Pulmonary Hypertension Association Research Articles

Impact of Pulmonary Hypertension on Outcomes After Transcatheter Tricuspid Valve Edge-to-Edge Repair

BackgroundData regarding the association of pulmonary hypertension (PH) and outcomes in patients undergoing transcatheter tricuspid valve edge-to-edge repair (T-TEER) are scarce. ObjectivesThe aims of this study were 1) to investigate the impact of PH on outcomes after T-TEER; and 2) to shed further light on the role of pre- and postcapillary PH in patients undergoing T-TEER for relevant tricuspid regurgitation (TR). MethodsThe study included patients from the EuroTR (European Registry of Transcatheter Repair for Tricuspid Regurgitation) registry (NCT06307262) who underwent T-TEER for relevant TR from 2016 until 2023 with available invasive evaluation of systolic pulmonary artery pressure (sPAP) using right heart catheterization. Study endpoints were procedural TR reduction, improvement in NYHA function class, and a combined endpoint of death or heart failure hospitalization (HFH) at 2 years. ResultsAmong a total of 1,230 patients (mean age 78.6 ± 7.0 years, 51.4% women), increasing sPAP was independently associated with increasing rates of 2-year death or HFH (HR: 1.027; 95% CI: 1.003-1.052; P = 0.030; median survival follow-up 343 days [Q1-Q3: 114-645 days]). No significant survival differences were observed for patients with pre- vs postcapillary PH. Sensitivity analysis revealed an sPAP value of 46 mm Hg as the optimized threshold for the prediction of death or HFH. Being observed in 526 patients (42.8%), elevated sPAP (>46 mm Hg) was associated with more severe heart failure symptoms at baseline and follow-up. Importantly, NYHA functional class significantly improved and TR severity was significantly reduced irrespective of PH. ConclusionsPH is an important outcome predictor in patients undergoing T-TEER for relevant TR. In contrast to previous studies, no significant differences were observed for patients with pre- and postcapillary PH in terms of survival free from HFH.

Gender-Dependent Association of Pulmonary Hypertension with Adverse Outcomes in Patients Undergoing Transcatheter Aortic Valve Replacement.

The relationship between pulmonary hypertension (PH) and outcomes after transcatheter aortic valve replacement (TAVR) has been shown to be unfavorable. The impact of gender on TAVR outcomes remains controversial. There have been no studies evaluating the simultaneous effects of both factors on TAVR outcomes. We retrospectively analyzed a prospective cohort of patients who underwent TAVR between January 2016 and December 2022. The patients were stratified by gender and the presence of PH. The primary outcome of the study was all-cause mortality. Secondary outcome was a composite of all-cause mortality and heart failure hospitalization. We identified a total of 133 female patients without PH, 179 males without PH, 87 females with PH, and 122 males with PH. The median follow-up period was 18 months. Female patients without PH demonstrated a lower cumulative mortality rate compared to those with male gender and/or PH. Adjusted multivariate Cox proportional hazard analyses revealed that male gender and PH status, either individually or in combination, were independently associated with long-term mortality when compared to female patients without PH. Specifically, females with PH (HR 6.80, 95% confidence interval (CI): 1.49-31.12, P=0.013), males without PH (HR 6.45, 95% CI: 1.47-28.22, P=0.013), and males with PH (HR 7.2, 95% CI: 1.63-31.81, P=0.009) demonstrated significantly higher risk for mortality. Patients who were male or had PH status had a higher risk of mortality. However, there was no synergistic effect between being male and having PH on the prognosis after TAVR.

Association of pulmonary hypertension with outcomes in patients with Systemic sclerosis and other connective tissue disorders: review and meta-analysis.

