Pulmonary Hypertension Association Research Articles

Health-related quality of life and hospitalizations in chronic thromboembolic pulmonary hypertension versus idiopathic pulmonary arterial hypertension: an analysis from the Pulmonary Hypertension Association Registry (PHAR).

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, morbid, potentially curable subtype of pulmonary hypertension that negatively impacts health-related quality of life (HRQoL). Little is known about differences in HRQoL and hospitalization between CTEPH patients and idiopathic pulmonary arterial hypertension (IPAH) patients. Using multivariable linear regression and mixed effects models, we examined differences in HRQoL assessed by emPHasis-10 (E10) and SF-12 between CTEPH and IPAH patients in the Pulmonary Hypertension Association Registry, a prospective multicenter cohort of patients newly evaluated at a Pulmonary Hypertension Care Center. Multivariable negative binomial regression models were used to estimate incidence rate ratios (IRR) for hospitalization amongst the two groups. We included 461 IPAH patients and 169 CTEPH patients. Twenty-one percent of CTEPH patients underwent pulmonary thromboendarterectomy (PTE) before the end of follow-up. At baseline, patients with CTEPH had significantly worse HRQoL (higher E10 scores) (ß 2.83, SE 1.11, p = 0.01); however, differences did not persist over time. CTEPH patients had higher rates of hospitalization (excluding the hospitalization for PTE) compared to IPAH patients after adjusting for age, sex, body mass index, WHO functional class and six-minute walk distance (IRR 1.66, 95%CI 1.04–2.65, p = 0.03). CTEPH patients who underwent PTE had improved HRQoL as compared to those who were medically managed, but patients who underwent PTE were younger, had higher cardiac outputs and greater six-minute walk distances. In this large, prospective, multicenter cohort, CTEPH patients had significantly worse baseline HRQoL and higher rates of hospitalizations than those with IPAH. CTEPH patients who underwent PTE had significant improvements in HRQoL.

Association of right ventricular dysfunction and pulmonary hypertension with adverse 30-day outcomes in COVID-19 patients.

BackgroundCardiac manifestations in COVID-19 are multifactorial and are associated with increased mortality. The clinical utility and prognostic value of echocardiography in COVID-19 inpatients is not clearly defined. We aim to identify echocardiographic parameters that are associated with 30-day clinical outcomes secondary to COVID-19 hospitalization.MethodsThis retrospective cohort study was conducted in a large tertiary hospital in New York City during the COVID-19 pandemic. It included 214 adult inpatients with a laboratory-confirmed diagnosis of COVID-19 by reverse transcriptase polymerase chain reaction assay (RT-PCR) for SARS-CoV-2 on nasopharyngeal swab and had a transthoracic echocardiogram performed during the index hospitalization. Primary outcome was 30-day all-cause inpatient mortality. Secondary outcomes were 30-day utilization of mechanical ventilator support, vasopressors, or renal replacement therapy.ResultsMild right ventricular systolic dysfunction (odds ratio (OR): 3.51, 95% confidence interval (CI): 1.63–7.57, p = 0.001), moderate to severe right ventricular systolic dysfunction (OR: 7.30, 95% CI: 2.20–24.25, p = 0.001), pulmonary hypertension (OR: 5.39, 95% CI: 1.96–14.86, p = 0.001), and moderate to severe tricuspid regurgitation (OR: 3.92, 95% CI: 1.71–9.03, p = 0.001) were each associated with increased odds of 30-day all-cause inpatient mortality. Pulmonary hypertension and moderate to severe right ventricular dysfunction were each associated with increased odds of 30-day utilization of mechanical ventilator support and vasopressors.ConclusionsRight ventricular dysfunction, pulmonary hypertension, and moderate to severe tricuspid regurgitation were associated with increased odds for 30-day inpatient mortality. This study highlights the importance of echocardiography and its clinical utility and prognostic value for evaluating hospitalized COVID-19 patients.

