Effects of COVID-19 pandemic on the management of pulmonary hypertension.

The coronavirus of 2019 (COVID-19) disrupted delivery of healthcare. Patients with pulmonary hypertension (PH), especially pulmonary arterial hypertension (PAH), require significant resources for both diagnosis and management and are at high risk for decompensation due to disruption in their care. A survey consisting of 47 questions related to the care of patients with PH was designed by the American College of Chest Physicians 2020-2021 Pulmonary Vascular Disease (PVD) NetWork Steering Committee and sent to all members of the PVD NetWork, as well as the multiple other professional networks for PH. Participation was voluntary and anonymous. Responses were collected from November 2020 through February 2021. Ninety-five providers responded to this survey. The majority (93%) believe that care of PH patients has been affected by the pandemic. Sixty-seven percent observed decreased referrals for PH evaluation. Prior to the pandemic, only 15% used telemedicine for management of PH patients compared to 84% during the pandemic. Telemedicine was used most for follow up of selected low-risk patients (49%). While 22% respondents were completely willing to prescribe new PAH therapy via telemedicine, 11% respondents were completely unwilling. Comfort levels differed based on type of medication being prescribed. Over 90% of providers experienced disruptions in obtaining testing and 31% experienced disruptions in renewal or approval of medications. Overall, providers perceived that the COVID-19 pandemic caused significant disruption of care for PH patients. Telemedicine utilization increased but was used mostly in low-risk patients. Some providers had a decreased level of comfort prescribing PAH therapy via telemedicine encounters.

Characteristics, Long-term Survival, and Risk Assessment of Pediatric Pulmonary Arterial Hypertension in China: Insights From a National Multicenter Prospective Registry.

BACKGROUND: Registry-based studies of pediatric pulmonary arterial hypertension (PPAH) are scarce in developing countries, including China. The PPAH risk assessment tool needs further evaluation and improvement. RESEARCH QUESTION: What are the characteristics and long-term survival of PPAH in China and what is the performance of the PPAH risk model in Chinese patients? STUDY DESIGN AND METHODS: Patients with PAH were enrolled in the national prospective multicenter registry from August 2009 through December 2019. Children 3 months to 18 years of age at the time of PAH diagnosis were analyzed. RESULTS: A total of 247 children with PAH were enrolled. The median patient age was 14.8 years, and 58.3% of patients were female. Most patients had a diagnosis of PAH associated with congenital heart disease (CHD; 61.5%) and idiopathic or heritable PAH (37.7%). The median time from symptom onset to PAH diagnosis was 24 months. The mean pulmonary artery pressure and pulmonary vascular resistance index were 70.78 ± 19.80 mm Hg and 21.82 ± 11.18 Wood Units·m2, respectively. Patients with CHD-associated PAH experienced a longer diagnostic delay and demonstrated higher pulmonary artery pressure, but better cardiac performance, than those with idiopathic or heritable PAH. An increased number of patients received targeted therapy at the last follow-up compared with baseline. The 5- and 10-year survival rates of the entire cohort were 74.9% and 55.7%, respectively, with better survival in patients with CHD-associated PAH than in those with idiopathic or heritable PAH. Patients with low risk had better survival than those with high risk according to the simplified noninvasive risk score model with weight, function class, and echocardiographic right ventricular size, both at baseline and follow-up. INTERPRETATION: Patients with PPAH in China showed severely compromised hemodynamics with marked diagnostic delay. The long-term survival of PPAH is poor despite the increased usefulness of targeted drugs. The simplified noninvasive risk model demonstrated good performance for predicting survival in Chinese children with PAH. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT01417338; URL: www. CLINICALTRIALS: gov.

The economic burden of pulmonary arterial hypertension in Spain.

