[Assessment of risk factors for bronchopulmonary dysplasia with pulmonary hypertension and construction of a prediction nomogram model].

Objective: To explore the risk factors of pulmonary hypertension (PH) in premature infants with bronchopulmonary dysplasia (BPD), and to establish a prediction model for early PH. Methods: This was a retrospective cohort study. Data of 777 BPD preterm infants with the gestational age of <32 weeks were collected from 7 collaborative units of the Su Xinyun Neonatal Perinatal Collaboration Network platform in Jiangsu Province from January 2019 to December 2022. The subjects were randomly divided into a training cohort and a validation cohort at a ratio of 8∶2 by computer, and non-parametric test or χ2 test was used to examine the differences between the two retrospective cohorts. Univariate Logistic regression and multivariate logistic regression analyses were used in the training cohort to screen the risk factors affecting the PH associated with BPD. A nomogram model was constructed based on the severity of BPD and its risk factors,which was internally validated by the Bootstrap method. Finally, the differential, calibration and clinical applicability of the prediction model were evaluated using the training and verification queues. Results: A total of 130 among the 777 preterm infants with BPD had PH, with an incidence of 16.7%, and the gestational age was 28.7 (27.7, 30.0) weeks, including 454 males (58.4%) and 323 females (41.6%). There were 622 preterm infants in the training cohort, including 105 preterm infants in the PH group. A total of 155 patients were enrolled in the verification cohort, including 25 patients in the PH group. Multivariate Logistic regression analysis revealed that low 5 min Apgar score (OR=0.87, 95%CI 0.76-0.99), cesarean section (OR=1.97, 95%CI 1.13-3.43), small for gestational age (OR=9.30, 95%CI 4.30-20.13), hemodynamically significant patent ductus arteriosus (hsPDA) (OR=4.49, 95%CI 2.58-7.80), late-onset sepsis (LOS) (OR=3.52, 95%CI 1.94-6.38), and ventilator-associated pneumonia (VAP) (OR=8.67, 95%CI 3.98-18.91) were all independent risk factors for PH (all P<0.05). The independent risk factors and the severity of BPD were combined to construct a nomogram map model. The area under the receiver operating characteristic (ROC) curve of the nomogram model in the training cohort and the validation cohort were 0.83 (95%CI 0.79-0.88) and 0.87 (95%CI 0.79-0.95), respectively, and the calibration curve was close to the ideal diagonal. Conclusions: Risk of PH with BPD increases in preterm infants with low 5 minute Apgar score, cesarean section, small for gestational age, hamodynamically significant patent ductus arteriosus, late-onset sepsis, and ventilator-associated pneumonia. This nomogram model serves as a useful tool for predicting the risk of PH with BPD in premature infants, which may facilitate individualized early intervention.

Burden of pulmonary hypertension due to chronic obstructive pulmonary disease: Analysis of exacerbations and healthcare resource utilization in the United States.

BACKGROUND: The burden of pulmonary hypertension (PH) among patients with chronic obstructive pulmonary disease (COPD) is not well understood. The present retrospective cohort study aimed to quantify the clinical and economic burden of PH in patients with COPD. METHODS: Adults with COPD were retrospectively identified in the Optum® Clinformatics® Data Mart between July 1, 2016 and June 30, 2021. Those diagnosed with PH were assigned to the PH-COPD cohort and those without a diagnosis of PH were assigned to the COPD cohort. Outcomes, including the number of visits for exacerbations and all-cause and COPD-related healthcare resource utilization (HCRU) and costs per patient per month (PPPM), were compared between cohorts. Baseline and study outcomes were analyzed descriptively. For significance testing, continuous variables were analyzed using Student's t-tests and categorical variables were analyzed using Chi-square tests. RESULTS: A total of 1627 patients with PH-COPD were matched 1:1 to COPD patients without PH. A greater percentage of PH-COPD patients experienced COPD exacerbations vs. the COPD cohort (p < 0.001) and the PH-COPD cohort had more total (p < 0.001) and severe exacerbation-related visits PPPM (p < 0.001). All-cause and COPD-related HCRU PPPM estimates were higher among the PH-COPD cohort vs. the COPD cohort (p < 0.01). Total all-cause (p < 0.001) and COPD-related costs (p < 0.001) were higher among PH-COPD patients than COPD patients. CONCLUSIONS: Patients with PH-COPD had higher rates of severe exacerbations, hospitalizations, and costs compared to COPD patients without PH, underscoring the need for targeted therapies to prevent and manage PH in patients with COPD.