Efeitos adversos do tratamento com inibidores seletivos da recaptação da serotonina sobre o feto e neonatais de mães com depressão gestacional: Uma revisão crítica

A depressão é um dos transtornos psiquiátricos mais comuns, tendo em vista a alta incidência nesta década, nota-se um predomínio maior de ocorrência em mulheres, destacando o transtorno depressivo maior na gestação. Quando diagnosticada, o tratamento é de acordo o nível da doença, e se tratando de nível moderado a grave inicia-se o tratamento farmacológico com antidepressivos. Os medicamentos mais prescritos são os inibidores seletivos da receptação de Serotonina (ISRS), que tornaram-se fármacos de primeira escolha pela maior segurança em relação aos demais. Entretanto, o tratamento com os ISRS durante a gestação tem implicado em efeitos indesejáveis nos fetos e neonatos. Portanto, o objetivo deste trabalho foi investigar os efeitos adversos sobre o feto/neonatais de gestantes depressivas que passaram por terapêutica com ISRS. Este estudo caracterizou-se em uma revisão crítica, sendo utilizados os bancos de dados da Pubmed/MEDLINE, Scopus e Web of Science, sendo incluídos 35 artigos. Após análise dos estudos, foi constatado que a terapia com ISRS na depressão gestacional aumenta os riscos no feto e neonatos para defeitos congênitos, como: má formação cardíaca, abstinência neonatal, hipertensão pulmonar persistente e possível associação com transtorno do espectro do autismo. Assim, baseado nas evidências científicas o protocolo medicamentoso com ISRS gera efeitos nocivos a criança, no entanto muitas vezes o seu uso na gestação é inevitável. Dessa forma, em situações em que é necessário usar ISRS, a decisão de tal conduta terapêutica deve ser avaliada analisando os riscos da depressão não tratada e a relação risco/benefício do uso dos medicamentos.

The Relationship between Anticoagulation and Mortality in Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry (PHAR)

PurposeLong-term anticoagulation (AC) in pulmonary arterial hypertension (PAH) remains controversial, with prior cohorts reporting 28-66% of participants using AC. In idiopathic PAH (IPAH), AC is associated with improved survival or neutral effect, but may be detrimental in those with PAH from connective tissue disease (CTD-PAH). We sought to evaluate the relationship between AC use and mortality in PHAR, a multi-center prospective registry which enrolled patients referred to pulmonary hypertension care centers between 2015-2020.MethodsForty-seven centers collected clinical characteristics, including PAH-specific therapy and survival, at roughly 6month intervals. We compared participants who received AC at baseline to those who did not. We excluded participants <18 years old and with PAH from congenital heart disease. Mortality was assessed using Kaplan-Meier plots and cox regression analysis adjusted for age, sex, body mass index, race, center, mean pulmonary artery pressure, functional class, and use of PAH specific therapy.ResultsThere were 817 participants who met inclusion criteria. The cohort had a mean age of 56 years, 76% were female, 41% had a diagnosis of IPAH, and 34% had a diagnosis of CTD-PAH. Most participants, 85%, had WHO functional class II-III disease. Overall, 21% of participants were on AC at enrollment; 26% of those with IPAH and 17% of those with CTD-PAH. AC use decreased between 2015 to 2019, from 29% to 19%. Median AC duration was 31 months. AC was not associated with mortality in IPAH (adjusted HR 0.9; CI 0.4-1.9) or CTD-PAH (adjusted HR 1.0; CI 0.4 - 2.5) (Figure).ConclusionAC use in PAH has decreased over time, and is lower than prior registries. AC use is not associated with increased mortality in this analysis of the contemporary PHAR cohort. Analyses examining the relationship between AC use and health care utilization and quality of life are forthcoming. Long-term anticoagulation (AC) in pulmonary arterial hypertension (PAH) remains controversial, with prior cohorts reporting 28-66% of participants using AC. In idiopathic PAH (IPAH), AC is associated with improved survival or neutral effect, but may be detrimental in those with PAH from connective tissue disease (CTD-PAH). We sought to evaluate the relationship between AC use and mortality in PHAR, a multi-center prospective registry which enrolled patients referred to pulmonary hypertension care centers between 2015-2020. Forty-seven centers collected clinical characteristics, including PAH-specific therapy and survival, at roughly 6month intervals. We compared participants who received AC at baseline to those who did not. We excluded participants <18 years old and with PAH from congenital heart disease. Mortality was assessed using Kaplan-Meier plots and cox regression analysis adjusted for age, sex, body mass index, race, center, mean pulmonary artery pressure, functional class, and use of PAH specific therapy. There were 817 participants who met inclusion criteria. The cohort had a mean age of 56 years, 76% were female, 41% had a diagnosis of IPAH, and 34% had a diagnosis of CTD-PAH. Most participants, 85%, had WHO functional class II-III disease. Overall, 21% of participants were on AC at enrollment; 26% of those with IPAH and 17% of those with CTD-PAH. AC use decreased between 2015 to 2019, from 29% to 19%. Median AC duration was 31 months. AC was not associated with mortality in IPAH (adjusted HR 0.9; CI 0.4-1.9) or CTD-PAH (adjusted HR 1.0; CI 0.4 - 2.5) (Figure). AC use in PAH has decreased over time, and is lower than prior registries. AC use is not associated with increased mortality in this analysis of the contemporary PHAR cohort. Analyses examining the relationship between AC use and health care utilization and quality of life are forthcoming.