Pulmonary hypertension (PH) is a frequent complication of connective tissue disorders (CTDs), with a major impact on the prognosis of the disease. The aim of our study was to perform a systemic review and meta-analysis of published literature evaluating survival function in patients with systemic sclerosis (SSc) with and without PH and to compare survival function between patients with SSc, systemic lupus erythematosus (SLE), other CTDs, and conditions associated with PH. The established protocol of the Cochrane Collaboration Steps and meta-analysis of observational studies in epidemiology recommendations (MOOSE) were used. 7 studies, including 1470 SSc-PH patients and 1368 SSc patients without PH, and 4 studies, including 108 SLE-PH patients and 1288 SLE patients without PH, assessed survival function were selected. Six studies (including 777 SSc, 249 SLE, 90 idiopathic pulmonary arterial hypertension (IPAH) and 29 primary Sjogren's syndrome patients) comparing survival function in different subgroups of patients with confirmed PH were included. SSc patients with PH showed the worst survival as compared to SSc patients without PH [OR (95% CI) 3.70 (2.42-5.67); p<0.00001]. The same pattern was observed in patients with SLE. SSc patients with PH were characterized by lower survival function compared to other reasons for PH, including SLE [OR (95% CI) 2.76 (1.95-3.91); p<0.00001]. Patients with SSc-PH are characterized by significantly lower survival function as compared to SSc patients without PH. Among the different entities of PH, SSc shows the worst survival, underlining prognostic significance of detection and evaluation of PH according to the currently established approach.

Association of pulmonary hypertension with the outcome in patients undergoing edge-to-edge mitral valve repair

ObjectivesThe association of pulmonary hypertension (PH) with the outcome after mitral transcatheter edge-to-edge repair (M-TEER) focusing on the new ESC/ERS guidelines definition for PH.BackgroundPH is frequently found in patients with...

Investigating the “sex paradox” in pulmonary arterial hypertension: Results from the Pulmonary Hypertension Association Registry (PHAR)

BackgroundFemale sex is a significant risk factor for pulmonary arterial hypertension (PAH), yet males with PAH have worse survival – a phenomenon referred to as the “sex paradox” in PAH. MethodsAll adult PAH patients in the Pulmonary Hypertension Association Registry (PHAR) with congruent sex and gender were included. Baseline differences in demographics, hemodynamics, functional parameters, and quality of life were assessed by sex. Kaplan-Meier survival analysis was used to evaluate survival by sex. Mediation analysis was conducted with Cox proportional hazards regression by comparing the unadjusted hazard ratios for sex before and after adjustment for covariates. The plausibility of collider-stratification bias was assessed by modeling how large an unmeasured factor would have to be to generate the observed sex-based mortality differences. Subgroup analysis was performed on idiopathic and incident patients. ResultsAmong the 1,891 patients included, 75% were female. Compared to men, women had less favorable hemodynamics, lower six-minute walk distance, more PAH therapies, and worse functional class; however, sex-based differences were less pronounced when accounting for body surface area or expected variability by gender. On multivariate analysis, women had a 48% lower risk of death compared to men (Hazard Ratio 0.52, 95% Confidence interval 0.36 – 0.74, p<0.001). Modeling found that under reasonable assumptions collider-stratification could account for sex-based differences in mortality. ConclusionsIn this large registry of PAH patients new to a care center, men had worse survival than women despite having more favorable baseline characteristics. Collider-stratification bias could account for the observed greater mortality among men.

Pulmonary hypertension patient perspectives towardpulmonary rehabilitation.

Pulmonary rehabilitation (PR) is a supervised exercise program for patients with chronic lung disease. Among patients with pulmonary hypertension (PH), PR has been shown to improve both quality of life and exercise capacity. The purpose of this study was to assess the prevalence of PR participation among PH patients, patient perspectives regarding PR, and to identify potential barriers to PR participation. We performed a cross-sectional survey of patients with self-reported PH who attended the Pulmonary Hypertension Association (PHA) conference in June 2022 in Atlanta, Georgia, and patients within the PHA listserv. A total of 429 participants completed the survey and were enrolled in the study. The average age of participants was 61 ± 14 years with 83% of participants identifying as female, 51% of patients self-reported as having group 1 PH. Among patients who completed the survey, 41% had previously attended a PR program. Of those who had completed a PR program, 83% reported being satisfied or very satisfied with the program and 86% reported that they would recommend PR to other PH patients. After completion of a PR program, 76% of patients reported an improvement in their quality of life and 88% reported improvement in exercise capacity. Among the patients who had not previously participated in PR (n = 254), 63% reported an interest in participation while 64% cited a lack of discussion from their treatment team as the primary reason for the lack of PR participation. Limitations of the study include sampling and response bias. According to this cross-sectional survey, the majority of PH patients who have participated in PR report improvement in both quality of life and exercise capacity and would recommend PR to other PH patients. The majority of PH patients who have not participated in PR were interested in participation and cited a lack of discussion with their treatment team as one of the primary reasons for the lack of participation. PR is associated with self-reported improvements in quality of life and exercise capacity but remains underutilized among patients with PH.