BACKGROUND: Pulmonary Arterial Hypertension (PAH) is a rare, debilitating, and potentially fatal disease. This study aims to quantify the economic burden of PAH in Spain. METHODS: The study was conducted from a societal perspective, including direct and indirect costs associated with incident and prevalent patients. Average annual costs per patient were estimated by multiplying the number of resources consumed by their unit cost, differentiating the functional class (FC) of the patient. Total annual costs per FC were also calculated, taking the 2020 prevalence and incidence ranges into account. An expert committee validated the information on resource consumption and provided primary information on pharmacological consumption. Unit costs were estimated using official tariffs and salaries in Spain. A deterministic sensitivity analysis was conducted to test the uncertainty of the model. RESULTS: The average annual total cost was estimated at €98,839 per prevalent patient (FC I-II: €65,233; FC III: €103,736; FC IV: €208,821), being €42,110 for incident patients (FC I-II: €25,666; FC III: €44,667; FC IV: €95,188). The total annual cost of PAH in Spain, taking into account a prevalence between 16.0 and 25.9 cases per million adult inhabitants (FC I-II 31.8%; FC III 61.3%; FC IV 6.9%) and an incidence of 3.7, was estimated at €67,891,405 to €106,131,626, depending on the prevalence considered. Direct healthcare costs accounted for 64% of the total cost, followed by indirect costs (24%), and direct non-healthcare costs (12%). The total costs associated with patients in FC I-II ranged between €14,161,651 and €22,193,954, while for patients in FC III costs ranged between €43,763,019 and €68,391,651, and for patients in FC IV between €9,966,735 and €15,546,021. In global terms, patients with the worst functional status (FC IV) account for only 6.9% of the adults suffering from PAH in Spain, but are responsible for 14.7% of the total costs. CONCLUSIONS: PAH places a considerable economic burden on patients and their families, the healthcare system, and society as a whole. Efforts must be made to improve the health and management of these patients since the early stages of the disease.

A pragmatic approach to risk assessment in pulmonary arterial hypertension using the 2015 European Society of Cardiology/European Respiratory Society guidelines.

OBJECTIVE: To optimise treatment of patients with pulmonary arterial hypertension (PAH), the 2015 European Society of Cardiology/European Respiratory Society guidelines recommend using risk stratification, with the aim of patients achieving low-risk status. Previous analyses of registries made progress in using risk stratification approaches, however, the focus is often on patients with a low-risk prognosis, whereas most PAH patients are in intermediate-risk or high-risk categories. Using only six parameters with high prognostic relevance, we aimed to demonstrate a pragmatic approach to individual patient risk assessment to discriminate between patients at low risk, intermediate risk and high risk of death. METHODS: Risk assessment was performed combining six parameters in four criteria: (1) WHO functional class, (2) 6 min walk distance, (3) N-terminal pro-brain natriuretic peptide (BNP)/BNP plasma levels or right atrial pressure and (4) cardiac index or mixed venous oxygen saturation. Assessments were made at baseline and at first follow-up after 3-4 months. RESULTS: 725 PAH treatment-naive patients were analysed. Survival estimates between risk groups were statistically significant at baseline and first follow-up (p<0.001), even when the analysis was performed within PAH etiological subgroups. Similar results were observed in 208 previously treated PAH patients. Furthermore, patients who remained at or improved to low risk had a significantly better estimated survival compared with patients who remained at or worsened to intermediate risk or high risk (p≤0.005). CONCLUSION: The simplified risk-assessment method can discriminate idiopathic, connective-tissue-disease-associated and congenital-heart-disease-associated PAH patients into meaningful high-risk, intermediate-risk and low-risk groups at baseline and first follow-up. This pragmatic approach reinforces targeting a low-risk profile for PAH patients.

Risk stratification of adults with congenital heart disease during the COVID-19 pandemic: insights from a multinational survey among European experts.

OBJECTIVE: Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies. METHODS: This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios. RESULTS: 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios. CONCLUSIONS: Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.

Cost-Effectiveness of Combination Therapy for Patients With Systemic Sclerosis-Related Pulmonary Arterial Hypertension.