Association of Pulmonary Hypertension With Inflammatory Markers and Volume Status in Hemodialysis Patients of End-Stage Renal Disease.

Background and objectivesPulmonary hypertension (PH) is an independent risk factor for increased mortality, especially in patients undergoing hemodialysis (HD), but the mechanism of its development is unknown. This study aimed at evaluating volume overload and inflammation as potential variables to cause its development in patients undergoing maintenance hemodialysis.Materials and methodsThis was an observational cross-sectional study conducted on patients undergoing hemodialysis at a tertiary hospital in northern India. Patients of end-stage renal disease, aged 18 years or more, on maintenance hemodialysis for over two months were included in the study. The patients were divided into two groups based on the presence or absence of PH, determined by measuring systolic pulmonary arterial pressure (SPAP). The severity of PH was defined as: mild (SPAP 35-45 mmHg), moderate (SPAP 46-55 mmHg), and severe (SPAP> 55mmHg). The two groups were evaluated for demographic variables, type of vascular access, biochemical parameters, and markers of inflammation and fluid overload. Data between the two groups were compared statistically.ResultsThis study included a total of 82 patients showing the prevalence of PH to be 25.6% with a men-to-women ratio of 2:1. Out of 21 cases of PH, mild PH was found in seven (33.3%) cases, moderate in 14 (66.7%), and cases with severe PH were none. The two groups differed significantly in ejection fraction and markers of inflammation and volume status. Laboratory data associated with PH were alpha-1-acid glycoprotein (p<0.05) and pro-b-type natriuretic peptide (p <0.05).ConclusionThe present study showed higher levels of inflammatory markers alpha-1-acid glycoprotein and pro-b-type natriuretic peptide and lower levels of ejection fraction in patients undergoing HD, indicating a significant association with PH.

Health disparities and treatment approaches in portopulmonary hypertension and idiopathic pulmonary arterial hypertension: an analysis of the Pulmonary Hypertension Association Registry.

Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hypertension (POPH) have worse survival. Health disparities may contribute to these differences but have not been studied. We sought to compare socioeconomic factors in patients with POPH and IPAH and to determine whether socioeconomic status and/or POPH diagnosis were associated with treatment and health-care utilization. We performed a cross-sectional study of adults enrolled in the Pulmonary Hypertension Association Registry. Patients with IPAH (n = 344) and POPH (n = 57) were compared. Compared with IPAH, patients with POPH were less likely to be college graduates (19.6% vs. 34.9%, p = 0.02) and more likely to be unemployed (54.7% vs. 30.5%, p < 0.001) and have an annual household income below poverty level (45.7% vs. 19.0%, p < 0.001). Patients with POPH had similar functional class, quality of life, 6-min walk distance, and mean pulmonary arterial pressure with a higher cardiac index. Compared with IPAH, patients with POPH were less likely to receive combination therapy (46.4% vs. 62.2%, p = 0.03) and endothelin receptor antagonists (28.6% vs. 55.1%, p < 0.001) at enrollment with similar treatment at follow-up. Patients with POPH had more emergency department visits (1.7 ± 2.1 vs. 0.9 ± 1.2, p = 0.009) and hospitalizations in the six months preceding enrollment (1.5 ± 2.1 vs. 0.8 ± 1.1, p = 0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of emergency department visits. Compared to IPAH, patients with POPH have lower socioeconomic status, are less likely to receive initial combination therapy and endothelin receptor antagonists but have similar treatment at follow-up, and have increased health-care utilization.

