women with PAH (aged 56.2 ± 8.8 yr, body mass index: 28.8 ± 7.3 kg/m) underwent 10 wk of vigorous AET. Cardiac function was observed at rest and peak exercise using bioelectrical impedance cardiography before and after the AET. Cardiac function was observed in a small PAH subset (n = 7) for 10 wk before beginning the AET. A cohort of sedentary women (n = 19) served as healthy controls. RESULTS:Left ventricular ejection fraction (48 ± 9.2 vs 61.5 ± 13.3%, P = .034) and the systemic vascular resistance index (2258 ± 419.1 vs 2939 ± 962.4 dyn·sec/cm·m, P = .008) were lower at supine rest in the baseline PAH group versus the healthy group, as were peak exercise heart rate (140 ± 13.3 vs 170 ± 13.8 beats/min, P < .001) and systemic vascular resistance index (828 ± 141.1 vs 824 ± 300.9 dyn·sec/cm·m, P = .050) after controlling for age and heart rate. Systemic vascular resistance index measured at peak exercise decreased in the PAH group after AET (828 ± 141.1 vs 766 ± 139.6 dyn·sec/cm·m, P = .020). Left ventricular early diastolic filling ratio worsened in the PAH subset prior to AET (95.9 ± 19.4 vs 76.2 ± 18.9%, P = .043) and remained unchanged after AET. CONCLUSION:Vigorous AET was not associated with significant declines in left ventricular systolic or diastolic function in women with PAH. Aerobic exercise training may be beneficial for reducing afterload and may preserve left ventricular diastolic function.
| S-EPMC6452473 | biostudies-literature