Ortner’s Syndrome With Primary Pulmonary Hypertension - A Case Report

Ortner’s Syndrome or Cardio-vocal syndrome is a manifestation of hoarseness secondary to left recurrent laryngeal nerve (RLN) palsy with abnormal cardiovascular components. The association of pulmonary hypertension with Ortner’s Syndrome has not been widely available in the literature with very few cases reported worldwide.We present a case of a 17 years old lady with underlying Primary Pulmonary Hypertension, who presented with progressive hoarseness over the past 3 years associated with breathlessness and easy fatigue on exertion. Herein we discuss the options of treatment modality for Ortner’s Syndrome.

Abstract 13633: Modern Treatment Patterns in Incident Pulmonary Arterial Hypertension (PAH) Patients: Real-World Data From the Pulmonary Hypertension Association (PHA) Registry

Purpose: New 2022 European Society of Cardiology/European Respiratory Society PAH guidelines recommend treatment guided by a multi-parameter risk assessment. 1 Patients with low/intermediate risk without cardiopulmonary comorbidities should start dual PAH combination therapy; those with cardiopulmonary comorbidities should be reassessed early for rapid escalation to dual therapy. Initial triple therapy is recommended for high-risk patients. We examined use of dual and triple PAH therapy in the US (before guideline update). Methods: We used the Pulmonary Hypertension Association Registry (PHAR), a prospective US registry of pulmonary hypertension (PH) patients, enrolled within 6 months of their first outpatient visit at one of 54 participating PH care centers in the US, between Sep 2015-Jun 2022. We included adults (≥18 years at enrollment) with incident PAH (newly diagnosed and treated within 6 months of enrollment) and ≥1 follow-up clinic visit post baseline visit. Chronic thromboembolic PH patients were excluded. Results: We analyzed (descriptive statistics) 794 incident patients (baseline characteristics in Table 1). Treatment patterns at baseline (n=794)/last follow-up visit (n=610) were 289 (36.4%)/102 (16.7%) on monotherapy, 419 (52.8%)/314 (51.5%) on dual combination, 83 (10.5%)/179 (29.3%) on triple combination therapy, respectively. Mean time from enrollment to dual and triple combination therapy was 86.1 (standard deviation [SD]: 209.0) and 346.1 days (SD: 430.8), respectively. Conclusions: Among incident PHAR patients, half received initial dual and 10% triple upfront combination therapy. At last follow-up, most (&gt;80%) patients had escalated to dual/triple combination therapies. Clinical Implications: At PH care centers, most incident PHAR patients are escalated to dual and triple therapy (at last follow-up) per current guidelines. Reference 1. Humbert M, et al. Eur Heart J. 2022;43:3618-731.

Association and risk factors of pediatric pulmonary hypertension with obstructive sleep apnea: A national study utilizing the Kids' Inpatient Database (KID)

