Hemodynamic trajectories and outcomes in patients with pulmonary arterial hypertension

Amsallem, Myriam; Bagherzadeh, Shadi P; Boulate, David; Sweatt, Andrew J.; Kudelko, Kristina; Sung, Yon K.; Feinstein, Jeffrey A.; Fadel, Élie; Mercier, Olaf; Denault, André; Haddad, François; Zamanian, Roham T.

doi:10.1177/2045894020941343

Cited by 4 publications

(5 citation statements)

References 21 publications

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“…The fundamental role of RHC in the follow-up assessment has long been supported. 17 – 19 Patients with clinical deterioration and poor treatment response may be benefit from prioritizing follow-up RHC to evaluate more precisely. Besides, the prognostic differences between thorough assessments, including RHC and noninvasive assessments, in patients with SLE-associated PAH need to be further studied.…”

Section: Discussionmentioning

confidence: 99%

Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Wang

Qian

et al. 2022

Therapeutic Advances in Chronic Disease

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Objective: This study evaluated the prognostic value of the multivariable risk assessment for systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH). Methods: A multicenter prospective cohort of SLE-associated PAH (CSTAR-PAH cohort) diagnosed based on right heart catheterization (RHC) was established. Baseline and follow-up records were collected. Three methods of risk assessment, including (1) the number of low-risk criteria, based on World Health Organization functional class (WHO FC), 6-min walking distance (6MWD), right atrial pressure (RAP), and cardiac index (CI); (2) the three-strata stratification based on the average risk score of four variables (WHO FC, 6MWD, RAP, and CI); and (3) the four-strata stratification based on COMPARE 2.0 model were applied. A risk-assessment method using three noninvasive low-risk criteria was applied at the first follow-up visit. Survival curves between patients with different risk groups were compared by Kaplan–Meier’s estimation and log-rank test. Results: Three-hundred and ten patients were enrolled from 14 PAH centers. All methods of stratification at baseline and first follow-up significantly discriminated long-term survival. Survival rates were also significantly different based on the noninvasive risk assessment in first follow-up visit. Survival deteriorated with the escalation of risk from baseline to first follow-up. Patients with baseline serositis had a higher rate of risk improvement in their follow-up. Conclusion: The risk assessment has a significant prognostic value at both the baseline and first follow-up assessment of SLE-associated PAH. A noninvasive risk assessment can also be useful when RHC is not available during follow-up. Baseline serositis may be a predictor of good treatment response in patients with SLE-associated PAH.

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Section: Discussionmentioning

confidence: 99%

Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Wang

Qian

et al. 2022

Therapeutic Advances in Chronic Disease

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“… 15 Integrating both pulmonary and systemic circulation into one metric, using the ratio of PVRI/SVRI or mPAP/mSAP has the advantage of being less susceptible to changes in CO, and makes up for some of the deficiencies of PAP and PVR. 15 None of these hemodynamic variables were included in the guidelines on the risk stratification of adults with PAH. 5 , 16 However, mPAP/mSAP and PVRI were included as risk stratification variables in the consensus statement on pediatric PH.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, PVR remains a more reliable measure of pulmonary vascular disease than PAP 15 . Integrating both pulmonary and systemic circulation into one metric, using the ratio of PVRI/SVRI or mPAP/mSAP has the advantage of being less susceptible to changes in CO, and makes up for some of the deficiencies of PAP and PVR 15 . None of these hemodynamic variables were included in the guidelines on the risk stratification of adults with PAH 5,16 .…”

Section: Discussionmentioning

confidence: 99%

“…When right ventricular function is severely impaired, the relation between PAP and PVR changes. Therefore, PVR remains a more reliable measure of pulmonary vascular disease than PAP 15 . Integrating both pulmonary and systemic circulation into one metric, using the ratio of PVRI/SVRI or mPAP/mSAP has the advantage of being less susceptible to changes in CO, and makes up for some of the deficiencies of PAP and PVR 15 .…”

Section: Discussionmentioning

confidence: 99%

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Long‐term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension

Zhang

Dimopoulos

et al. 2022

Pulm. circ.

