Abstract
Aims
Several risk prediction models have been developed for risk assessment at the time of diagnosis and during follow-up in patients (pts) with pulmonary arterial hypertension (PAH). In this single-centre study we aimed to compare baseline REVEAL, REVEAL 2.0, COMPERA and FPHN risk scores in predicting the mortality in pts with PAH.
Methods
Study group comprised of 504 pts (age 54.4±18.9 years, female 64.4%) with PAH out of the overall 852 pts with pulmonary hypertension enrolled fourteen year period. Subgroups of PAH were as follows; IPAH (39%), CHD-PAH (51%), CTD-PAH (7.7%) and other PAH (3.3%). Functional-class (FC), six-minute-walking-distance (6MWD), pericardial effusion (PE), right atrial area (RAA), tricuspid-annular-planary-systolic-excursion (TAPSE) and systolic annular velocity (St), Echo and Catheter pulmonary artery mean presures (PAMP) and N-terminal-pro brain natriuretic-peptide (NT-proBNP) measures were evaluated at baseline and periodical control examinations with 6-months apart. Baseline assessments for REVEAL, REVEAL 2.0, COMPERA and FPHN risk scores were performed in all pts.
Results
In baseline assessment mean FC and 6MWD were 3 (3–4 IQR), and 230±100 m, repectively. Mean PAMP was 51.5±26 mm Hg, and pulmonary vascular resistance was 8.8±6.5 Wood units. Mono, dual and triple combination therapies were noted in 42%, 40%, and 18% of pts. Median follow-up time was (1470 (275–4840 IQR)) and overall all-cause mortality was 32.1%. Using the Cox proportional hazard model, likelihood ratio (LR) of scores and p values were as follows; REVEAL: 95.09 (p<0.001), REVEAL-2: 122.16 (p<0.001), COMPERA: 22.73 (p<0.001) and FPHN: 1.63 (p=0.210). Adding the TAPSE on REVEAL 2.0 score did not increase the LR of REVEAL 2.0.
When comparing the different combinations including REVEAL 2.0, with likelihood ratio test, the LR of REVEAL 2.0 plus TAPSE vs REVEAL 2.0 was 7.32 (p=0.12),
Conclusions
In this single-centre study, REVEAL 2.0 as compared to REVEAL, COMPERA and FPHN models provided the highest prediction for mortality, and adding the TAPSE on the REVEAL 2.0 scoring offered no additional benefit for risk assessment.
Funding Acknowledgement
Type of funding source: None
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