Burden and Unmet Needs with Portable Oxygen in Patients on Long-Term Oxygen Therapy.

Rationale: Over 1.5 million Americans receive long-term oxygen therapy (LTOT) for the treatment of chronic hypoxemia to optimize functional status and quality of life. However, current portable oxygen equipment, including portable gas tanks (GTs), portable liquid tanks (LTs), and portable oxygen concentrators (POCs), each have limitations that can hinder patient mobility and daily activities. Objectives: To examine patient experiences with portable oxygen to guide equipment innovation and thereby improve patient care on oxygen therapy. Methods: The burden and unmet needs with portable oxygen equipment were assessed in 836 LTOT patients with chronic lung disease (chronic obstructive pulmonary disease [COPD], interstitial lung disease, and pulmonary hypertension) through an online survey. The survey included a combination of multiple-choice, Likert-scale, short-answer, and open-ended questions. Distribution was achieved through patient support organizations, including the U.S. COPD Coalition, the Pulmonary Fibrosis Foundation, and the Pulmonary Hypertension Association. Results: Improvements in portability were ranked as the highest priority by patients across all equipment types, followed by increases in the duration of oxygen supply for GTs, accessibility for LTs, and flow capabilities for POCs. All device types were found to be burdensome, with the greatest burden among GT users, 51% of whom characterized GT use as "strenuous" or "extremely strenuous" (high burden). POCs ranked as the most common (61%) and least burdensome devices; however, 29% of POC users still reported a high associated burden. Forty-seven percent of POC respondents described using a POC despite it not meeting their oxygen needs to benefit from advantages over alternative equipment. Among non-POC users, limited oxygen flow rate capabilities and cost were the top reasons preventing POC use. Conclusions: Although improvements have been made to portable oxygen equipment, this study highlights the burden that remains and reveals a clear need for advances in technology to improve the functional status and quality of life of portable LTOT users.

Factors associated with incident severe pulmonary arterial hypertension in systemic autoimmune rheumatic diseases: a nationwide study.

To assess the association of severe pulmonary arterial hypertension (PAH) with particulate matter <2.5 μm (p.m.2.5) and clinical data in patients with systemic autoimmune rheumatic diseases (SARDs). We used the 2003-2017 nationwide data in Taiwan to identify patients with SARDs, including systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, dermatomyositis/polymyositis and primary Sjögren's syndrome. We identified 479 cases with severe PAH and selected controls matched (1:4) for age, sex, and index year. We used conditional logistic regression analysis to determine factors associated with risks for severe PAH shown as odds ratios (ORs) with 95% confidence intervals (CIs). We found that severe PAH was highly associated with interstitial lung disease (OR, 8.57; 95% CI: 5.52, 13.32), congestive heart failure (OR, 7.62; 95% CI: 5.02, 11.55), valvular heart disease (OR, 3.34; 95% CI: 2.03, 5.50) and slightly associated with thyroid diseases (OR, 1.88; 95% CI: 1.18, 3.00), but not the level of exposure to p.m.2.5. Increased risk for PAH was found in patients receiving corticosteroid (prednisolone equivalent dosage, mg/day, OR, 1.03; 95% CI: 1.01, 1.05), biologics (OR, 2.18; 95% CI: 1.15, 4.12) as well as immunosuppressants, including ciclosporin (OR, 2.17; 95% CI: 1.31, 3.59), azathioprine (OR, 1.96; 95% CI: 1.48, 2.61), cyclophosphamide (OR, 2.01; 95% CI: 1.30, 3.11) and mycophenolate mofetil/mycophenolic acid (OR, 2.42; 95% CI: 1.37, 4.27), and those with the highest level of insured amount (reference, lowest level; OR, 0.53; 95% CI: 0.34, 0.83). The population-based study identified risks for severe PAH in patients with SARDs, and these findings provide evidence for PAH risk stratification in patients with SARDs.

