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Introduction: Pulmonary hypertension (PH) is prevalent in those with end-stage kidney disease (ESKD) and poses a barrier to kidney transplant due to its association with poor outcomes. Studies examining these adverse outcomes are limited and often utilize echocardiographic measurements of pulmonary artery systolic pressure (PASP) instead of the gold standard right heart catheterization (RHC). We hypothesized that in ESKD patients deemed ineligible for kidney transplant because of an echocardiographic diagnosis of PH the predominant cause of PH is hypervolemia and is potentially reversible., Methods: We conducted a prospective study of 16 patients with ESKD who were denied transplant candidacy. Prior echocardiograms and RHCs were reviewed for confirmation of PH. Patients were admitted for daily sessions of ultrafiltration for volume removal and repeat RHCs were performed following intervention. RHC parameters and body weight were compared before and after intervention. Statistical analysis was performed using PRISM GraphPad software. A p-value <.05 was considered statistically significant., Results: Following intervention, the mean pulmonary artery pressure (mPAP) and pulmonary arterial wedge pressure decreased from 45.0 ± 3.06 to 29.1 ± 7.77 mmHg (p < .0001) and 22.2 ± 5.06 to 13.1 ± 7.25 mmHg (p = .003), respectively. The pulmonary vascular resistance decreased from 4.73 ± 1.99 to 4.28 ± 2.07 WU (p = .30). Eleven patients from the initial cohort underwent successful kidney transplantation post-intervention with 100% survival at 1-year., Conclusions: In ESKD patients, diagnoses of PH made by echocardiography may be largely due to hypervolemia and may be optimized using an intensive ultrafiltration strategy to restore transplant candidacy., (© 2022 The Authors. Clinical Transplantation published by John Wiley & Sons Ltd.)

Purpose of Review: Left heart disease is the most common cause of pulmonary hypertension. This review summarizes the current care of patients with pulmonary hypertension caused by left heart disease (PH-LHD) and discusses recent and active clinical trials in this patient population., Recent Findings: The primary focus of interventions aimed at treating PH-LHD address the treatment of left heart disease. Significant advancements in the treatment of heart failure with preserved ejection fraction (HFpEF), a frequent cause of PH-LHD, are supported in the current literature. Patients with residual pulmonary hypertension despite optimal treatment of left heart disease have poor outcomes. Yet, interventions targeting the pulmonary vasculature in PH-LHD patients have not demonstrated significant benefits in studies to date. Current work focuses on differentiating isolated postcapillary pulmonary hypertension (IpcPH) from combined precapillary and postcapillary pulmonary hypertension (CpcPH) in a clinically consistent manner. It is hopeful that thorough phenotyping of PH-LHD patients will translate into effective treatment strategies addressing pulmonary vascular disease., Summary: Referral to centers of excellence, considerations for enrollment in clinical trials, and evaluation for transplant is recommended for patients with residual pulmonary hypertension despite optimal treatment of left heart disease, particularly those with CpcPH., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Pulmonary artery (PA) pulsitility index (PAPi) is a novel haemodynamic index shown to predict right ventricular failure in acute inferior myocardial infarction and post left ventricular assist device surgery. We hypothesised that PAPi calculated as [PA systolic pressure - PA diastolic pressure]/right atrial pressure (RAP) would be associated with mortality in the National Institutes of Health Registry for Primary Pulmonary Hypertension (NIH-RPPH)., Methods: The impact of PAPi, the Pulmonary Hypertension Connection (PHC) risk score, right ventricular stroke work, pulmonary artery capacitance (PAC), other haemodynamic indices, and demographic characteristics was evaluated in 272 NIH-RPPH patients using multivariable Cox proportional hazards (CPH) regression and receiver operating characteristic (ROC) analysis., Results: In the 272 patients (median age 37.7+/-15.9years, 63% female), the median PAPi was 5.8 (IQR 3.7-9.2). During 5years of follow-up, 51.8% of the patients died. Survival was markedly lower (32.8% during the first 3years) in PAPi quartile 1 compared with the remaining patients (58.5% over 3years in quartiles 2-4; p<0.0001). The best multivariable CPH survival model included PAPi, the PHC-Risk score, PAC, and