Study objectiveAssess the prevalence of and risk factors for pediatric pulmonary hypertension (PH) in the 2016 Kids' Inpatient Database (KID), including obstructive sleep apnea (OSA) and obesity. MethodsRetrospective cross-sectional cohort study utilizing 6,081,132 weighted pediatric discharges from the 2016 KID. Study variables included age, length of stay, mortality, gender, hospital region, primary payer, race, median household income for patient's ZIP code, OSA, central sleep apnea (CSA), obesity, Down syndrome, sickle cell disease (SCD), thalassemia, congenital heart disease (CHD), hypertension, asthma and chronic lung disease of prematurity (CLDP). PH was the primary outcome of interest. Bivariate and multivariable logistic regression models were utilized with odds ratios and 95 % confidence intervals. ResultsThe mean age was 3.76 years, the mean hospital length of stay was 3.85 days, 48.9 % were male, 52.6 % had government health insurance, 51.0 % were White, 16.1 % were Black, 21.1 % were Hispanic, 5.0 % were Asian or Pacific Islander, 0.80 % were Native American and 6.1 % identified as “other”. The prevalence of PH was 0.21 % (12,777 patients). There were 37,631 patients with OSA and the prevalence of PH among this cohort was 3.3 %, over 10x greater than the overall prevalence of PH in the 2016 KID (0.21 %). Risk factors associated with PH included CLDP, CHD, Down syndrome, asthma, OSA, CSA, hypertension, SCD, obesity, race/ethnicity, government insurance, age, male gender (p < 0.0001), and hospital region (p = 0.0002). ConclusionsSeveral risk factors were independently associated with PH, such as OSA, CSA, obesity, asthma, and insurance status. Prospective multi-institutional studies are needed to assess the relationships between these risk factors, severity metrics, and causative links in the development of PH; in addition to identifying children with OSA who are most likely to benefit from cardiopulmonary screening prior to adenotonsillectomy. Level of evidenceLevel III.

The association of Pulmonary Hypertension and right ventricular systolic function – updates in diagnosis and treatment

Right ventricular (RV) systolic function is an essential but neglected component in cardiac evaluation, and its importance to the contribution to overall cardiac function is undermined. It is not only sensitive to the effect of left heart valve disease but is also more sensitive to changes in pressure overload than the left ventricle. Pulmonary Hypertension is the common and well-recognized complication of RV systolic dysfunction. It is also the leading cause of pulmonary valve disease and right ventricular dysfunction. Patients with a high pulmonary artery pressure (PAP) and a low RV ejection fraction have a seven-fold higher risk of death than heart failure patients with a normal PAP and RV ejection fraction. Furthermore, it is an independent predictor of survival in these patients. In this review, we examine the association of right ventricular systolic function with Pulmonary Hypertension by focusing on various pathological and clinical manifestations while assessing their impact. We also explore new 2022 ESC/ERS guidelines for diagnosing and treating right ventricular dysfunction in Pulmonary Hypertension.

Regional Variation in Pulmonary Arterial Hypertension in the United States: The Pulmonary Hypertension Association Registry.

Rationale: Pulmonary arterial hypertension (PAH) is a heterogeneous disease within a complex diagnostic and treatment environment. Other complex heart and lung diseases have substantial regional variation in characteristics and outcomes; however, this has not been previously described in PAH. Objectives: To identify baseline differences between U.S. census regions in the characteristics and outcomes for participants in the Pulmonary Hypertension Association Registry (PHAR). Methods: Adults with PAH were divided into regional groups (Northeast, South, Midwest, and West), and baseline differences between census regions were presented. Kaplan-Meier survival analyses and Cox proportional hazards were used to estimate the association between region and mortality in unadjusted and adjusted models. Results: Substantial differences by census regions were seen in age, race, ethnicity, marital status, employment, insurance payor breakdown, active smoking, and current alcohol use. Differences were also seen in PAH etiology and baseline 6-minute walk distance test results. Treatment characteristics varied by census region, and mortality appeared to be lower in PHAR participants in the West (hazard ratio, 0.60; 95% confidence interval, 0.43-0.83, P = 0.005). This difference was not readily explained by differences in demographic characteristics, PAH etiology, baseline severity, baseline medication regimen, or disease prevalence. Conclusions: The present study suggests significant regional variation among participants at accredited pulmonary vascular disease centers in multiple baseline characteristics and mortality. This variation may have implications for clinical research planning and represent an important focus for further study to better understand whether there are remediable care aspects that can be addressed in the pursuit of providing equitable care in the United States.