Impact of cancer on the prevalence, management, and outcome of patients with chronic thromboembolic pulmonary hypertension.

INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved thrombotic obstruction of the pulmonary vasculature. Cancer is a known risk factor for CTEPH. This study aimed to determine the impact of cancer on the prevalence, management, and outcomes of patients with CTEPH. MATERIALS AND METHODS: In this retrospective study involving 99 patients sequentially diagnosed with CTEPH in our hospital, the prevalence of 10 comorbid conditions including a past history of cancer at the time of CTEPH diagnosis were calculated. RESULTS: Among the 99 patients, 17 (17%) had a history of cancer. Breast cancer (n = 6) was the most common cancer type, followed by gastrointestinal cancer (n = 3), uterine cancer (n = 2), and malignant lymphoma (n = 2). Between patients with and without cancer, there were no differences in the demographics, severity of CTEPH, and management; however, the 5-year survival rate was lower for patients with cancer (65%) than for those without (89%). In addition, patients with cancer had significantly worse survival than those without (p = 0.03 by log-rank test). During follow-up, nine patients developed cancer after the diagnosis of CTEPH. Among the 99 patients, 13 died during follow-up, 6 (46%) of whom died of cancer. CONCLUSIONS: 17% of our patients with CETPH were diagnosed with cancer, with breast and gastrointestinal tract cancers being the most common. Cancer comorbidity was associated with a poor prognosis and contributed to death in 46% of deceased patients. The impact of cancer on CTEPH should be further evaluated in the future.

COVID-19 Patients With Pulmonary Hypertension Hospitalized in the United States During the Early Pandemic: Analysis of In-Hospital Mortality, Clinical Outcomes, and Racial Disparities.

Coronavirus Disease 2019 (COVID-19) has been linked to severe consequences among hospitalized patients diagnosed with pulmonary hypertension (PH), as evidenced by a limited number of studies. Our retrospective study employed the National Inpatient Sample (NIS) database to evaluate in-hospital mortality and various clinical outcomes in COVID-19 patients with and without PH. This study included all patients ages 18 years and above who were hospitalized in the United States from January 1,2020 to December 31, 2020 with a COVID-19 diagnosis. The patients were then divided into 2 cohorts based on their PH status. After multivariate adjustment, we discovered that COVID-19 patients with PH experienced considerably higher in-hospital mortality, longer hospital stays, and higher costs of hospitalization when compared to COVID-19 patients without PH. Moreover, we observed an increased dependence on invasive and noninvasive positive pressure ventilation among COVID-19 patients with PH, indicating more severe respiratory failure. Our findings suggest that COVID-19 patients with PH had a heightened risk of acute pulmonary embolism and myocardial infarction while hospitalized. Lastly, among COVID-19 patients with PH, Hispanic and Native American patients demonstrated a persistently higher risk of in-hospital mortality compared to other racial groups. To our knowledge, this is the most comprehensive study of outcomes for COVID-19 patients with PH. The observed inpatient mortality appears to be driven by in-hospital complications, particularly pulmonary embolism. Given the substantial mortality and complications associated with COVID-19 and PH, we advocate for SARS-CoV-2 vaccination and the implementation of aggressive nonpharmacological preventive measures.

Clinical assessment for pulmonary hypertension in interstitial lung disease.