Background To evaluate the cost-effectiveness of combination pulmonary arterial hypertension specific therapy in systemic sclerosis-related PAH. Methods and Results Health outcomes and costs were captured through data linkage. Health utility was derived from Medical Outcomes Study Short Form-36 scores. A probabilistic discrete-time model was developed to simulate lifetime changes in costs and health utility. Mortality was predicted using a Gompertz parametric survival model. For both treatment arms, the simulations were started using the same cohort of 10 000 patients. Probabilistic sensitivity analysis was performed using the Monte Carlo simulation with 1000 sets of sampled parameter values. Of 143 patients with systemic sclerosis-related pulmonary arterial hypertension, 89 were on monotherapy and 54 on combination therapy. Mean simulated costs per patient per year in monotherapy and combination therapy groups were AU$23 411 (US$16 080) and AU$29 129 (US$19 982), respectively. Mean life years and quality-adjusted life years from pulmonary arterial hypertension diagnosis to death of patients receiving monotherapy were 7.1 and 3.0, respectively, and of those receiving combination therapy were 9.2 and 3.9, respectively. Incremental costs per life year and quality-adjusted life year gained of combination therapy compared with monotherapy were AU$47 989 (US$32 920) and AU$113 823 (US$78 082), respectively. At a willingness-to-pay threshold of AU$102 000 (US$69 972) per life year gained, and of AU$177 222 (US$121 574) per quality-adjusted life year gained, the probability of combination therapy being cost-effective was 0.95. Conclusions The incremental cost per quality-adjusted life year gained of combination therapy compared with monotherapy was substantial in the base case analysis. Given the fatal prognosis of systemic sclerosis-related pulmonary arterial hypertension and the incremental cost per life year of AU$47 989 (US$32 920), combination therapy could be considered cost-effective in systemic sclerosis-related pulmonary arterial hypertension.

Burden of pulmonary arterial hypertension in England: retrospective HES database analysis.

BACKGROUND: The clinical and economic burden of pulmonary arterial hypertension (PAH) is poorly understood outside the United States. This retrospective database study describes the characteristics of patients with PAH in England, including their healthcare resource utilisation (HCRU) and associated costs. METHODS: Data from 1 April 2012 to 31 March 2018 were obtained from the National Health Service (NHS) Digital Hospital Episode Statistics database, which provides full coverage of patient events occurring in NHS England hospitals. An adult patient cohort was defined using an algorithm incorporating pulmonary hypertension (PH) diagnosis codes, PAH-associated procedures, PH specialist centre visits and PAH-specific medications. HCRU included inpatient admissions, outpatient visits and Accident and Emergency (A&E) attendances. Associated costs, calculated using national tariffs inflation-adjusted to 2017, did not include PAH-specific drugs on the High Cost Drugs list. RESULTS: The analysis cohort included 2527 patients (68.4% female; 63.6% aged ⩾50 years). Mean annual HCRU rates ranged from 2.9 to 3.2 for admissions (21-25% of patients had ⩾5 admissions), 9.4-10.3 for outpatient visits and 0.8-0.9 for A&E attendances. Costs from 2013 to 2017 totalled £43.2M (£33.9M admissions, £8.3M outpatient visits and £0.9M A&E attendances). From 2013 to 2017, mean cost per patient decreased 13% (from £4400 to £3833) for admissions and 13% (from £1031 to £896) for outpatient visits, but increased 52% (from £81 to £123) for A&E attendances. CONCLUSION: PAH incurs a heavy economic burden on a per-patient basis, highlighting the need for improved treatment strategies able to reduce disease progression and hospitalisations.The reviews of this paper are available via the supplemental material section.

Risk assessment in patients with pulmonary arterial hypertension in the era of COVID 19 pandemic and the telehealth revolution: State of the art review.