Obesity in Pulmonary Arterial Hypertension (PAH): The Pulmonary Hypertension Association Registry (PHAR).

Obesity is associated with pulmonary arterial hypertension (PAH), but its impact on outcomes such as health-related quality of life (HRQoL), hospitalizations and survival is not well understood. To assess the effect of obesity on health-related quality of life (HRQoL), hospitalizations and survival in patients with PAH. We performed a cohort study of adults with PAH from the Pulmonary Hypertension Association Registry, a prospective multicenter registry. Multivariate linear mixed effects regression was used to examine the relationship between weight categories and HRQoL using the Short Form-12 (SF-12) and emPHasis-10 (e10). We used multivariable negative binomial regression to estimate hospitalization incidence rate ratios (IRRs) and Cox regression to estimate hazard ratios (HRs) for transplant-free survival by weight status. 767 subjects were included: mean age of 57 years, 74% female, 33% overweight and 40% obese, with median follow-up duration of 527 days. Overweight and obese patients had higher baseline e10 scores (worse HRQoL), which persisted over time (p<0.001). The overweight and obese have a trend towards increased incidence of hospitalizations compared to normal weight (IRR 1.34, 95% confidence interval (95%CI) 0.94-1.92 and 1.33, 95%CI 0.93-1.89, respectively). Overweight and obese patients had lower risk of transplant or death as compared to normal weight patients (HR 0.45, 95%CI 0.25-0.80 and 0.39, 95%CI 0.22-0.70, respectively). In a large multicenter, prospective cohort of PAH, overweight and obese patients had worse disease-specific HRQoL despite better transplant-free survival compared to normal weight patients. Future interventions should address the specific needs of these patients.

Outcomes of infants with hypoxic ischemic encephalopathy and persistent pulmonary hypertension of the newborn: results from three NICHD studies

Objective:To determine the association of persistent pulmonary hypertension of the newborn (PPHN) with death or disability among infants with moderate or severe hypoxic ischemic encephalopathy (HIE) treated with therapeutic hypothermia.Methods:We compared infants with and without PPHN enrolled in the hypothermia arm from three randomized controlled trials (RCTs): Induced Hypothermia trial, “usual-care” arm of Optimizing Cooling trial, and Late Hypothermia trial. Primary outcome was death or disability at 18–22 months adjusted for severity of HIE, center, and RCT.Results:Among 280 infants, 67 (24%) were diagnosed with PPHN. Among infants with and without PPHN, death or disability was 47% vs. 29% (adjusted OR 1.65, 0.86–3.14) and death was 26% vs. 12% (adjusted OR 2.04, 0.92–4.53), respectively.Conclusions:PPHN in infants with moderate or severe HIE was not associated with a statistically significant increase in primary outcome. These results should be interpreted with caution given the limited sample size.

Pulmonary Hypertension Clinical Trials and COVID-19: A Discussion With John Ryan and Roham Zamanian

Pulmonary Hypertension Clinical Trials and COVID-19: A Discussion With John Ryan and Roham Zamanian

PH Roundtable: Pulmonary Hypertension and Telehealth in the Time of Coronavirus Disease 2019

PH Roundtable: Pulmonary Hypertension and Telehealth in the Time of Coronavirus Disease 2019

Memos

Memos

Health Disparities in Pulmonary Arterial Hypertension and the Impact of the COVID-19 Pandemic

Health Disparities in Pulmonary Arterial Hypertension and the Impact of the COVID-19 Pandemic

A Survey-based Estimate of COVID-19 Incidence and Outcomes among Patients with Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension and Impact on the Process of Care.

