Objective To identify predictive factors associated with mortality in connective tissue disease‐associated pulmonary arterial hypertension (CTD‐PAH) patients who were complicated with right heart failure (RHF). Methods In this single‐center retrospective study, baseline demographics, clinical features, laboratory results, and hemodynamic assessments were collected. Kaplan–Meier analysis was applied to analyze all‐cause mortality. Univariate and forward stepwise multivariate Cox proportional regression analyses were performed to identify independent predictors of mortality. Results A total of 51 right heart catheterization‐confirmed CTD‐PAH patients complicated with RHF were consecutively enrolled in this study from 2012 to 2022. Forty‐eight (94%) enrolled patients were female and the mean age was 36.0 ± 11.8 years. Thirty‐two (61.5%) were systemic lupus erythematosus‐PAH and 33%/67% showed World Health Organization functional class III/IV, respectively. Twenty‐five (49%) of those patients died and Kaplan–Meier analysis showed the overall 1‐, 3‐, and 5‐week survival rates from the time of hospitalization as 86.28%, 60.78%, and 56.86%, respectively. RHF in CTD‐PAH patients mainly resulted from progression of PAH (n = 19) and infection (n = 5), which also contributed to the leading causes of death. Statistical analysis between survivors and non‐survivors showed that death from RHF was associated with higher levels of urea (9.66 vs 6.34 mmol/L, P = 0.002), lactate (cLac: 2.65 vs 1.9 mmol/L, P = 0.006), total bilirubin (23.1 vs 16.9 μmol/L, P = 0.018) and direct bilirubin (10.5 vs 6.5 μmol/L, P = 0.004), but with lower levels of hematocrit (33.7 vs 39, P = 0.004), cNa+ (131 vs 136 mmol/L, P = 0.003). Univariate and forward stepwise multivariate Cox proportional regression analyses indicated that the level of cLac (hazards ratio:1.297; 95% CI: 1.076–1.564; P = 0.006) was an independent risk factor for mortality. Conclusion The short‐term prognosis of CTD‐PAH complicated with RHF was very poor, and hyperlactic acidemia (cLac > 2.85 mmoL/L) was an independent predicting factor for mortality of CTD‐PAH patients complicated with RHF.

Objective Connective tissue disease associated pulmonary hypertension (CTD-PH) is classified as a subgroup of WHO group 1 PH, also called pulmonary arterial hypertension (PAH). However, not all CTD-PH fit hemodynamic definition of PAH. This study investigates the diversity of hemodynamic types of CTD-PH, their differences in clinical characteristics and outcomes. Method We performed a retrospective cohort study. CTD-PH patients were enrolled and divided into WHO group1 PH, WHO group 2 PH and hyperdynamic PH (mPAP > 20 mmHg, PVR < 3WU, PAWP < 15 mmHg) according to hemodynamics obtained by right heart catheterization. Patients with severe lung diseases, heart failure with reduced ejection fraction, pulmonary embolism, and hepatic cirrhosis were excluded. Baseline characteristics, autoantibodies, cardiac function, echocardiogram parameters, hemodynamics and survival rates were compared. Result A total of 202 CTD-PH patients were included, 138 in WHO group 1 PH, 33 in WHO group 2 PH and 31 in hyperdynamic PH. We found hyperdynamic PH is less severe, presenting lower NT-proBNP level, better WHO function class, lower mPAP and PVR, higher cardiac output, and less cardiac remodeling. Incidence of anti-RNP was significantly lower in patients with elevated PAWP. Short-term survival was worse in WHO group 2 PH, yet 5-year survival rates didn’t differ between groups. Conclusion Considering diversity in hemodynamic types, CTD-PH is more than a subgroup of PAH. Different types of CTD-PH present different clinical phenotypes and outcome. Phenotyping PH in CTD-PH patients is important.

Backgrounds The EmPHasis-10 questionnaire is a disease-specific quality of life (QoL) measurement in patients with pulmonary hypertension. We report the results of cross-cultural validation of the Chinese version of the EmPHasis-10 and its relationship with risk stratification in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). Methods The Emphasis-10 was administered to 75 CTD-PAH patients along with the 36-item Medical Outcomes Study Short Form Survey (SF-36) and EuroQol five dimensions questionnaire (EQ-5D). The diagnosis of PAH was confirmed by right heart catheterization. Demographic and clinical data were obtained. Multivariable logistic regression was conducted based on the low risk profile assessed by a 4-strata risk assessment model (COMPERA 2.0) at follow-up. Results Date from 75 patients with CTD-PAH were analysed. The EmPHasis-10 demonstrated satisfactory reliability (Cronbach α = 0.95) and convergent validity showed by the significant relationship with WHO Functional Class (P = 0.003), SF-36 (P < 0.001) and EQ-5D (P = 0.002). EmPHasis-10 was significantly associated with achieving the low risk profile at 12 months of follow-up (Odds ratio: 0.928, P = 0.029) after adjusting for WHO Functional Class. Conclusion EmPHasis-10 has acceptable reliability and validity in CTD-PAH patients and may serve as an additional parameter in risk stratification.