Patients affected by pulmonary arterial hypertension (PAH) benefit from intensive, continuous clinical monitoring to guide escalation of treatments that carry the potential to improve survival and quality of life. During the coronavirus disease 2019 pandemic, the need for physical distancing has fueled the expeditious expansion of various telehealth modalities, which may apply in a unique manner to individuals with PAH. Performance of objective risk assessments in patients with PAH remotely via telemedical visits and other telehealth mechanisms is unprecedented and not yet rigorously validated. The uniquely high risk for rapid deterioration in patients with PAH demands a high degree of sensitivity to detect changes in functional assessments. In this review, several telehealth modalities for potential utilization in risk assessment and treatment titration in patients with PAH are explored, yet additional study is needed for their validation with the pre-pandemic care paradigm.

Risk assessment in patients with functional class II pulmonary arterial hypertension: Comparison of physician gestalt with ESC/ERS and the REVEAL 2.0 risk score.

BACKGROUND: Accurate and regular risk assessment is important for evaluation and treatment of pulmonary arterial hypertension (PAH) patients, including those with functional class (FC) II symptoms, a population considered at low risk for disease progression. Risk assessment methods include subjective and objective evaluations. Multiparametric assessments include tools based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines (COMPERA and FPHR methods, respectively) and the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL; REVEAL 2.0 tool). To better understand risk status determination in FC II patients, we compared physician-reported risk assessments with objective multiparameter assessment tools. METHODS: This retrospective chart analysis included PAH patients with FC II symptoms receiving monotherapy or dual therapy. Physicians were surveyed (via telephone) to obtain an assessment of patient risk using their typical methodology, which might have been informed by objective risk assessment. Patient risk was then calculated independently using COMPERA, FPHR and REVEAL 2.0 tools. Factors associated with incongruent risk assessment were identified. RESULTS: Of the 153 patients, 41%, 46%, and 13% were classified as low, intermediate, and high risk, respectively, by physicians. Concordance between physician gestalt and objective methods ranged from 43%-54%. Among patients considered as low risk by physician gestalt, 4%-28% were categorized as high risk using objective methods. The most common physician factor associated with incongruent risk assessment was less frequent echocardiography during follow-up (every 7-12 months vs. every 3 months; p = 0.01). CONCLUSIONS: More than half of FC II PAH patients were classified as intermediate/high risk using objective multiparameter assessments. Incorporating objective risk-assessment algorithms into clinical practice may better inform risk assessment and treatment strategies.

Inpatient Palliative Care Use in Patients With Pulmonary Arterial Hypertension: Temporal Trends, Predictors, and Outcomes.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive disease associated with significant morbidity and mortality. Despite the negative impact of PAH on quality of life and survival, data on use of specialty palliative care services (PCS) is scarce. RESEARCH QUESTION: We sought to evaluate the inpatient use of PCS in patients with PAH. STUDY DESIGN AND METHODS: Using the National (Nationwide) Inpatient Sample, 30,495 admissions with a primary diagnosis of PAH were identified from 2001 through 2017. The primary outcome of interest was temporal trends and predictors of inpatient PCS use in patients with PAH. RESULTS: The inpatient use of PCS was low (2.2%), but increased during the study period from 0.5% in 2001 to 7.6% in 2017, with a significant increase starting in 2009. White race, private insurance, higher socioeconomic status, hospital-specific factors, higher comorbidity burden (Charlson Comorbidity Index), cardiac and noncardiac organ failure, and use of extracorporeal membrane oxygenation and noninvasive mechanical ventilation were independent predictors of increased PCS use. PCS use was associated with a higher prevalence of do-not-resuscitate status, a longer length of stay, higher hospitalization costs, and increased in-hospital mortality with less frequent discharges to home, likely because these patients were also sicker (higher comorbidity index and illness acuity). INTERPRETATION: The inpatient use of PCS in patients with PAH is low, but has been increasing over recent years. Despite increased PCS use over time, patient- and hospital-specific disparities in PCS use continue. Further studies evaluating these disparities and the role of PCS in the comprehensive care of PAH patients are warranted.