Rationale: Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) typically undergo frequent clinical evaluation. The incidence and outcomes of coronavirus disease (COVID-19) and its impact on routine management for patients with pulmonary vascular disease is currently unknown.Objectives: To assess the cumulative incidence and outcomes of recognized COVID-19 for patients with PAH/CTEPH followed at accredited pulmonary hypertension centers, and to evaluate the pandemic’s impact on clinic operations at these centers.Methods: A survey was e-mailed to program directors of centers accredited by the Pulmonary Hypertension Association. Descriptive analyses and linear regression were used to analyze results.Results: Seventy-seven center directors were successfully e-mailed a survey, and 58 responded (75%). The cumulative incidence of COVID-19 recognized in individuals with PAH/CTEPH was 2.9 cases per 1,000 patients, similar to the general U.S. population. In patients with PAH/CTEPH for whom COVID-19 was recognized, 30% were hospitalized and 12% died. These outcomes appear worse than the general population. A large impact on clinic operations was observed including fewer clinic visits and substantially increased use of telehealth. A majority of centers curtailed diagnostic testing and a minority limited new starts of medical therapy. Most centers did not use experimental therapies in patients with PAH/CTEPH diagnosed with COVID-19.Conclusions: The cumulative incidence of COVID-19 recognized in patients with PAH/CTEPH appears similar to the broader population, although outcomes may be worse. Although the total number of patients with PAH/CTEPH recognized to have COVID-19 was small, the impact of COVID-19 on broader clinic operations, testing, and treatment was substantial.

EmPHasis-10 as a measure of health-related quality of life in pulmonary arterial hypertension: data from PHAR.

While the performance of the emPHasis-10 (e10) score has been evaluated against limited patient characteristics within the United Kingdom, there is an unmet need for exploring the performance of the e10 score among pulmonary arterial hypertension (PAH) patients in the United States. Using the Pulmonary Hypertension Association Registry, we evaluated relationships between the e10 score and demographic, functional, haemodynamic and additional clinical characteristics at baseline and over time. Furthermore, we derived a minimally important difference (MID) estimate for the e10 score. We analysed data from 565 PAH (75% female) adults aged mean±sd 55.6±16.0 years. At baseline, the e10 score had notable correlation with factors expected to impact quality of life in the general population, including age, education level, income, smoking status and body mass index. Clinically important parameters including 6-min walk distance and B-type natriuretic peptide (BNP)/N-terminal proBNP were also significantly associated with e10 score at baseline and over time. We generated a MID estimate for the e10 score of -6.0 points (range -5.0--7.6 points). The e10 score was associated with demographic and clinical patient characteristics, suggesting that health-related quality of life in PAH is influenced by both social factors and indicators of disease severity. Future studies are needed to demonstrate the impact of the e10 score on clinical decision-making and its potential utility for assessing clinically important interventions.

Clinical Differences and Outcomes between Methamphetamine-associated and Idiopathic Pulmonary Arterial Hypertension in the Pulmonary Hypertension Association Registry.

Rationale: Single-center studies demonstrated that methamphetamine use is associated with pulmonary arterial hypertension (Meth-APAH). We used the Pulmonary Hypertension Association Registry to evaluate the national distribution of Meth-APAH and to compare its impact on patient-reported and clinical outcomes relative to idiopathic PAH.Objectives: To determine if patients with Meth-APAH differ from those with idiopathic PAH in demographics, regional distribution in the United States, hemodynamics, health-related quality of life, PAH-specific treatment, and health care use.Methods: The Pulmonary Hypertension Association Registry is a U.S.-based prospective cohort of patients new to care at a Pulmonary Hypertension Care Center. The registry collects baseline demographics, clinical parameters, and repeated measures of health-related quality of life, World Health Organization functional class, 6-minute walk distance, therapy, and health care use. Repeated measures of functional class, health-related quality of life, type of therapy, emergency department visits, and hospitalizations were compared using generalized estimating equations.Results: Of 541 participants included, 118 had Meth-APAH; 83% of Meth-APAH arose in the western United States. The Meth-APAH group was younger and had a poorer socioeconomic status and lower cardiac index than the idiopathic PAH group, despite no difference in mean pulmonary artery pressure or pulmonary vascular resistance. The Meth-APAH group had a more advanced functional class in longitudinal models (0.22 points greater; 95% confidence interval [CI], 0.07 to 0.37) and worse PAH-specific (emPHasis-10) health-related quality of life (-5.4; 95% CI, -8.1 to -2.8). There was no difference in dual combination therapy; however, participants with Meth-APAH were less likely to be initiated on triple therapy (odds ratio [OR], 0.43; 95% CI, 0.24 to 0.77) or parenteral therapy (OR, 0.10; 95% CI, 0.04 to 0.24). Participants with Meth-APAH were more likely to seek care in the emergency department (incidence rate ratio, 2.30; 95% CI, 1.71 to 3.11) and more likely to be hospitalized (incidence rate ratio, 1.42; 95% CI, 1.10 to 1.83).Conclusions: Meth-APAH represents a unique clinical phenotype of PAH, most common in the western United States. It accounts for a notable proportion of PAH in expert centers. Assessment for methamphetamine use is necessary in patients with PAH.