body mass index (BMI). In this model, the adjusted hazard ratio for death with increasing PAPi was 0.946 (95% CI 0.905-0.989). The independent ROC areas for 5-year survival based on bivariable logistic regression for PAPi, BMI, PHC Risk, and PAC were 0.63, 0.62, 0.64, and 0.65, respectively (p<0.01). The ROC area for 5-year survival for the multivariable logistic model with all four covariates was 0.77 (p<0.0001)., Conclusions: Pulmonary artery pulsatility index was independently associated with survival in PAH, highlighting the utility of PAPi in combination with other key measures for risk stratification in this population., (Copyright © 2018 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objectives: Pulmonary hypertension portends worse outcomes in cardiac valve surgery; however, isolated pulmonary artery pressures may not reflect patients' global cardiac function accurately. To better account for the interventricular relationship, the authors hypothesized that patients with greater pulmonary-systemic ratios (mean pulmonary arterial pressure)/(mean systemic arterial pressure) would correlate with worse outcomes after valve surgery., Design: Retrospective cohort study., Setting: Single academic hospital., Participants: The study comprised 314 patients undergoing valve surgery with or without coronary artery bypass grafting (2004-2016) with Society of Thoracic Surgeons predicted risk scores and preoperative right heart catheterization., Interventions: None., Measurements and Main Results: The pulmonary-systemic ratio was calculated as follows: mean pulmonary arterial pressure/mean systemic arterial pressure. Patients were stratified by pulmonary-systemic ratio quartile. Logistic regression was used to assess the risk-adjusted association between pulmonary-systemic ratio or mean pulmonary arterial pressure. Median pulmonary-systemic ratio was 0.33 (Q1-Q3: 0.23-0.65); median pulmonary arterial pressure was 29 (21-30) mmHg. Patients with the highest pulmonary-systemic ratio had the highest rates of morbidity and mortality (p < 0.0001). A high pulmonary-systemic ratio was associated with longer duration in the intensive care unit (p < 0.0001) and hospital (p < 0.0001). After risk-adjustment, pulmonary-systemic ratio and pulmonary arterial pressure were independently associated with morbidity and mortality, but the pulmonary-systemic ratio (odds ratio 23.88, p = 0.008, Wald 7.1) was more strongly associated than the pulmonary arterial pressure (odds ratio 1.035, p = 0.011, Wald 6.5)., Conclusions: The pulmonary-systemic ratio is more strongly associated with risk-adjusted morbidity and mortality in valve surgery than pulmonary arterial pressure. By integrating ventricular interactions, this metric may better characterize the risk of valve surgery., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Pulmonary hypertension (PH) is characterized by increased pulmonary vascular resistance leading to right heart failure. Elevated right atrial (RA) pressure reflects right ventricular (RV) pressure overload and is an established risk factor for mortality in PH. We hypothesized that PH patients with an increased ratio of RA to LA volume index (RAVI/LAVI), would have increased mortality., Methods: We evaluated the association of RAVI/LAVI with mortality in 124 patients seen at a single academic center's PH clinic after adjusting for the REVEAL risk score, an established risk score in PH. LA and RA volume indices were measured in the four-and two-chamber views by two independent researchers. Multivariable logistic regression was used to model the independent association of RAVI/LAVI with survival., Results: Among 124 patients (mean age 62 ± 12.7 years, 68.6% female), each unit increase in RAVI/LAVI was associated with a nearly twofold increase in mortality (OR: 1.91, 95% CI: 1.20-3.04). In a multivariable logistic regression, each unit increase in RAVI/LAVI was associated with a nearly twofold increase in mortality (OR: 1.73, 95% CI: 1.003-2.998). Furthermore, RAVI/LAVI in the highest quartile (>1.42) was significantly associated with elevated right atrial pressure (RAP) to pulmonary artery wedge pressure ratio (RAP/PAWP) (0.76 ± 0.41, P = 0.02) compared with the lowest quartile (<0.77), suggesting an interaction between invasive hemodynamic data, atrial structural changes, and mortality in PH., Conclusions: Increased RAVI/LAVI in PH is associated with decreased survival and accounts for atrial structural remodeling related to invasive hemodynamics. These findings support further study of this index in predicting outcomes in PH., (© 2018 Wiley Periodicals, Inc.)