Prevalence of pulmonary arterial hypertension in the Camerino area of central Italy and savings resulting from generic bosentan.

Objective: Pulmonary arterial hypertension is a rare and progressive respiratory disease characterised by high blood pressure and vascular resistance producing right ventricular fatigue. In Italy, pulmonary hypertension can be treated with different drugs available on the market at different costs, and in the Marche region distributed exclusively by hospital pharmacies. The present study examined in an area of the Marche region the use of drugs specifically indicated for pulmonary hypertension, and evaluated how the introduction of the generic bosentan might lower pharmaceutical costs for the healthcare budget. Methods: The study examined oral administration prescriptions and costs using data from the Apotheke Gold (Record Data) database from 1 January 2012 to 31 August 2017. Results: Annually (from 1 January 2012 to 31 August 2017), an average of 4.83 patients were treated (prevalence of 102.35 cases per 1 million residents) with ambrisentan (Volibris), bosentan (Tracleer), macitentan (Opsumit), tadalafil (Adcirca) or sildenafil (Revatio). The total expenditure during the 5-year 8-month period was €472 405. Ambrisentan was by far the most expensive product overall, with a total expenditure of €222 380 for the period studied (a daily cost of €67.39), even though Tracleer had the highest cost for a day of treatment (a daily cost of €94.48, but a total expenditure of €163 976 for the period, due to its more recent marketing). Providing patients with the generic form bosentan in place of Tracleer would lower the costs dramatically. A very significant annual savings per patient of approximately €31 879 would be achieved, a striking 92.4% reduction in costs. Conclusion: The prevalence of pulmonary arterial hypertension reported for Camerino and its surrounding area in the Marches region is quite high compared with that reported by other authors for France and Scotland. The introduction of the generic bosentan would cut costs drastically. It is to be hoped that centralised procurement at the regional level would bring further savings.

Risk assessment in pulmonary arterial hypertension: Insights from the GRIPHON study.

BACKGROUND: Approaches to risk assessment in pulmonary arterial hypertension (PAH) include the noninvasive French risk assessment approach (number of low-risk criteria based on the European Society of Cardiology and European Respiratory Society guidelines) and Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) 2.0 risk calculator. The prognostic and predictive value of these methods for morbidity/mortality was evaluated in the predominantly prevalent population of GRIPHON, the largest randomized controlled trial in PAH. METHODS: GRIPHON randomized 1,156 patients with PAH to selexipag or placebo. Post-hoc analyses were performed on the primary composite end-point of morbidity/mortality by the number of low-risk criteria (World Health Organization functional class I-II; 6-minute walk distance >440 m; N-terminal pro-brain natriuretic peptide <300 ng/liter) and REVEAL 2.0 risk category. Hazard ratios and 95% confidence intervals were calculated using Cox proportional hazard models. RESULTS: Both the number of low-risk criteria and the REVEAL 2.0 risk category were prognostic for morbidity/mortality at baseline and any time-point during the study. Patients with 3 low-risk criteria at baseline had a 94% reduced risk of morbidity/mortality compared to patients with 0 low-risk criteria and were all categorized as low-risk by REVEAL 2.0. The treatment effect of selexipag on morbidity/mortality was consistent irrespective of the number of low-risk criteria or the REVEAL 2.0 risk category at any time-point during the study. Selexipag-treated patients were more likely to increase their number of low-risk criteria from baseline to week 26 than placebo-treated patients (odds ratio 1.69, p = 0.0002); similar results were observed for REVEAL 2.0 risk score. CONCLUSIONS: These results support the association between risk profile and long-term outcome and suggest that selexipag treatment may improve risk profile.