Background: Mini-fluid challenge may predict fluid responsiveness and limit fluid overload. This study was designed to explore the minimal infusion volume in effectively predicting fluid responsiveness in septic shock patients.Methods: ICU septic shock patients with indwelling pulmonary artery catheter received five sequential intravenous boluses of 100 mL 4% gelatin. Cardiac output was measured with thermodilution before fluid challenge (baseline) and three minutes after each bolus. Fluid responsiveness (FR) was defined as an increase in CO greater than 10% after 500 mL fluid infusion. The smallest volume which can perform an effective fluid challenge was analyzed. Results: Forty-seven patients were included in this prospective study. After 500 mL volume expansion, thirty-six patients presented with FR (77%) and 11 patients were fluid nonresponders. A mini fluid of 100 mL colloid had a poor predictive value (AUC = 0.67, p > 0.05). The minimal volume required to predict FR in a mini fluid challenge was 200 mL. An increase in CO greater than 5.2% after 200 mL colloid infusion was able to predict FR with a sensitivity of 83.3% and specificity of 90.9%. The AUC under the ROC curve was 0.93 (95% CI: 0.84 – 1, p < 0.05). Conclusion: In septic shock patients, a minimal volume of 200 mL 4% gelatin could reliably detect fluid responders and nonresponders.Trial registration: ClinicalTrial.gov (NCT01941472). Registered on 13 September 2013.

Background: The etiologies of acute respiratory failure (ARF) in critically ill rheumatology patients remain unknown. We aimed to describe the clinical features, etiologies and outcomes of adult patients with systemic rheumatic diseases (SRDs) who were admitted to intensive care unit (ICU). Methods: We performed a retrospective study of all SRD patients with ARF who were admitted to a medical ICU between 2014 and 2018. We collected data on demographics, clinical characteristics, reasons for ICU admission and outcomes. Etiologies of ARF were classified as infection, SRD exacerbation, and undetermined. Independent predictors of ICU mortality were identified with multivariate logistic regression analysis. Results: A total of 259 patients admitted to ICU due to ARF were included in final analysis. Systemic lupus erythematosus, dermatomyositis/polymyositis (DM/PM), vasculitis and rheumatoid arthritis were the most common SRDs (78% of patients). Etiologies of ARF included infection (n = 209, 80.7%), SRD exacerbation (n = 71, 27.4%), and undetermined (n = 21, 8.1%). The most common pathogen was Pneumocystis jirovecii (39.8%), followed by Aspergillus spp. (33.2%), and cytomegalovirus (23.2%). One hundred and fifty-five patients (59.8%) died during ICU. Higher acute physiology and chronic health evaluation II score (odds ratio [OR] 1.118, 95% confidence interval [CI] 1.054 to 1.186, p < 0.001) and PaO2/FiO2 < 100 mmHg (OR 3.918, 95% CI 2.199 to 6.892, p < 0.001), DM/PM (OR 4.898, 95% CI 1.949 to 12.309, p = 0.001), vasculitis (OR 3.007, 95% CI 1.237 to 7.309, p = 0.015) and Pneumocystis pneumonia (OR 2.345, 95% CI 1.168 to 4.705, p = 0.016) were independent predictors of ICU mortality. Conclusions: Opportunistic infections and SRD exacerbation were the most common etiologies of ARF in patients with SRDs requiring ICU admission, with high ICU mortality. Development of a standard protocol for differential diagnosis in this group of immunocompromised patients might help initiate definitive therapy and improve clinical outcome. Keywords: Infection, Systemic rheumatic disease, Acute respiratory failure, Etiology

Objective: Connective tissue disease associated pulmonary hypertension (CTD-PH) is classified as a subgroup of WHO group 1 PH, also called pulmonary arterial hypertension (PAH). However, not all CTD-PH fit hemodynamic definition of PAH. This study investigates the diversity of hemodynamic types of CTD-PH, their differences in clinical characteristics and outcomes. Method: We performed a retrospective cohort study. CTD-PH patients were enrolled and divided into WHO group1 PH, WHO group 2 PH and hyperdynamic PH (mPAP>20mmHg, PVR<3WU, PAWP<15mmHg) according to hemodynamics obtained by right heart catheterization. Patients with severe lung diseases, left heart disease, pulmonary embolism, and hepatic cirrhosis were excluded. Baseline characteristics, autoantibodies, cardiac function, echocardiogram parameters, hemodynamics and survival rates were compared. Result: A total of 207 CTD-PH patients were included, 139 in WHO group 1 PH, 36 in WHO group 2 PH and 32 in hyperdynamic PH. We found hyperdynamic PH is less severe, presenting lower NT-proBNP level, better WHO function class, lower mPAP and PVR, higher cardiac output, and less cardiac remodeling. Incidence of anti-RNP was significantly lower in patients with elevated PAWP. Short-term survival was worse in WHO group 2 PH, yet 5-year survival rates didn’t differ between groups. Conclusion: Considering diversity in hemodynamic types, CTD-PH is more than a subgroup of PAH. Different types of CTD-PH present different clinical phenotypes and outcome. Phenotyping PH in CTD-PH patients is important