PHYSICIANS ATTITUDES TOWARD ANTICOAGULATION USE IN PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION

SESSION TITLE: Pulmonary Vascular Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: The benefit of anticoagulant use in pulmonary arterial hypertension (PAH) patients remains unclear. Multiple studies have shown conflicting results regarding the utility of anticoagulation in PAH. Moreover, other points of controversies include selection of specific anticoagulant agent, intensity of anticoagulation, and patient selection based on PAH subtype. This study aims to evaluate the practice habits of physicians caring for patients with PAH in the United States of America (USA). METHODS: A seven-question web-based survey regarding anticoagulation management in patients with PAH in the USA was distributed by email to clinicians with experience treating patients with pulmonary hypertension and are registered members of the Pulmonary Hypertension Association (PHA). RESULTS: We received a total of 56 responses, predominantly from academic, non-profit institutions (82.14%, n=46); by PHA members serving as non-trainee physicians (98.21%, n=55). The majority of respondents (53.57%, n=31) rarely start anticoagulation in patients with WHO group 1 PAH. In idiopathic PAH (IPAH) subtype, providers mainly start in severe disease (22.82%, n=12) or for other coexisting indications (e.g., an indwelling catheter, underlying thrombogenic disorder, prior clots). Nearly all (86%, n=50) respondents rarely start anticoagulation in connective tissue disease related PAH (CTD-PAH). While the majority of respondents do not routinely use anticoagulation in general, those who do, use warfarin (46.3%, n=25) and target a lower range of INR (1.5–2). Few providers (18.18%, n=10) reported using direct oral anticoagulants. CONCLUSIONS: Despite the presence of limited evidence showing that the use of anticoagulation in patients with PAH may be associated with survival benefit—specifically in the idiopathic PAH subtype—the majority of clinicians who care for these patients believe that the risks outweigh the benefits unless there is a coexisting indication. Prospective and randomized studies are warranted for further evaluation. CLINICAL IMPLICATIONS: This study highlights the uncertainty among clinicians about the benefit of anticoagulation in patients with WHO group 1 PAH and the need for randomized trials. DISCLOSURES: No relevant relationships by Noura Alturaif, source=Web Response No relevant relationships by Jamie Kennedy, source=Web Response No relevant relationships by Sula Mazimba, source=Web Response Clinical Trial support relationship with Complexa, Inc Please note: $1001 - $5000 Added 04/02/2020 by Andrew Mihalek, source=Web Response, value=Grant/Research Support

Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry.