Characterizing Health Outcomes in Idiopathic Pulmonary Fibrosis using US Health Claims Data.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a life-threatening interstitial lung disease (ILD). Characterizing health outcomes of IPF patients is challenging due to disease rarity. OBJECTIVE: This study aimed to identify the burden of disease in patients newly diagnosed with IPF. METHODS: Patients with ≥1 claim with an IPF diagnosis were identified from a United States healthcare insurer's database (2000-2013). Patients with other known causes of ILD or aged <40 years were excluded. Subgroups were compared based on the 2011 change in International Classification of Diseases, 9th Revision (ICD-9) definition of IPF and occurrence of IPF testing. The prevalence and incidence of preselected health conditions of clinical interest were estimated. RESULTS: Median age of newly diagnosed patients (n = 7,298) was 62 years (54.0% male). Restricting to patients with IPF diagnostic testing did not substantially affect cohort characteristics, nor did ICD-9 IPF coding change. Mean follow-up was 1.7 years; 16.8% of patients died; and a substantial proportion of patients were censored due to end of health plan enrollment (50.7%) and other causes of ILD (19.6%). The incidence of pulmonary hypertension, lung cancer, and claims-based algorithm proxy for acute respiratory worsening of unknown cause was 22.5, 17.6, and 12.6 per 1,000 person-years, respectively. CONCLUSIONS: Patients with IPF had a high disease burden with a variety of health outcomes observed, including a high rate of mortality. Database censoring due to changes in enrollment or other ILD diagnoses limited follow-up. Altering cohort entry definitions, including IPF testing or ICD-9 IPF coding change, had little impact on cohort baseline characteristics.

Hospital burden of pulmonary arterial hypertension in France.

BACKGROUND & AIMS: Pulmonary arterial hypertension is a severe disease associated with frequent hospitalisations. This retrospective analysis of the French medical information PMSI-MSO database aimed to describe incident cases of patients with pulmonary arterial hypertension hospitalised in France in 2013 and to document associated hospitalisation costs from the national health insurance perspective. METHODS: Cases of pulmonary arterial hypertension were identified using a diagnostic algorithm. All cases hospitalised in 2013 with no hospitalisation the previous two years were retained. All hospital stays during the year following the index hospitalisation were extracted, and classified as incident stays, monitoring stays or stays due to disease worsening. Costs were attributed from French national tariffs. RESULTS: 384 patients in France were hospitalised with incident pulmonary arterial hypertension in 2013. Over the following twelve months, patients made 1,271 stays related to pulmonary arterial hypertension (415 incident stays, 604 monitoring stays and 252 worsening stays). Mean age was 59.6 years and 241 (62.8%) patients were women. Liver disease and connective tissue diseases were documented in 62 patients (16.1%) each. Thirty-one patients (8.1%) died during hospitalisation and four (1.0%) received a lung/heart-lung transplantation. The total annual cost of these hospitalisations was € 3,640,382. € 2,985,936 was attributable to standard tariffs (82.0%), € 463,325 to additional ICU stays (12.7%) and € 191,118 to expensive drugs (5.2%). The mean cost/stay was € 2,864, ranging from € 1,282 for monitoring stays to € 7,285 for worsening stays. CONCLUSIONS: Although pulmonary arterial hypertension is rare, it carries a high economic burden.

Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study.

INTRODUCTION: Current guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH-CHD). OBJECTIVES: The aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH-CHD patients during 12-month follow-up. METHODS: We reviewed 288 Chinese PAH-CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low-risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow-up were collected. The association between low-risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed. RESULTS: There were 105 PAH-CHD patients included in the final analysis. Twenty-nine patients had AEs defined as death, initiation of new or combined medication treatment, or re-hospitalisation because of the PAH worsening. Among the low-risk criteria, WHO/NYHA functional class, 6-minute walking distance (6MWD), NT-proBNP and SvO2 were significantly different between AE and AE-free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03-0.19, P < 0.001) and NT-proBNP (HR = 0.35, 95% CI: 0.16-0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low-risk criteria at diagnosis discriminated the risk of AEs (P < 0.001). CONCLUSIONS: Among the low-risk criteria proposed by current guidelines, 6MWD and NT-proBNP predicted AEs independently for PAH-CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH-CHD patients.