0834 Association of Sleep Disordered Breathing and Hemodynamics of World Symposium on Pulmonary Hypertension Groups in PVDOMICS Cohort

Abstract Introduction As sleep disordered breathing(SDB)-related pathophysiology of intermittent hypoxia, autonomic dysfunction and intrathoracic pressure alterations, etc. likely exerts differential influences across World Symposium on Pulmonary Hypertension(WSPH) groups that have yet to be characterized , we postulate differing SDB-pulmonary hemodynamics relationships across these groups. Methods Pulmonary Vascular Disease Phenomics(PVDOMICS-NCT02980887), a multicenter cohort study, included patients with mean pulmonary artery pressure(mPAP) on right heart catheterization(RHC) ≥ 25mmHg and home sleep apnea testing(HSAT) within 6 weeks of RHC. SDB measures included apnea-hypopnea index(AHI, 3% desaturation hypopnea definition used) and % recording time&amp;lt; 90% SpO2(TRT&amp;lt; 90%). Pulmonary hemodynamic indices included:mPAP, mean pulmonary capillary wedge pressure(PCWP), right atrial mean pressure(RAP). Linear regression models(beta coefficients±standard error) were constructed to assess sleep indices and RHC measurements with adjustment for age, sex, race, body-mass index(BMI), PH medication and supplemental oxygen use; PH group interaction was analyzed. Results We included 424 participants with available data in the final analytic sample. Groups 2 and 3 were older(65.6±11.9, 64.2±10.8 years, respectively) and Group 5 had the highest % males(71.4%). Group 2 had highest BMI: 34.3±9.2kg/m2 and Group 5 lowest: 28.7±6.4k g/m2. SDB(AHI≥5), was most prevalent in Group 2(71.4%) and least in Group 4(42.9%). TRT&amp;lt; 90% was highest in Group 1(37.0%, [P25=2.2, P75=87.3]) followed by Group 4(35.7% [5.3,82.9]) and lowest in Group 2(6.4% [0.61,41.8]). AHI and RAP association differed across groups, p=0.027 and was strongest in Group 2; per 5-unit increase in AHI, RAP increased by 0.75mmHg (estimate=0.75, standard error[0.15,1.36],p=0.015). TRT&amp;lt; 90% and mPAP had significant cross-group differences, p=0.004. TRT&amp;lt; 90% was associated with mPAP in Groups 1 and 4:per 5-unit increase in TRT&amp;lt; 90%, mPAP increased by 2mmHg(2.10,[1.52,2.68],p&amp;lt; 0.001) and 1.4mmHg(1.40,[0.07,2.73],p=0.039) respectively. Other associations were not significant. Conclusion SDB prevalence differs across WSPH groups and was highest in Group 2 PH. Findings suggest that AHI and TRT&amp;lt; 90%SaO2 differentially contribute to the pathogenesis in PH groups; with AHI associating with RAP, especially in Group 2 PH, and TRT&amp;lt; 90%SaO2 with mPAP in Groups 1 and 4 PH, perhaps via hypoxia-induced pulmonary vasoconstriction and remodeling. Support (if any) Funding: U01 HL125218, U01 HL125205, U01 HL125212, U01 HL125208, U01 HL125175, U01 HL125215, and U01 HL125177 and the Pulmonary Hypertension Association.

A World of Maximalist Medicine: Physician Perspectives on Palliative Care and End-of-life for Patients With Pulmonary Arterial Hypertension.

