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Abstract
Background To verify the prognostic value of simplified risk assessment strategy based on 2015 European pulmonary hypertension (PH) guidelines at baseline and long-term follow-up in Chinese connective tissue disease (CTD) associated pulmonary arterial hypertension (PAH) patients.
Methods This single-center retrospective study included 50 patients with right heart catheterization (RHC) diagnosed PAH accompanied by systemic lupus erythematosus (SLE), primary Sjogrens syndrome (pSS), mixed CTD (MCTD) or systemic sclerosis (SSc) who hospitalized in the first affiliated hospital of Nanjing Medical University from April 2009 to May 2018. The data of demographics, symptoms, WHO functional class, 6 min walking distance (6MWD), blood biochemistry, transthoracic echocardiography, high-resolution computer tomography, RHC and treatment was collected at the baseline and follow-up visit. The risk stratification was performed according to the 2015 European PH guidelines. WHO functional class, 6MWD, N-terminal pro-B-type natriuretic peptide (NT-proBNP), pericardial effusion, right atrial pressure, cardiac index and mixed venous oxygen saturation (SvO2) were the mainly variables to calculate the risk grade. Survival rate was analysed by the Kapla-Meier method and differences between groups were assessed by Long-Rank test.
Results In this study, SLE-PAH was the most common CTD-PAH (44%), followed by pSS-PAH (28%). We divided all patients into two groups (low risk and intermediate-high risk group) according to the risk stratification. The change of risk stratification during follow-up visits was evaluated. Intermediate-high risk group at baseline exhibited a poorer long-term outcome than low risk group (p=0.0098), the 1-, 3- and 5 year survival rate were 97%, 69% and 39%, respectively. While no patient was died in low risk group. 24 patients in intermediate-high risk group were degraded into low risk group during the follow-up visit. The estimated 1-, 3- and 5 year survival rate were 100%, 84% and 39% for patients who degraded from intermediate-high risk group to low risk group while 88%, 26% and 26% for patients who remained in intermediate-high risk group.
Included variables and cut-off values used for simplified risk assessment instrument
Characteristics of the patients included in the baseline risk grade group.
(A) five-year survival rate based on baseline risk stratification. The estimated survival was differed between low risk group and intermediate-high risk group (p=0.019); (B) three-year survival rate of CTD-PAH patients based on follow-up risk assessment. Patients who remained in intermediate-high risk group after treatment initiation have poorer outcome
Conclusions The simplified risk stratification model based on 2015 European PH guidelines effectively identified the prognosis of Chinese CTD-PAH patients. Patients have a reduced mortality risk in low risk grade at baseline and stable in low risk grade after long-term treatment.
Funding Source(s): Kaplan-Meier analysis of survival in different risk group patients at baseline and follow-up visit.