Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease

Hemnes, Anna R.; Leopold, Jane A.; Radeva, Milena; Beck, Gerald J.; Abidov, Aiden; Aldred, Micheala A.; Barnard, John; Rosenzweig, Erika B.; Borlaug, Barry A.; Chung, Wendy K.; Comhair, Suzy; Desai, Ankit A.; DuBrock, Hilary M.; Erzurum, Serpil C.; Finet, J. Emanuel; Frantz, Robert P.; Garcia, Joe G.N.; Geraci, Mark W.; Gray, Michael P.; Grünig, Gabriele; Hassoun, Paul M.; Highland, Kristin B.; Hill, Nicholas S.; Hu, Bo; Kwon, Deborah; Jacob, Miriam; Jellis, Christine; Larive, A. Brett; Lempel, Jason K.; Maron, Bradley A.; Mathai, Stephen C.; McCarthy, Kevin; Mehra, Reena; Nawabit, R; Newman, John H.; Olman, Mitchell A.; Park, Margaret M.; Ramos, José A; Renapurkar, Rahul D.; Rischard, Franz; Sherer, Susan G.; Tang, W.H. Wilson; Thomas, James D.; Vanderpool, Rebecca; Waxman, Aaron B.; Wilcox, Jennifer; Yuan, Jason X.‐J.; Horn, Evelyn M.

doi:10.1016/j.jacc.2022.05.038

Cited by 47 publications

(33 citation statements)

References 30 publications

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“…This may not be surprising in lieu of existing literature showing that non‐PAH forms of PH have higher mortality than PAH. 37 , 38 Additionally, subjects in our prospective cohort with PAH were more likely to be taking PAH‐targeted therapy at the time of echocardiography, even when compared to the retrospective subgroup with PAH (Supporting Information: Table 2 ). This higher proportion on PAH‐targeted therapy and having marginally increased age (median 52 vs. 44) may suggest that the prospective cohort was more likely to have established PAH rather than incident or early‐stage disease.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of right ventricular strain in two separate cohorts with precapillary pulmonary hypertension

Crossman

Rajaram²,

Hart

et al. 2023

Pulm. circ.

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Evaluation for right ventricular (RV) dysfunction is an important part of risk assessment in care of patients with pulmonary hypertension (PH) as it is associated with morbidity and mortality. Echocardiography provides a widely available and acceptable method to assess RV function. RV global longitudinal strain (RVGLS), a measure of longitudinal shortening of RV deep muscle fibers obtained by two‐dimensional echocardiography, was previously shown to predict short‐term mortality in patients with PH. The purpose of the current study was to assess the performance of RVGLS in predicting 1‐year outcomes in PH. We retrospectively identified 83 subjects with precapillary PH and then enrolled 50 consecutive prevalent pulmonary arterial hypertension (PAH) subjects into a prospective validation cohort. Death as well as combined morbidity and mortality events at 1 year were assessed as outcomes. In the retrospective cohort, 84% of patients had PAH and the overall 1‐year mortality rate was 16%. Less negative RVGLS was marginally better than tricuspid annular plane systolic excursion (TAPSE) as a predictor for death. However, in the prospective cohort, 1‐year mortality was only 2%, and RVGLS was not predictive of death or a combined morbidity and mortality outcome. This study supports that RV strain and TAPSE have similar 1‐year outcome predictions but highlights that low TAPSE or less negative RV strain measures are often false‐positive in a cohort with low baseline mortality risk. While RV failure is considered the final common pathway for disease progression in PAH, echocardiographic measures of RV function may be less informative of risk in serial follow‐up of treated PAH patients.

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Section: Discussionmentioning

confidence: 99%

Evaluation of right ventricular strain in two separate cohorts with precapillary pulmonary hypertension

Crossman

Rajaram²,

Hart

et al. 2023

Pulm. circ.