Physicians who specialize in pulmonary arterial hypertension (PAH) care for patients facing a serious, life-limiting illness. Palliative care is underutilized in patients with PAH, and little is known about how best to provide palliative care to this patient population. Using a qualitative approach, assess physicians' perspectives on barriers and facilitators to the use of palliative care in PAH. Participants were board-certified pulmonologists and cardiologists recruited from the Pulmonary Hypertension Association's list of physician specialists and academic center websites. We performed one-on-one semi-structured interviews that were recorded, transcribed, and analyzed using thematic analysis. Twelve physicians participated in the study, with a median age of 48.5 years and 20.5 years of clinical experience caring for patients with PAH. We identified the following themes and associated barriers and facilitators to effective implementation of palliative care for patients with PAH: a tailored approach to the individual patient; a PAH-specialist-led culture of care; effective collaboration with palliative care clinicians; and limitations imposed by health systems. PAH physicians are open to palliative care for their patients and are willing to partner with palliative care clinicians to implement this effectively and in the right setting. Areas for targeted improvement in enhancing palliative care for patients with PAH exist, especially enhancing collaboration between PAH physicians and palliative care specialists and navigating barriers in health systems.

Impact of the COVID-19 pandemic on chronic disease management and patient reported outcomes in patients with pulmonary hypertension: The Pulmonary Hypertension Association Registry.

To better understand the impact of the COVID-19 pandemic on the care of patients with pulmonary hypertension, we conducted a retrospective cohort study evaluating health insurance status, healthcare access, disease severity, and patient reported outcomes in this population. Using the Pulmonary Hypertension Association Registry (PHAR), we defined and extracted a longitudinal cohort of pulmonary arterial hypertension (PAH) patients from the PHAR's inception in 2015 until March 2022. We used generalized estimating equations to model the impact of the COVID-19 pandemic on patient outcomes, adjusting for demographic confounders. We assessed whether insurance status modified these effects via covariate interactions. PAH patients were more likely to be on publicly-sponsored insurance during the COVID-19 pandemic compared with prior, and did not experience statistically significant delays in access to medications, increased emergency room visits or nights in the hospital, or worsening of mental health metrics. Patients on publicly-sponsored insurance had higher healthcare utilization and worse objective measures of disease severity compared with privately insured individuals irrespective of the COVID-19 pandemic. The relatively small impact of the COVID-19 pandemic on pulmonary hypertension-related outcomes was unexpected but may be due to pre-established access to high quality care at pulmonary hypertension comprehensive care centers. Irrespective of the COVID-19 pandemic, patients who were on publicly-sponsored insurance seemed to do worse, consistent with prior studies highlighting outcomes in this population. We speculate that previously established care relationships may lessen the impact of an acute event, such as a pandemic, on patients with chronic illness.

(115) Female Sex is Associated with Worse Functional Impairment and Health-Related Quality of Life In Pulmonary Arterial Hypertension: Baseline Results from the Pulmonary Hypertension Association Registry (PHAR)

(115) Female Sex is Associated with Worse Functional Impairment and Health-Related Quality of Life In Pulmonary Arterial Hypertension: Baseline Results from the Pulmonary Hypertension Association Registry (PHAR)

Pulmonary artery compliance in different forms of pulmonary hypertension

ObjectivePulmonary artery compliance (PAC), estimated as stroke volume (SV) divided by pulmonary artery pulse pressure (PP), may be a predictor of survival in pulmonary arterial hypertension (PAH). Resistance–compliance (RC) time,...

Minimal Clinically Important Difference in the 6-minute-walk Distance for Patients with Pulmonary Arterial Hypertension.

Rationale: The 6-minute-walk distance (6MWD) is an important clinical and research metric in pulmonary arterial hypertension (PAH); however, there is no consensus about what minimal change in 6MWD is clinically significant. Objectives: We aimed to determine the minimal clinically important difference in the 6MWD. Methods: We performed a meta-analysis using individual participant data from eight randomized clinical trials of therapy for PAH submitted to the U.S. Food and Drug Administration to derive minimal clinically important differences in the 6MWD. The estimates were externally validated using the Pulmonary Hypertension Association Registry. We anchored the change in 6MWD to the change in the Medical Outcomes Survey Short Form physical component score. Measurements and Main Results: The derivation (clinical trial) and validation (Pulmonary Hypertension Association Registry) samples were comprised of 2,404 and 537 adult patients with PAH, respectively. The mean ± standard deviation age of the derivation sample was 50.5 ± 15.2 years, and 1,849 (77%) were female, similar to the validation sample. The minimal clinically important difference in the derivation sample was 33 meters (95% confidence interval, 27-38), which was almost identical to that in the validation sample (36 m [95% confidence interval, 29-43]). The minimal clinically important difference did not differ by age, sex, race, pulmonary hypertension etiology, body mass index, use of background therapy, or World Health Organization functional class. Conclusions: We estimated a 6MWD minimal clinically important difference of approximately 33 meters for adults with PAH. Our findings can be applied to the design of clinical trials of therapies for PAH.

