[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.

Assessment of Knowledge, Attitude, and Practice Patterns in Pulmonary Arterial Hypertension among Cardiologists and Pulmonologists: Evidence from Turkey.

Background and Objectives: Pulmonary arterial hypertension (PAH) is a rare chronic disease of the small pulmonary arteries that causes right heart failure and death. Accurate management of PAH is necessary to decrease morbidity and mortality. Understanding current practices and perspectives on PAH is important. For this purpose, we intended to determine physicians' knowledge, attitudes, and practice patterns in adult pulmonary arterial hypertension (PAH) in Turkey. Materials and Methods: Between January and February 2022, an online questionnaire was sent via e-mail to all cardiologists and pulmonologists who were members of the Turkish Society of Cardiology (TSC) and the Turkish Thoracic Society (TTS). Results: A total of 200 physicians (122 pulmonologists and 78 cardiologists) responded to the questionnaire. Cardiologists were more frequently involved in the primary diagnosis and treatment of PAH than pulmonologists (37.2% vs. 23.8%, p = 0.042). More than half of the physicians had access to right heart catheterization. In mild/moderate PAH patients with a negative vasoreactivity test, the monotherapy option was most preferred (82.8%) and endothelin receptor antagonists (ERAs) were the most preferred group in these patients (73%). ERAs plus phosphodiesterase-5 inhibitors (PDE-5 INH) were the most preferred (69%) combination therapy, and prostacyclin analogues plus PDE-5 INH was preferred by only pulmonologists. Conclusions: Overall, clinical management of patients with PAH complied with guideline recommendations. Effective clinical management of PAH in specialized centers that having right heart catheterization achieve better outcomes.

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.

Right ventricular assessment in pulmonary hypertension.

PURPOSE OF REVIEW: The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis. RECENT FINDINGS: The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme. SUMMARY: Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.

Continuous long-term heart rate variability and risk assessment in pulmonary hypertension.

OBJECTIVE: Current multimodal risk assessment for pulmonary hypertension (PH) has been redefined with a simplified assessment for follow-up in the new European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. Follow-up risk assessment parameters include WHO functional class, 6 min walk test and N-terminal pro-brain natriuretic peptide. Although these parameters have prognostic implications assessment reflect data relating to specific time points. METHODS: Patients diagnosed with PH received an implantable loop recorder (ILR) to monitor daytime and night-time heart rate (HR), HR variability (HRV) and daily physical activity. Associations between the ILR measurements and established risk parameters were analysed using correlations, linear mixed models as well as logistical mixed models for addressing the ESC/ERS risk-score. RESULTS: 41 patients (median age: 56 years, range: 44-61.5 years) were included. Continuous monitoring had a median duration of 755 days (range: 343-1138 days), totalling 96 patient-years. In the linear mixed models, HRV and physical activity indexed by daytime HR (PAiHR) were significantly associated with the ERS/ERC risk parameters. In a logistical mixed model, HRV revealed a significant difference between 1-year mortality (<5% vs >5%) (p=0.027) with an OR of 0.82 for being in the group with 1-year mortality >5% for every increase by one HRV unit. CONCLUSIONS: Risk assessment in PH can be refined with continuous monitoring of HRV and PAiHR. These markers were associated with the ESC/ERC parameters. Our study with continuous risk stratification in PH demonstrated that a lower HRV predict worse prognosis.

The Effect of Perioperative Fluid Management and Operative Modifications on Mortality and Morbidity in Patients Undergoing Pulmonary Endarterectomy.

INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe disease treated with pulmonary endarterectomy. Our study aims to reveal the differences in liquid modalities and operation modifications, which can affect the patients' mortality and morbidity. METHODS: One hundred twenty-five patients who were diagnosed with CTEPH and underwent pulmonary thromboendarterectomy (PTE) at our center between February 2011 and September 2013 were included in this retrospective study with prospective observation. They were in New York Heart Association functional class II, III, or IV, and mean pulmonary artery pressure was > 40 mmHg. There were two groups, the crystalloid (Group 1) and colloid (Group 2) liquid groups, depending on the treatment fluids. P-value < 0.05 was considered statistically significant. RESULTS: Although the two different fluid types did not show a significant difference in mortality between groups, fluid balance sheets significantly affected the intragroup mortality rate. Negative fluid balance significantly decreased mortality in Group 1 (P<0.01). There was no difference in mortality in positive or negative fluid balance in Group 2 (P>0.05). Mean duration of stay in the intensive care unit (ICU) for Group 1 was 6.2 days and for Group 2 was 5.4 days (P>0.05). Readmission rate to the ICU for respiratory or non-respiratory reasons was 8.3% (n=4) in Group 1 and 11.7% (n=9) in Group 2 (P>0.05). CONCLUSION: Changes in fluid management have an etiological significance on possible complications in patient follow-up. We believe that as new approaches are reported, the number of comorbid events will decrease.

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.

Multiple Intravenous Bolus Dosing and Invasive Hemodynamic Assessment in a Hypoxia-Induced Mouse Pulmonary Artery Hypertension Model.

Pulmonary arterial hypertension (PAH) is a progressive life-threatening disease, primarily affecting small pulmonary arterioles of the lung. Currently, there is no cure for PAH. It is important to discover new compounds that can be used to treat PAH. The mouse hypoxia-induced PAH model is a widely used model for PAH research. This model recapitulates human clinical manifestations of PAH Group 3 disease and is an important research tool to evaluate the effectiveness of new experimental therapies for PAH. Research using this model often requires the administration of compounds in mice. For a compound that needs to be given directly into the bloodstream, optimizing intravenous (IV) administration is a key part of the experimental procedures. Ideally, the IV injection system should permit multiple injections over a set time course. Although the mouse hypoxia-induced PAH model is very popular in many laboratories, it is technically challenging to perform multiple IV bolus dosing and invasive hemodynamic assessment in this model. In this protocol, we present step-by-step instructions on how to carry out multiple IV bolus dosing via mouse jugular vein and perform arterial and right ventricle catheterization for hemodynamic assessment in mouse hypoxia-induced PAH model.

Surgical Repair of Truncus Arteriosus: A Long-Term Hemodynamic Assessment.

Background: Unrepaired truncus arteriosus (TA) carries poor prognosis due to complications of unrestricted pulmonary flow, truncal valve insufficiency, and pulmonary vascular disease. Currently, the hemodynamic profile of adults late after TA repair is unknown. We reviewed the hemodynamics, prevalence, and pathophysiology of pulmonary hypertension (PH) in this population. Methods: Eighteen adult patients with repaired TA who underwent cardiac catheterization at Mayo Clinic, MN, between 1997 and 2021 were identified. PH was defined as either precapillary (mean pulmonary artery pressure [mPAP] ≥25 mm Hg, pulmonary artery wedge pressure [PAWP] ≤15 mm Hg, and pulmonary vascular resistance [PVR] >3 Wood units), isolated postcapillary (mPAP ≥25, PAWP >15, PVR ≤3), or combined (mPAP ≥25, PAWP >15, and PVR >3). Diastolic pressure and transpulmonary gradients were used as ancillary data for classification. Results: Mean age at catheterization was 34 ± 10 years. Mean right ventricular (RV) systolic pressure was 82 ± 22.6 mm Hg, mean right and left mPAPs 28.1 ± 16.2 and 27.9 ± 11.9 mm Hg, respectively. Seven patients (41.2%) had PAWP >15 mm Hg and, among those undergoing arterial catheterization, 7 (53.8%) had a left ventricular (LV) end-diastolic pressure >15 mm Hg. PH was diagnosed in 13 patients (72.2%): 6 (33.3%) precapillary, 4 (22.2%) isolated postcapillary, and 3 (16.7%) combined. PAWP >15 mm Hg was associated with male sex (P = .049),

Cardiopulmonary exercise testing for treatment effect assessment in chronic thromboembolic pulmonary hypertension patients.