BACKGROUND: Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD), as its development confers a poor prognosis. There are no specific recommendations for methods of assessment for PH in ILD populations. AIMS: To determine current assessment practices for PH in an Australian ILD centre. METHODS: In the Austin Health ILD database, 162 consecutive patients with idiopathic pulmonary fibrosis or connective tissue disease-associated ILD were identified and retrospectively evaluated for methods of PH assessment with transthoracic echocardiography (TTE), serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and right heart catheterisation (RHC) in relation to patient demographic and physiological parameters. RESULTS: The median follow-up was 30 (14.4-56.4) months. At baseline, vital capacity was 80.0 ± 18.4% predicted, and diffusing capacity for carbon monoxide was 59.6 ± 15.2% predicted. Evaluation for PH was performed in 147 (90.7%) patients, among whom 105 (64.8%) had TTE performed at least once. At the initial TTE, 33.7% patients had high probability of PH, defined as RVSP >40 mmHg + RAp and/or right ventricular dysfunction. At the time of the most recent TTE, these criteria were met in 45 (52.3%) patients. Elevated serum NT-proBNP levels during the first year were observed in 47 (38.8%) patients. Only 14 (8.6%) patients had RHC. CONCLUSION: Our institutional PH assessment practice in ILD demonstrates a substantial prevalence of probable PH at baseline. As new therapies emerge for the treatment of PH in ILD, well-defined screening practices are important in this population for early identification and optimal management.

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.

Five year risk assessment and treatment patterns in patients with chronic thromboembolic pulmonary hypertension.

AIMS: Repeated risk assessments and treatment patterns over long time are sparsely studied in chronic thromboembolic pulmonary hypertension (CTEPH); thus, we aimed to investigate changes in risk status and treatment patterns in incident patients with CTEPH over a 5 year period. METHODS AND RESULTS: Descriptive and explorative study including 311 patients diagnosed with CTEPH 2008-2019 from the Swedish pulmonary hypertension registry, stratified by pulmonary endarterectomy surgery (PEA). Risk and PH-specific treatment were assessed in surgically treated (PEA) and medically treated (non-PEA) patients at diagnosis and up to 5 years follow-up. Data are presented as median (Q1-Q3), count or per cent. Prior to surgery, 63% in the PEA-group [n = 98, age 64 (51-71) years, 37% female] used PH-specific treatment and 20, 69, and 10% were assessed as low, intermediate or high risk, respectively. After 1 year post-surgery, 34% had no PH-specific treatment or follow-up visit registered despite being alive at 5 years. Of patients with a 5 year visit (n = 23), 46% were at low and 54% at intermediate risk, while 91% used PH-specific treatment. In the non-PEA group [n = 213, age 72 (65-77) years, 56% female], 28% were assessed as low, 61% as intermediate and 11% as high risk. All patients at high risk versus 50% at low risk used PH-specific treatment. The 1 year mortality was 6%, while the risk was unchanged in 57% of the patients; 14% improved from intermediate to low risk, and 1% from high to low risk. At 5 years, 27% had a registered visit and 28% had died. Of patients with a 5 year visit (n = 58), 38% were at low, 59% at intermediate and 1% at high risk, and 86% used PH-specific treatment. CONCLUSIONS: Risk status assessed pre-surgery did not foresee long-term post-PEA risk and pre-surgery PH-specific treatment did not foresee long-term post-PEA treatment. Medically treated CTEPH patients tend to remain at the same risk over time, suggesting a need for improved treatment strategies in this group.

Pulmonary hypertension in infants with bronchopulmonary dysplasia: risk factors, mortality and duration of hospitalisation.

OBJECTIVES: Pulmonary hypertension (PH) is a complication of bronchopulmonary dysplasia (BPD) and associated with increased mortality and morbidity. Our aim was to identify, in infants with BPD, the effect of PH on health-care utilisation and health related cost of care. METHODS: An electronic data recording system was used to identify infants ≤32 weeks of gestation who developed BPD. PH was classified as early (≤28 days after birth) or late (>28 days after birth). RESULTS: In the study period, 182 infants developed BPD; 22 (12.1%) developed late PH. Development of late PH was associated with a lower gestational age [24.6 (23.9-26.9) weeks, p=0.001] and a greater need for positive pressure ventilation on day 28 after birth (100%) compared to infants without late PH (51.9%) (odds ratio (OR) 19.5, 95% CI: 2.6-148), p<0.001. Late PH was associated with increased mortality (36.4%) compared those who did not develop late PH (1.9%) after adjusting for gestational age and ventilation duration (OR: 26.9, 95% CI: 3.8-189.4), p<0.001. In infants who survived to discharge, late PH development was associated with a prolonged duration of stay [147 (118-189) days] compared to the infants that did not develop late PH [109 (85-149) days] (p=0.03 after adjusting for gestational age). Infants who had late PH had a higher cost of stay compared to infants with BPD who did not develop late PH (median £113,494 vs. £78,677, p=0.016 after adjusting for gestational age). CONCLUSIONS: Development of late PH was associated with increased mortality, a prolonged duration of stay and higher healthcare cost.