Graded Epidural Anaesthesia with Low-Dose Phenylephrine Infusion for Management of Caesarean Section Complicated with Severe Mitral Stenosis: A Case Report

A pregnant patient with a heart condition poses a unique challenge for the obstetrician and anaesthesiologist. Understanding the physiology of pregnancy and the pathophysiology of primary heart disease is important when providing anaesthesia for these highrisk patients during childbirth. A 32-year-old primigravida at 35 weeks and two days of gestation was diagnosed with Rheumatic Heart Disease (RHD), severe Mitral Stenosis (MS), moderate Mitral Regurgitation (MR), mild Atrial Regurgitation (AR), moderate Tricuspid Regurgitation (TR), Pulmonary Arterial Hypertension (PAH), Atrial Fibrillation (AF), and Grade-II New York Heart Association (NYHA). The patient underwent an Elective Lower Segment Cesarean Section (LSCS) performed under epidural anaesthesia, and complications were satisfactorily managed.

Pulmonary Hypertension Association's 2022 International Conference Scientific Sessions Overview.

The considerable progress made in recent years in the diagnosis, risk stratification, and treatment of pulmonary hypertension was highlighted during the most recent edition of the Pulmonary Hypertension Association Scientific Sessions, which was held in Atlanta, Georgia from June 9 to 11, 2022, with the theme: Vision for the PHuture: The Evolving Science and Management of PH. Content presented over the 3-day conference focused on scientific and management updates since the last sessions were held in 2018 and included didactic talks, debates, and roundtable discussions across a broad spectrum of topics related to pulmonary hypertension. This article aims to summarize the key messages from each of the sessiontalks.

Anticoagulation in pulmonary arterial hypertension - association with mortality, healthcare utilization, and quality of life: The Pulmonary Hypertension Association Registry (PHAR).

Routine long-term anticoagulation in pulmonary arterial hypertension (PAH) is controversial. To date, anticoagulation has been found to be beneficial or neutral in idiopathic disease (IPAH) and neutral-to-harmful in connective tissue disease (CTD-PAH). We sought to examine the association between anticoagulation and mortality, healthcare utilization, and quality of life (QoL) in PAH. The PHAR is a prospective registry of PAH patients referred to 58 pulmonary hypertension care centers in the United States. We compared patients who received anticoagulation during enrollment (questionnaire documented) to those who did not. Cox proportional hazard models were used for mortality, Poisson multivariate regression models for healthcare utilization, and generalized estimating equations for QOL RESULTS: Of 1175 patients included, 316 patients were treated with anticoagulation. IPAH/hereditary PAH (HPAH) comprised 46% of the cohort and CTD-PAH comprised 33%. After adjustment for demographics, clinical characteristics, site and disease severity, anticoagulation was not associated with mortality in the overall population (HR, 1.00; 95% CI, 0.72-1.36), IPAH/HPAH (HR, 1.19; 95% CI, 0.74-1.94), or CTD-PAH (HR 0.87; 95% CI, 0.53-1.42). Anticoagulation was associated with an increased rate of emergency department visits (IRR: 1.41), hospitalizations (IRR: 1.30), and hospital days (IRR 1.33). QOL measured by emPHasis-10 score was worse in patients receiving anticoagulation (mean difference 1.74; 95% CI 0.40-3.09). Anticoagulation is not associated with higher mortality, but is associated with increased healthcare utilization in the PHAR. PAH-specific QoL may be worse in patients receiving anticoagulation. The risks and benefits surrounding routine prescription of anticoagulation for PAH should be carefully considered.