Aim      To determine possibilities of the cardiopulmonary stress test (CPST) as an unbiassed, noninvasive method for evaluation of the effect of managing patients with chronic thromboembolic pulmonary hypertension (CTEPH).Material and methods  This study included 37 patients with CTEPH, 24 men (mean age, 53±15 years) and 13 women (mean age, 58±8.5 years). The diagnosis was verified and theCoperability was assessed according to 2015 European Society of Cardiology Clinical Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension (PH). The surgical treatment was used in 65 % (n=24) of CTEPH patients: the group with pulmonary thromboendarterectomy constituted 35 % (n=13); the group with balloon pulmonary angioplasty 30% (n=11); and the conservative tactics was used in 27 % (n=10) of patients.Results Baseline CPST parameters significantly correlated with parameters of right heart catheterization (RHC): mixed venous oxygen saturation (SvO2) significantly positively correlated with V´O2peak (r=0.640, p<0.05), V´O2 / heart rate (HR) (r=0.557; p<0.001), PETCO2 peak (r=0.598, p<0.05), and V´E / V´CO2 (r=0.587; p<0.001); cardiac output (CO) correlated with V´O2 / HR (r=0.555, p<0.001), PETCO2peak (r= -0.476; p<0.05 and r=0.555, p<0.001 for ´E / V´CO2). In repeated testing, the physical working capacity (V´O2peak) increased only in patients after the surgical treatment of CTEPH. Importantly in this process, significant correlations remained between a number of CPST and RHC parameters: SvO2 correlated with V´O2peak (r=0.743; p<0.05), V´O2 /HR (r=0.627; p<0.001), PETCO2peak (r=0.538; p<0.05), and V´E / V´CO2 (r=0.597; p<0.001); V´O2 / HR, PETCO2peak, and V´E / V´CO2 significantly correlated with CO (r=0.645, p<0.001; r= -0.516, p<0.001, and r=0.555, p<0.001, respectively.Conclusion      CPST can be used as a noninvasive instrument for evaluation of the effect of CTEPH treatment, particularly in the absence of echocardiographic data for residual PH.

The Clinical and Cost Utility of Cardiac Catheterizations in Infants with Bronchopulmonary Dysplasia.

OBJECTIVE: To evaluate the cost-utility of catheterization-obligate treatment in preterm infants with pulmonary hypertension, as compared with empiric initiation of sildenafil based on echocardiographic findings alone. STUDY DESIGN: A Markov state transition model was constructed to simulate the clinical scenario of a preterm infant with echocardiographic evidence of pulmonary hypertension associated with bronchopulmonary dysplasia (BPD) and without congenital heart disease under consideration for the initiation of pulmonary vasodilator therapy via one of two modeled treatment strategies-empiric or catheterization-obligate. Transitional probabilities, costs and utilities were extracted from the literature. Forecast quality-adjusted life-years was the metric for strategy effectiveness. Sensitivity analyses for each variable were performed. A 1000-patient Monte Carlo microsimulation was used to test the durability of our findings. RESULTS: The catheterization-obligate strategy resulted in an increased cost of $10 778 and 0.02 fewer quality-adjusted life-years compared with the empiric treatment strategy. Empiric treatment remained the more cost-effective paradigm across all scenarios modeled through one-way sensitivity analyses and the Monte Carlo microsimulation (cost-effective in 98% of cases). CONCLUSIONS: Empiric treatment with sildenafil in infants with pulmonary hypertension associated with BPD is a superior strategy with both decreased costs and increased effectiveness when compared with catheterization-obligate treatment. These findings suggest that foregoing catheterization before the initiation of sildenafil is a reasonable strategy in preterm infants with uncomplicated pulmonary hypertension associated with BPD.

Incidence, Clinical Correlates, and Outcomes of Pulmonary Hypertension After Kidney Transplantation: Analysis of Linked US Registry and Medicare Billing Claims.