Background: This study evaluated the utility of a novel index, pulmonary arterial (PA) proportional pulse pressure (PAPP; range 0-1, defined as [PA systolic pressure - PA diastolic pressure] / PA systolic pressure), in predicting mortality in patients with World Health Organization group 1 pulmonary hypertension (PH)., Hypothesis: Low PAPP is associated with increased 5-year mortality independent of a validated contemporary risk-prediction equation (Pulmonary Hypertension Connection [PHC] equation)., Methods: In a group of 262 patients in the National Institutes of Health Primary Pulmonary Hypertension (NIH-PPH) Registry, PAPP and the PHC risk equation were used to predict mortality during 5 years of follow-up using Cox proportional hazards models. Kaplan-Meier survival curves were used to compare mortality among PAPP quartiles, and significance was tested using the log-rank test., Results: Patients in the lowest quartile (PAPP ≤0.47) had a significantly higher 5-year mortality than did patients in higher quartiles (log-rank P = 0.016). In a Cox model adjusted for the PHC equation, PAPP remained significantly associated with 5-year mortality (hazard ratio: 0.74 per 0.10 increase in PAPP, 95% confidence interval: 0.61-0.90). The χ 2 statistic for the single PAPP covariate in this model was 8.8 (P = 0.003), which compared favorably with the χ 2 statistic of 15.2 (P < 0.0001) for the multivariable PHC equation., Conclusions: PAPP, an index of ventricular-arterial coupling, is independently associated with survival in World Health Organization group 1 PH. The use of this easily measurable index for guiding risk stratification needs further investigation., (© 2017 Wiley Periodicals, Inc.)

Background: Diastolic pulmonary gradient (DPG), calculated as the difference between pulmonary artery diastolic pressure and mean pulmonary capillary wedge pressure ≥ 7 mmHg is associated with pulmonary vascular disease and portends poor prognosis in heart failure (HF). The prognostic relevance of DPG in group 1 pulmonary hypertension (PH) is uncertain., Methods: Using the Pulmonary Hypertension Connection (PHC) risk equation for 225 patients in the NIH-PPH, the 5-year probability of death was calculated, which was then compared with DPG using a Cox proportional hazards model. Kaplan-Meier survival curves were determined for two cohorts using the median DPG of 30 mmHg as cutoff, and significance was tested using the log-rank test., Results: The mean age was 38.1 ± 16.0 years old, 63% female, and 72% were "white". The mean DPG was 31.6 mmHg ± 13.8 mm Hg and only 1.8% had a DPG <7 mm Hg. Increasing DPG was significantly associated with increased 5-year mortality even after adjustment for the PHC risk equation (HR 1.29 per 10 mm Hg increase). When DPG was dichotomized based on the median of 30 mm Hg, the HR for DPG >30 mm Hg with respect to 5-year mortality was 2.03. After adjustment for pulmonary artery systolic pressure (PASP), increasing DPG remained significantly associated with decreased 5 years survival (HR 1.99 for DPG > 30 mm Hg)., Conclusions: DPG is independently associated with survival in group 1 PH patients even after adjustment for the PHC risk equation or PASP. Patients with increased DPG had a 2-fold increased risk of mortality. The use of DPG for guiding treatment and prognosis in group 1 PH should be further investigated., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Background: Assessment of diffuse right ventricular (RV) fibrosis is of particular interest in pulmonary hypertension (PH) and heart failure (HF). Current cardiovascular magnetic resonance (CMR) T1 mapping techniques such as Modified Look-Locker inversion recovery (MOLLI) imaging have limited resolution, but accelerated and navigator-gated Look-Locker imaging for cardiac T1 