Risk classification of pulmonary arterial hypertension by echocardiographic combined assessment of pulmonary vascular resistance and right ventricular function.

Which combination of clinical parameters improves the prediction of prognosis in patients with pulmonary arterial hypertension (PAH) remains unclear. We examined whether combined assessment of pulmonary vascular resistance and right ventricular function by echocardiography is useful for classifying risks in PAH. In 41 consecutive patients with PAH (mean age of 48.9 ± 17.3 years, 31 females), a 6-min walk test, pulmonary function test, and echocardiography were performed at baseline and during PAH-specific therapies. The study endpoint was defined as a composite of cardiovascular death and hospitalization for PAH and/or right ventricular failure. During a follow-up period of 9.2 ± 8.7 months, 18 patients reached the endpoint. Multivariate regression analysis showed that the ratio of tricuspid regurgitation pressure gradient to the time-velocity integral of the right ventricular outflow tract (TRPG/TVI) and tricuspid annular plane systolic excursion (TAPSE) during PAH-specific treatment were independent prognostic predictors of the endpoint. Using cutoff values indicated by receiver operating characteristic analysis, the patients were divided into four subsets. Multivariate analyses by Cox's proportional hazards model adjusted for age, sex and body mass index indicated that subset 4 (TRPG/TVI ≥ 3.89 and TAPSE ≤ 18.9 mm) had a significantly higher event risk than did subset 1 (TRPG/TVI < 3.89 and TAPSE > 18.9 mm): HR = 25.49, 95% CI 4.70-476.97, p < 0.0001. Combined assessment of TRPG/TVI and TAPSE during adequate PAH-specific therapies enables classification of risks for death and/or progressive right heart failure in PAH.

The Use of Risk Assessment Tools and Prognostic Scores in Managing Patients with Pulmonary Arterial Hypertension.

PURPOSE OF REVIEW: Pulmonary arterial hypertension (PAH) is a chronic, progressive, and incurable disease with significant morbidity and mortality. Despite increasingly available treatment options, PAH patients continue to experience disease progression and increased rates of hospitalizations due to right heart failure. Physician's ability to comprehensively assess PAH patients, determine prognosis, and monitor disease progression and response to treatment remains critical in optimizing outcomes. RECENT FINDINGS: Risk assessment in PAH should include a range of clinical, hemodynamic, and exercise parameters, performed in a serial fashion over the course of treatment. Approaches to risk assessment in PAH patients include the use of risk variables, scores, and equations that stratify the impact of both modifiable (e.g., 6-min walk distance, functional class, brain natriuretic peptide), and non-modifiable (e.g., age, gender, PAH etiology) risk factors. Such tools allow physicians to better determine prognosis, allocate treatment resources, and enhance the consistency of treatment approaches across providers. Comprehensive and accurate risk prediction is essential to make individualized treatment decisions and optimizing outcomes in PAH.

Identifying Patients with Pulmonary Arterial Hypertension Using Administrative Claims Algorithms.

Retrospective administrative claims database studies provide real-world evidence about treatment patterns, healthcare resource use, and costs for patients and are increasingly used to inform policy-making, drug formulary, and regulatory decisions. However, there is no standard methodology to identify patients with pulmonary arterial hypertension (PAH) from administrative claims data. Given the number of approved drugs now available for patients with PAH, the cost of PAH treatments, and the significant healthcare resource use associated with the care of patients with PAH, there is a considerable need to develop an evidence-based and systematic approach to accurately identify these patients in claims databases. A panel of pulmonary hypertension clinical experts and researchers experienced in retrospective claims database studies convened to review relevant literature and recommend best practices for developing algorithms to identify patients with PAH in administrative claims databases specific to a particular research hypothesis.