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“…We selected a cohort of patients all placed on parenteral treprostinil (± oral therapy) to optimize the possibility of seeing large changes in afterload (and thus, RVFnRec) 17 . Ninety-six healthy control subjects were recruited for the NHLBI Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics (PVDOMICS) 18 . These subjects underwent extensive testing including CMR and non-invasive CPET at a single time point.…”

Section: Methodsmentioning

confidence: 99%

“…. Ninety-six healthy control subjects were recruited for the NHLBI Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics (PVDOMICS)18 . These subjects underwent extensive testing including CMR and noninvasive CPET at a single time point.…”

mentioning

confidence: 99%

Classification and Predictors of Right Ventricular Functional Recovery in Pulmonary Arterial Hypertension

Rischard

Bernardo

Vanderpool

et al. 2023

Preprint

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Background: Normative changes in right ventricular (RV) structure and function have not been characterized in the context of treatment-associated functional recovery (RVFnRec). The aim of this study is to assess the clinical relevance of a proposed RVFnRec definition. Methods: We evaluated 63 incident patients with PAH by right heart catheterization and cardiac MRI (CMR) at diagnosis and CMR and invasive cardiopulmonary exercise (CPET) following treatment (~11 months). Sex, age, race/ethnicity matched healthy control subjects (n=62) with one-time CMR and non-invasive CPET were recruited from the PVDOMICS project. We examined therapeutic CMR changes relative to the evidence-based peak oxygen consumption (VO2peak)>15mL/kg/min to define RVFnRec by receiver operating curve analysis. Afterload was measured in the as mean pulmonary artery pressure, resistance, compliance, and elastance. Results: A drop in RV end-diastolic volume of -15 mL best defined RVFnRec (AUC 0.87, P=0.0001) and neared upper 95% CI RVEDV of controls. 22/63 (35%) of subjects met this cutoff which was reinforced by freedom from clinical worsening, RVFnRec 1/21 (5%) versus no RVFnRec 17/42, 40%, (log rank P=0.006). A therapy-associated increase of 0.8 mL/mmHg in compliance had the best predictive value of RVFnRec (AUC 0.76, CI 0.64-0.88, P=0.001). RVFnRec subjects had greater increases in stroke volume, and cardiac output at exercise. Conclusions: RVFnRec defined by RVEDV therapeutic decrease of -15mL predicts exercise capacity, freedom from clinical worsening, and nears normalization. A therapeutic improvement of compliance is superior to other measures of afterload in predicting RVFnRec. RVFnRec is also associated with increased RV output reserve at exercise.

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“…Details of PVDO-MICS methodology, PVDOMICS Study Group members, core adjudication of hemodynamic measurements, and overall cohort characteristics are as previously reported. [3][4][5] The PVDOMICS study was approved by local institutional review boards and all patients provided written informed consent.…”

Section: Study Cohort and Vasoreactivity Testingmentioning

confidence: 99%

Acute vasoreactivity testing during right heart catheterization in chronic thromboembolic pulmonary hypertension: Results from the pulmonary vascular disease phenomics study

et al. 2023

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Chronic thromboembolic pulmonary hypertension (CTEPH) is believed to involve both vascular obstruction and vasoconstriction; hence, pulmonary vasodilators such as riociguat may be beneficial. Acute vasoreactivity testing (AVT) is seldom performed routinely in CTEPH patients, so there is limited understanding of the frequency and significance of an acute vasodilator response. Systematic vasodilator testing with oxygen (O 2 ) and oxygen plus inhaled nitric oxide (O 2 + iNO) was performed as part of the Pulmonary Vascular Disease Omics (PVDOMICS) NHLBI project, providing an opportunity to examine AVT responses in CTEPH. Patients with CTEPH enrolled in PVDOMICS (n = 49, 40 with prevalent CTEPH [82%]) underwent right heart catheterization including AVT with O 2 and O 2 + iNO. Hemodynamics were obtained at baseline and with each challenge. Fourteen of 49 patients (29%) had >20% drop in pulmonary vascular resistance (PVR) with O 2 . With O 2 + iNO, 30/ 49 (61%) had >20% drop in PVR, 20% had >20% drop in mean pulmonary artery

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Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease

Cited by 47 publications

References 30 publications

Evaluation of right ventricular strain in two separate cohorts with precapillary pulmonary hypertension

Evaluation of right ventricular strain in two separate cohorts with precapillary pulmonary hypertension

Classification and Predictors of Right Ventricular Functional Recovery in Pulmonary Arterial Hypertension

Acute vasoreactivity testing during right heart catheterization in chronic thromboembolic pulmonary hypertension: Results from the pulmonary vascular disease phenomics study

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