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.

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.

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.