estimation (ANGIE) is a novel CMR sequence with spatial resolution suitable for T1 mapping of the RV. We tested the hypothesis that patients with PH would have significantly more RV fibrosis detected with MRI ANGIE compared with normal volunteers and patients having HF with reduced (LV) ejection fraction (HFrEF) without co-existing PH, independent of RV dilitation and dysfunction., Methods: Patients with World Health Organization group 1 or group 4 PH, patients with HFrEF without PH, and normal volunteers were recruited to undergo contrast-enhanced CMR. RV and LV extracellular volume fractions (RV-ECV and LV-ECV) were determined using pre-contrast and post-contrast T1 mapping using ANGIE (RV and LV) and MOLLI (LV only)., Results: Thirty-two participants (53.1% female, median age 52 years, IQR 26-65 years) were enrolled, including n = 12 with PH, n = 10 having HFrEF without co-existing PH, and n = 10 normal volunteers. ANGIE ECV imaging was of high quality, and ANGIE measurements of LV-ECV were highly correlated with those of MOLLI (r = 0.91; p < 0.001). The RV-ECV in PH patients was 27.2% greater than the RV-ECV in normal volunteers (0.341 v. 0.268; p < 0.0001) and 18.9% greater than the RV-ECV in HFrEF patients without PH (0.341 v. 0.287; p < 0.0001). RV-ECV was greater than LV-ECV in PH (RV-LV difference = 0.04), but RV-ECV was nearly equivalent to LV-ECV in normal volunteers (RV-LV difference = 0.002) (p < 0.0001 for RV-LV difference in PH versus normal volunteers). RV-ECV was linearly associated with both increasing RVEDVI (p = 0.049) and decreasing RVEF (p = 0.04) in a multivariable linear model, but PH was still associated with greater RV-ECV even after adjustment for RVEDVI and RVEF., Conclusions: Pre- and post-contrast ANGIE imaging provides high-resolution ECV determination for the RV. PH is independently associated with increased RV-ECV even after adjustment for RV dilatation and dysfunction, consistent with an independent effect of PH on fibrosis. ANGIE RV imaging merits further clinical evaluation in PH.

Objective: This study assessed the impact of pulmonary hypertension (PH) on morbidity and mortality after the most common cardiac operations and evaluated the accuracy of the Society of Thoracic Surgeons (STS) risk model for patients with PH., Methods: At a single center between 1994 and 2010, all adult cardiac operations performed with recorded preoperative mean pulmonary arterial pressure (MPAP) and STS predicted mortality were reviewed. MPAP was defined as normal (<25 mm Hg) or as mild (25-34 mm Hg), moderate (35-44 mm Hg), or severe (≥ 45 mm Hg) PH. Multivariate analysis was performed to elucidate the contribution of PH to morbidity and mortality., Results: In all, 3343 patient records were reviewed. Coronary artery bypass grafting (CABG) was the most common procedure (67.5%), followed by aortic valve replacement (24.9%) and mitral valve procedures (6.3%). Postoperative complications and mortality increased with increasing MPAP. Multivariable analysis found that both moderate (odds ratio, 7.17; P < .001) and severe (odds ratio, 13.73; P < .001) PH were significantly associated with increased mortality, even after accounting for STS risk. A subset analysis of isolated CABG cases revealed markedly increased mortality for all categories of PH (mild odds ratio, 1.99; moderate odds ratio, 11.5; severe odds ratio, 38.9; P < .001)., Conclusions: Morbidity and mortality were independently associated with PH. Observed mortality was significantly higher than predicted by the STS model for patients with moderate and severe PH, particularly in isolated CABG. Addition of PH to the STS risk model should be considered, or alternative tools should be used to assess risk in these patients., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)