BACKGROUND: The incidence, risks, and outcomes associated with pulmonary hypertension (P-HTN) in the kidney transplant (KTx) population are not well described. METHODS: We linked US transplant registry data with Medicare claims (2006-2016) to investigate P-HTN diagnoses among Medicare-insured KTx recipients (N = 35 512) using billing claims. Cox regression was applied to identify independent correlates and outcomes of P-HTN (adjusted hazard ratio [aHR] 95%LCLaHR95%UCL) and to examine P-HTN diagnoses as time-dependent mortality predictors. RESULTS: Overall, 8.2% of recipients had a diagnostic code for P-HTN within 2 y preceding transplant. By 3 y posttransplant, P-HTN was diagnosed in 10.310.6%11.0 of the study cohort. After adjustment, posttransplant P-HTN was more likely in KTx recipients who were older (age ≥60 versus 18-30 y a HR, 1.912.403.01) or female (aHR, 1.151.241.34), who had pretransplant P-HTN (aHR, 4.384.795.24), coronary artery disease (aHR, 1.051.151.27), valvular heart disease (aHR, 1.221.321.43), peripheral vascular disease (aHR, 1.051.181.33), chronic pulmonary disease (aHR, 1.201.311.43), obstructive sleep apnea (aHR, 1.151.281.43), longer dialysis duration, pretransplant hemodialysis (aHR, 1.171.371.59), or who underwent transplant in the more recent era (2012-2016 versus 2006-2011: aHR, 1.291.391.51). Posttransplant P-HTN was associated with >2.5-fold increased risk of mortality (aHR, 2.572.843.14) and all-cause graft failure (aHR, 2.422.642.88) within 3 y posttransplant. Outcome associations of newly diagnosed posttransplant P-HTN were similar. CONCLUSIONS: Posttransplant P-HTN is diagnosed in 1 in 10 KTx recipients and is associated with an increased risk of death and graft failure. Future research is needed to refine diagnostic, classification, and management strategies to improve outcomes in KTx recipients who develop P-HTN.

Proposition of quantitative parameters for pre- and early post-operative assessment in pulmonary thromboendarterectomy - An Indian prospective study.

INTRODUCTION: Chronic thromboembolic pulmonary hypertension results from the incomplete resolution of the vascular obstruction associated with pulmonary embolism. Symptoms are exertional dyspnoea and fatigue, and over a period of time, right ventricular dysfunction sets in. Pulmonary thromboendarterectomy is an effective surgical remedy for this condition. Our study is an initial post-operative experience of pulmonary thromboendarterectomy and we have also tried to formulate quantitative parameters for the prediction of the post-operative course in patients who are undergoing surgery. METHODS: Twenty patients with chronic thromboembolic pulmonary hypertension underwent pulmonary thromboendarterectomy between July 2017 and January 2020. Pre-operatively, each patient was subjected to the (i) 6-min walk test, (ii) pre-operative brain natriuretic peptide values and (iii) pulmonary artery systolic pressure. Following the surgery and subsequent discharge, the patients were followed up at intervals of 15 days, 1, 3, 6, 9 months and at 1 year. At one year post-operatively, the same three quantitative tests were performed on each subject. RESULTS: Post-operatively, the mean 6-min walk distance was 499.75 m as against 341.35 m pre-operatively (p < 0.0001). Mean brain natriuretic peptide was 8.69 pm/l as against 47.58 pm/l pre-operatively (p < 0.0001). Mean pulmonary artery systolic pressure was 22.25 as against 67.1 pre-operatively (p < 0.0001). CONCLUSION: 6-Min walk test, brain natriuretic peptide and pulmonary artery systolic pressure could be considered as useful predictors of the haemodynamic severity of disease and predict the post-operative outcome.

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.

Assessment of pulmonary arterial stiffness in patients with systemic sclerosis without overt pulmonary hypertension.

Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and complicate most systemic diseases. Systemic sclerosis (SSc), represents the leading cause of connective tissue disease (CTD) associated with PAH. Although SSc is a rare disease, it is associated with higher morbidity and early mortality than other rheumatological diseases due to developing SSc-associated interstitial pulmonary disease (ILD) and/or pulmonary arterial hypertension (PAH). The impact of the early diagnosis on the prognosis is evident. In this context, in our study, we aimed to investigate the early changes in pulmonary vascular bed by measuring pulmonary arterial stiffness (PAS) in SSc patients without overt PAH. Sixty-two SSc patients and fifty-eight gender and age-matched, healthy subjects enrolled in this cross-sectional observational study. SSc patients were evaluated in terms of disease duration and severity. Modified rodnan skin score (mRSS) was calculated as disease severity index. Echocardiographic parameters were assessed and compared to the control group. Right ventricular (RV) diameters, systolic pulmonary artery pressure (sPAP), and right ventricle myocardial performance index (RV-MPI) were significantly higher in the SSc group compared to the control group (p < 0.05). Tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) were significantly lower in the SSc group compared to the control group (p < 0.05). PAS value (25.5 ± 9.2 kHz/ms vs. 18.1 ± 7.4 kHz/ms, p < 0.001) was significantly higher in the SSc group than in the control group. A statistically significant positive correlation relationship was detected between the PAS value and CRP, ESR, disease duration, mRSS. According to these results, in SSc patients, PAS as an inexpensive and easily applicable echocardiographic method might serve as a marker of early detection of PAH.

[Morphological assessment of pulmonary vessels in pulmonary arterial hypertension associated with congenital heart disease]. / Morfologicheskaya otsenka sosudov legkikh pri legochnoi gipertenzii, obuslovlennoi vrozhdennymi porokami serdtsa.

Pulmonary vascular remodeling is the key structural alteration in pulmonary arterial hypertension associated with congenital heart disease (CHD). Changes in the pulmonary vessels in CHD generally occur in the inner and middle coats. This review considers the pathology of these changes and highlights some issues of a stereological approach to the morphometry of pulmonary vasculature. It also touches upon the issues of the morphology of pulmonary vessels in post-capillary and segmental pulmonary hypertension.

Serial right heart catheter assessment between balloon pulmonary angioplasty sessions identify procedural factors that influence response to treatment.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is delivered as a series of treatments for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) however, there is little published data on the procedural determinants of outcome. METHODS: Pre- and post-BPA clinical and hemodynamic data, as well as serial hemodynamic and procedural data at each BPA session were evaluated to determine patient and procedure-related factors that influence hemodynamic response. RESULTS: Per procedure data from 210 procedures in 84 patients and per patient data from 182 procedures in 63 patients with completed treatment and 3-month follow-up were analyzed. A median of 3 (range 1-6) BPA procedures treating a median of 2 segments per procedure (range 1-3) were performed per patient with a median interval between procedures of 42 (range 5-491) days. Clinical outcome correlated with hemodynamic change (pulmonary vascular resistance [ΔPVR] vs Cambridge Pulmonary Hypertension Outcome Review [CAMPHOR] symptom score: p < 0.001, Pearson's r = 0.48, n = 49). Responders to BPA had more severe disease at baseline and 37.5 % of non-responders were post-PEA. There was a dose-response relationship between per procedure and total number of segments treated and hemodynamic improvement (ΔPVR: 1 segment: -0.9%, 2: -14.5%, 3 or more: -16.1%, p < 0.001). Treating totally occluded vessels had a greater hemodynamic effect (mean pulmonary artery pressure [ΔmPAP]: sessions with occlusion: -8.0%, without occlusion treated: -3.2%, p < 0.05) without an increased complication rate. CONCLUSIONS: The magnitude of clinical benefit is related to the hemodynamic effect of BPA which in turn is related to the number of segments treated and lesion severity. Patients who were post-PEA were less likely to respond to BPA.