Risk assessment in pulmonary hypertension based on routinely measured laboratory parameters.

BACKGROUND: γ-glutamyl transferase (GGT), the aspartate aminotransferase/alanine aminotransferase (AST/ALT) ratio, and the neutrophil-to-lymphocyte ratio (NLR) are prognostic biomarkers in several cardiovascular diseases, but their relevance in pulmonary hypertension (PH) is not fully understood. We aimed to assess their prognostic value in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). METHODS: We retrospectively analyzed 731 incident patients with idiopathic PAH or CTEPH who entered the Giessen PH registry during 1993-2019. A risk stratification score based on GGT, AST/ALT ratio, and NLR tertiles was compared with a truncated version of the European Society of Cardiology/European Respiratory Society (ESC/ERS) risk stratification scheme. Associations with survival were evaluated using Kaplan-Meier and Cox regression analyses. External validation was performed in 311 patients with various types of PAH or CTEPH from a second German center. RESULTS: GGT levels, AST/ALT, and NLR independently predicted mortality at baseline and during follow-up. The scoring system based on these biomarkers predicted mortality at baseline and during follow-up (both log-rank p < 0.001; hazard ratio [95% confidence interval], high vs low risk: baseline, 7.6 [3.9, 15.0]; follow-up, 13.3 [4.8, 37.1]). Five-year survival of low, intermediate, and high risk groups was 92%, 76%, and 51%, respectively, at baseline and 95%, 78%, and 50%, respectively, during follow-up. Our scoring system showed characteristics comparable to the ESC/ERS scheme, and predicted mortality in the validation cohort. CONCLUSION: GGT, AST/ALT, and NLR were reliable prognostic biomarkers at baseline and during follow-up, with predictive power comparable to the gold standard for risk stratification.

Healthcare utilization and economic burden in systemic sclerosis: a systematic review.

OBJECTIVE: Systemic sclerosis (SSc) is characterized by vasculopathy, fibrosis of skin and internal organs, and autoimmunity with complications including interstitial lung disease, pulmonary hypertension, and digital ulcers with substantial morbidity and disability. Patients with SSc may require considerable healthcare resources with economic impact. The purpose of this systematic review was to provide a narrative synthesis of the economic impact and healthcare resource utilization associated with SSc. METHODS: MEDLINE and EMBASE were searched from inception to 20 January 2021. Studies were included if they provided information regarding the total, direct and indirect cost of SSc. The cost of SSc subtypes and associated complications was determined. Risk of bias assessments through the Joanna Briggs Institute cross-sectional and case series checklists, and the Newcastle-Ottawa Cohort and Case-Control study scales were performed. A narrative synthesis of included studies was planned. RESULTS: The number of publications retrieved was 1778, of which 34 were included representing 20 cross-sectional, 11 cohort, and three case-control studies. Studies used various methods of calculating cost including prevalence-based cost-of-illness approach and health resource units cost analysis. Overall SSc total annual cost ranged from USD $14 959 to $23 268 in USA, CAD $10 673 to $18 453 in Canada, €4607 to €30 797 in Europe, and AUD $7060 to $11 607 in Oceania. Annual cost for SSc-associated interstitial lung disease and pulmonary hypertension was USD $31 285-55 446 and $44 454-63 320, respectively. CONCLUSION: Cost-calculation methodology varied greatly between included studies. SSc represents a significant patient and health resource economic burden. SSc-associated complications increase economic burden and are variable depending on geographical location and access.

Comparison of In-Hospital Outcomes of Transcatheter Mitral Valve Repair in Patients With vs Without Pulmonary Hypertension (From the National Inpatient Sample).