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To assess the long‐term prognostic value of cardiac catheterization and acute vasodilator testing (AVT) with inhaled iloprost in children with idiopathic pulmonary arterial hypertension (IPAH). Data on 81 consecutive children with IPAH referred to our center who underwent cardiac catheterization and AVT between June 2008 and August 2019 were collected. The correlation between the invasive hemodynamic data and transplant‐free survival was analyzed. Twenty‐four patients died and 1 underwent lung transplantation during a median follow‐up of 3.8 years, with a 5‐year transplant‐free survival rate of 64.9%. Univariate analysis showed that predictors associated with improved survival included a lower pulmonary vascular resistance index (PVRI), PVRI/systemic vascular resistance index (SVRI), mean pulmonary arterial pressure (mPAP)/mean systemic arterial pressure, mean right atrial pressure, and a higher cardiac index (CI), mixed systemic venous oxygen saturations (SvO 2 ), and acute vasodilator response (AVR) according to the Barst criteria (decrease in mPAP and PVRI/SVRI ratio of >20% without a decrease in CI). In multivariate Cox regression analysis, Barst AVR and SvO 2 were independently related to transplant‐free survival. Multiple hemodynamic variables from cardiac catheterization and AVT with inhaled iloprost have important prognostic value for long‐term survival in children with IPAH, of which pulmonary vasoreactivity defined by the Barst criteria and SvO 2 are independent prognostic factors.

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“…Thus, the exhibited ventricular interdependence of mean pressure is consistent with clinical and physiologic observations. One may counter that for PAH patients, while there is evidence of left-sided dysfunction (68), the literature does not indicate that there is a clear inverse relationship between pulmonary and systemic pressure, with some observations even indicating a weak positive correlation (69,70). Figure 4A indicates that the linear models established a weakly negative relationship between PAPs and SBP.…”

Section: Interconnectedness Of Ventricular Responsesmentioning

confidence: 94%

The sine transform is the sine qua non of the pulmonary and systemic pressure relationship

Doyle

Rayarao

Biederman

2023

Front. Cardiovasc. Med.

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Assessment of therapeutic interventions in patients with pulmonary arterial hypertension (PAH) suffers from several commonly encountered limitations: (1) patient studies are often too small and short-term to provide definitive conclusions, (2) there is a lack of a universal set of metrics to adequately assess therapy and (3) while clinical treatments focus on management of symptoms, there remain many cases of early loss of life in a seemingly arbitrary distribution. Here we provide a unified approach to assess right and left pressure relationships in PAH and pulmonary hypertension (PH) patients by developing linear models informed by the observation of Suga and Sugawa that pressure generation in the ventricle (right or left) approximately follows a single lobe of a sinusoid. We sought to identify a set of cardiovascular variables that either linearly or via a sine transformation related to systolic pulmonary arterial pressure (PAPs) and systemic systolic blood pressure (SBP). Importantly, both right and left cardiovascular variables are included in each linear model. Using non-invasively obtained cardiovascular magnetic resonance (CMR) image metrics the approach was successfully applied to model PAPs in PAH patients with an r2 of 0.89 (p < 0.05) and SBP with an r2 of 0.74 (p < 0.05). Further, the approach clarified the relationships that exist between PAPs and SBP separately for PAH and PH patients, and these relationships were used to distinguish PAH vs. PH patients with good accuracy (68%, p < 0.05). An important feature of the linear models is that they demonstrate that right and left ventricular conditions interact to generate PAPs and SBP in PAH patients, even in the absence of left-sided disease. The models predicted a theoretical right ventricular pulsatile reserve that in PAH patients was shown to be predictive of the 6 min walk distance (r2 = 0.45, p < 0.05). The linear models indicate a physically plausible mode of interaction between right and left ventricles and provides a means of assessing right and left cardiac status as they relate to PAPs and SBP. The linear models have potential to allow assessment of the detailed physiologic effects of therapy in PAH and PH patients and may thus permit cross-over of knowledge between PH and PAH clinical trials.

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Hemodynamic trajectories and outcomes in patients with pulmonary arterial hypertension

Cited by 4 publications

References 21 publications

Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Long‐term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension

The sine transform is the sine qua non of the pulmonary and systemic pressure relationship

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