Page 54 - Assessing right ventricular function and the pulmonary circulation in pulmonary hypertension Onno Anthonius Spruijt

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Right heart score and other validated scores
A right heart score was built on the basis of the beta coefficients of the multivariable model, assigning a baseline value of 1 and additional points for each category of risk (Table 5). The right heart score had a c-statistic of 0.88 (95%CI: 0.79-0.94), the REVEAL score had a c-statistic of 0.80 (95%CI: 0.69-0.88), and the NIH survival equation had a c-statistic of 0.60 (95%CI: 0.49-0.71). With the DeLong method, both the right heart score and the REVEAL score had significantly higher c- statistics than the NIH survival equation (p<0.001 and p=0.013, respectively). There was no statistical difference between the right heart score and the REVEAL score in the cohort (p=0.097). Figure 5 illustrates the Kaplan-Meier survival curves associated with the right heart score, as well as its relationship with other scores.
Baseline value 1 RV function
Normal 0 Mild 1 Moderate to severe 2
RA size
Less than severe RAE 0 Severe RAE 1
SBP
SBP >110mmHg 0 SBP <110mmHg 1
RH score 1-5
Table 5: Example of RH Score and Point Allocation. RV dysfunction was classified into normal (no dysfunction), mild, or moderate to severe according to the American Society of Echocardiography criteria.
Validation cohort
The validation cohort included 87 patients with idiopathic or familial PAH followed at VU Medical Center between 2001 and 2012. The average age was 47.8±16.0 years, the majority of patients were female (75%), baseline PVR was 11.2±5 Wood units, and baseline 6MWD was 407±127 m. All patients were on disease-modifying therapy, the average time between CMR and diagnosis was 1.5±1.5 years, and the average follow-up time was 4.2±3.2 years. The composite endpoint occurred in 29 patients, including 23 deaths and 6 lung transplants. On univariate analysis, the strongest