Pulmonary hypertension (PH) is common in patients with left heart disease and is present in varying degrees in patients with severe mitral valve disease. There is paucity of data regarding outcomes following transcatheter mitral valve repair (TMVr) in patients with PH. For this study, we analyzed NIS data from 2014 to 2018 using the ICD-9-CM and 10-CM codes. Baseline characteristics were compared using a Pearson chi-squared test for categorical variables and independent samples t-test for continuous variables. To account for selection bias, a 1:1 propensity match cohort was derived using logistic regression. Trend analysis was- done using linear regression. Of 21,505 encounters, 6780 encounters had PH. 6610 PH encounters were matched with 6610 encounters without PH. In-hospital mortality (3.3% versus 1.9%, p <0.01) was higher in PH population. Complications such as blood transfusion (3.6% versus 1.7%, p <0.01), GI bleed (1.4% versus 1%, p = 0.04), vascular complications (5.3% versus 3.3%, p <0.01), vasopressors use (2.9% versus 1.7%, p <0.01) and pacemaker placement (1.3% versus 0.8%, p = 0.01) remained significantly higher for encounters with PH. Multiple Logistic regression showed PH was associated with higher mortality (adjusted odds ratio [AOR], 1.68 [95% confidence interval [CI], 1.39-2.05], p <0.01). The mean length of stay (6.2 versus 5.3 days, p <0.01) and cost per hospitalization ($53,780 versus $50,801, p <0.01) remained significantly higher in the PH group when compared to group without PH. In conclusion, TMVr in PH as compared to without PH is associated with higher mortality, post-procedure complication rates, length of stay, and cost of stay.

The Evolution of Risk Assessment in Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a chronic debilitating disease that carries an unacceptably high morbidity and mortality rate despite improved survival with modern therapies. The combination of several modifiable and nonmodifiable variables yields a robust risk assessment across various available clinical calculators. The role of risk calculation is integral to managing PAH and aids in the timely referral to expert centers and potentially lung transplantation. Studies are ongoing to determine the role of risk calculators in the framework of clinical trials and to elucidate novel markers of high risk in PAH.

Mortality, Kidney Failure, and Hospitalization Among Medicare Beneficiaries With CKD and Pulmonary Hypertension.

RATIONALE & OBJECTIVE: Pulmonary hypertension (PH) is highly prevalent among patients with chronic kidney disease (CKD) not requiring kidney replacement therapy. We studied the associations of PH with mortality, kidney failure, as well as cardiovascular (CV) and non-CV hospitalization among Medicare beneficiaries with a CKD diagnosis. STUDY DESIGN: Retrospective, observational study using a matched cohort design. SETTING & PARTICIPANTS: Patients with PH (based on 2 claims within 2 years) and patients without PH matched on CKD stage from the Medicare 5% CKD sample (1996-2016). PREDICTOR: Presence of pulmonary hypertension. OUTCOME: Mortality, kidney failure, and all-cause, CV, and non-CV hospitalization. ANALYTICAL APPROACH: Cox proportional hazards models to assess the association between PH and mortality, adjusting for age, sex, race, and comorbidities. Death was considered as a competing event in Fine-Gray models to assess the association between PH and kidney failure. Negative binomial model was used to evaluate the relationship between PH and all-cause, CV, and non-CV hospitalizations. RESULTS: 30,052 patients with PH and CKD and 150,260 CKD stage-matched patients without diagnosed PH were studied. The median age of the study population was 80.7 years, 57.8% were women, and 10.3% were African Americans. The presence of PH was associated with an increased risk of mortality after 1 (HR, 2.87 [95% CI, 2.79-2.95]), 2-3 (HR, 1.56 [95% CI, 1.51-1.61]), and 4-5 (HR, 1.47 [95% CI, 1.40-1.53]) years of follow-up, and a higher risk of all-cause, CV, and non-CV hospitalization during the same period. PH was also associated with kidney failure in after 1 and 2-3 years but not after 4-5 years of follow-up evaluation. Patients with PH also experienced higher rates of acute kidney injury (AKI), and AKI requiring dialysis support within 30 and 90 days of AKI. LIMITATIONS: Reliance on billing codes and lack of echocardiogram or right heart catheterization data CONCLUSIONS: Among older Medicare beneficiaries with a CKD diagnosis not requiring kidney replacement therapy, the presence of PH was associated with an increased risk of mortality, kidney failure, and hospitalization. Understanding of the mechanism of these associations, especially the increased risk of kidney failure, requires further study.

Assessment of cardiac arrhythmias using long-term continuous monitoring in patients with pulmonary hypertension.