BackgroundWHO Group 1 pulmonary arterial hypertension is a progressive and potentially fatal disease. Individuals living at higher altitude are exposed to lower barometric pressure and hypobaric hypoxemia. This may result in pulmonary vasoconstriction and contribute to disease progression. We sought to examine the relationship between living at moderately high altitude and pulmonary arterial hypertension characteristics.MethodsForty-two US centers participating in the Pulmonary Hypertension Association Registry enrolled patients who met the definition of WHO Group 1 pulmonary arterial hypertension. We utilized baseline data and patient questionnaire responses. Patients were divided into two groups: moderately high altitude residence (home ≥4000 ft) and low altitude residence (home <4000 ft) based on zip-code. Clinical characteristics, hemodynamic data, patient demographics, and patient reported quality of life metrics were compared.ResultsControlling for potential confounders (age, sex at birth, body mass index, supplemental oxygen use, race, 100-day cigarette use, alcohol use, and pulmonary arterial hypertension medication use), subjects residing at moderately high altitude had a 6-min walk distance 32 m greater than those at low altitude, despite having a pulmonary vascular resistance that was 2.2 Wood units higher. Additionally, those residing at moderately high altitude had 3.7 times greater odds of using supplemental oxygen.ConclusionPatients with pulmonary arterial hypertension who live at moderately high altitude have a higher pulmonary vascular resistance and are more likely to need supplemental oxygen. Despite these findings, moderately high altitude Pulmonary Hypertension Association Registry patients have better functional tolerance as measured by 6-min walk distance. It is possible that a “high-altitude phenotype” of pulmonary arterial hypertension may exist. These findings warrant further study.

Association of Pulmonary Hypertension With End-Stage Renal Disease Among the Obese Population.

IntroductionPulmonary hypertension (PH) is a known complication that occurs in patients of end-stage renal disease (ESRD) that have an arteriovenous fistula (AVF) for hemodialysis (HD). It is defined as pulmonary artery pressure (PAP) of greater than 30 mmHg on echocardiography. The presence of PH in ESRD is an independent risk factor and decreases the survival likelihood among HD patients. Unexplained PH is frequently seen in ESRD following AVF.Obesity can lead to various complications, such as sleep apnea, cardiac complications, pulmonary hypertension, and mortality. Data on the prevalence of coexisting PH and obesity are scarce.Obese patients often have increased albumin excretion rates (AER) that can lead to early renal impairment and an increase in intraglomerular pressure, which may increase the risk of cardiovascular (CV) morbidity and mortality.Therefore, the study aimed to evaluate and compare the associated PH and obesity separately and collectively among ESRD patients.MethodsThis comparative cross-sectional study was conducted in a tertiary care public sector hospital with the approval of the medical ethics review board committee.The study enrolled all consecutive patients with ESRD as defined by having an estimated glomerular filtration rate (GFR) of <15 mL/min/1.7 3 m2 from April 2017 till March 2019, who presented to our facility. These patients underwent dialysis twice or thrice a week, each session lasting three to four hours approximately. On initial encounter, trans-thoracic echocardiography (TTE) was done by the cardiologist to diagnose pulmonary hypertension.In addition, body mass index (BMI) was calculated for all patients, and the patients were categorized into underweight, normal, overweight, or obese. All patients underwent post-dialysis TTE at one hour or when patients were at the optimal dry weight. Systolic PAP and ejection fraction were measured, and pulmonary hypertension was defined as a PAP of 30 mmHg or greater on TTE.ESRD patients that were diagnosed with PH prior to hemodialysis or had primary PH were excluded from the study. Only ESRD patients developing secondary PH after hemodialysis were included in the study.The chi-square test was used to see the correlation of gender, ambulation status, smoking status, obesity, pulmonary hypertension, body mass index (BMI), and pulmonary hypertension and obesity combined on the final outcome. A p-value of 0.05 was considered significant. Odds ratio (OR) and relative risk (RR) were calculated for pulmonary hypertension and obesity combined, obesity, and pulmonary hypertension in the final outcome.ResultsThe study enrolled 204 patients with a mean age of 46.23 (±20.45 SD) having higher female participation of 108 (52.9%), whereas 96 (47.1%) were males. The average weight of the cohort was 66.78 kg (±22.98 SD) with a mean BMI of 29.91 kg/m2 (±13.29SD), 52 (25.5%) patients were underweight, 40 (19.6%) had a normal BMI, 29 (14.2%) were overweight, and 83 (40.7%) patients were obese.Pulmonary hypertension and obesity combined were observed in 48 (23.5%) of the cases and there was a 4.60 relative risk of death among these individuals, with an odds ratio of 13.35 and a p-value of 0.00.ConclusionThe study shows a strong synergistic effect of pulmonary hypertension and obesity towards the final survival outcome in ESRD patients who are on hemodialysis.