BACKGROUND: Cardiac arrhythmias are considered a prominent phenomenon in patients with pulmonary hypertension (PH). Older studies reported that 8% to 35% of patients with PH had supraventricular tachycardia (SVT), associated with adverse outcomes. Still, these arrhythmias have only been investigated via short-term monitoring or limited electrocardiogram recordings. METHODS: Patients without previous arrhythmias diagnosed with PH at a tertiary facility received an insertable cardiac monitor as part of a prospective cohort study. Baseline assessments included World Health Organization functional class, six-minute walk test, echocardiography, and cardiac magnetic resonance imaging. RESULTS: Thirty-four patients with PH were included. Twenty-four patients had pulmonary arterial hypertension (PAH) and 10 had chronic thromboembolic PH (CTEPH). During 46 patient-years of continuous monitoring (median: 594 (range: 334-654) days per patient), 70 arrhythmia episodes were recorded in 13 patients (38%), with a median of two (range: 1-3) episodes and an arrhythmic burden median of 1.6 (range: 0.1-228) minutes per patient. SVTs were the most common arrhythmias, with 16% of episodes being atrial fibrillation and 84% being other types of SVTs. Additionally, three patients experienced bradycardias, including one resulting in syncope and subsequent pacemaker implantation. None of the patients had sustained ventricular arrhythmias. CONCLUSIONS: Arrhythmias were seen in 38% of contemporary patients with PH during long-term continuous monitoring; however, the vast majority of episodes were short and self-limiting. Modern therapy may alleviate the development of arrhythmias in stable patients with PH. This study is the first study to deploy long-term continuous monitoring in patients with PH.

Preoperative Assessment and Perioperative Management of the Patient with Pulmonary Vascular Disease.

The incidence of pulmonary hypertension (PH) in patients undergoing noncardiac surgery has increased steadily over the past decade. Patients with known PH have significantly higher perioperative morbidity and mortality than those without PH. Moreover, a substantial number of patients may have occult disease. It, therefore, is of paramount importance for perioperative providers to recognize high-risk patients and treat them appropriately. This review first provides an overview of PH pathophysiology, then estimates the perioperative incidence of PH and its impact on surgical outcomes, and finally outlines a perioperative management strategy.

Thirty-day hospital readmission in systemic sclerosis associated pulmonary hypertension: A nationwide study.

OBJECTIVE: To identify risk-factors for 30-day hospital readmission in systemic sclerosis pulmonary hypertension (SSc-PH) and to compare trends and characteristics of 30-day readmissions in SSc-PH versus non-SSc pulmonary arterial hypertension (non-SSc PAH). METHODS: In this retrospective study, we identified SSc-PH and non-SSc PAH hospitalizations using ICD-9 codes within the Healthcare Cost and Utilization Project-National Readmission Database. Thirty-day readmission rates were calculated between 2010 and 2015. Characteristics were compared using chi-square, Wilcoxon rank-sum, or two-sample t-tests between (A) SSc-PH patients with versus without readmission and (B) patients with ≥1 readmission with SSc-PH versus non-SSc PAH. Adjusted logistic regression models were generated for readmission in SSc-PH. RESULTS: 4,846 of 22,420 (22%) with SSc-PH and 10,573 of 49,254 (21%) with non-SSc PAH had ≥1 30-day readmission. Between 2010-2015, readmission rate decreased in non-SSc PAH (23% to 20%; p<0.001) and was unchanged in SSc-PH (23% to 23%; p = 0.77). In SSc-PH, independent predictors of 30-day readmission include male sex, age <60, Medicare or Medicaid, higher Charlson/Deyo comorbidity index, and congestive heart failure (CHF). A higher proportion of patients with SSc-PH (vs. non-SSc PAH) died during index hospitalizations (p = 0.001) and readmissions (p <0.001). Readmitted patients with SSc-PH (vs. non-SSc PAH) were younger and less often had CHF. In SSc-PH, the most common readmission primary diagnosis was infection, followed by respiratory and heart failure. CONCLUSION: In SSc-PH, 30-day readmission is frequent, and in-hospital deaths occur at a higher rate compared to those with non-SSc PAH. This study identifies factors that may characterize those with SSc-PH at highest risk for readmission.