Table 1.
Demographics and characteristics at baseline.
| Variable | Triple oral combination
therapy N = 26 |
|---|---|
| Female | 19 (73.1) |
| Age, years | 38 (23–48) |
| WHO-FC | |
| I/II/III/IV | 0/9/16/1 (0.0/34.6/61.5/3.8) |
| BNP, pg/mL | 105.25 (36.7–285.5) |
| 6MWD, m | 397.5 (312.8–441.8) |
| PAH etiology | |
| IPAH | 10 (38.5) |
| HPAH | 5 (19.2) |
| CTD-PAH | 7 (26.9) |
| CHD-PAH | 4 (15.4) |
| Medication | |
| Treatment-naïve | 17 (65.4) |
| Single | 3 (11.5) |
| Double | 2 (7.7) |
| Triple | 4 (15.4) |
| Risk assessment* | |
| High risk | 12 (46.2) |
| Intermediate risk | 4 (15.4) |
| Low risk | 10 (38.5) |
| Genetic mutation** | |
| BMPR2 | 5 (23.8) |
| ACVRL | 1 (4.8) |
| RNF213 | 1 (4.8) |
Data are expressed as number (%) or median (interquartile range).
Risk assessment was calculated using the three-category REVEAL 2.0 risk score.16 The high-risk group was defined as patients with a predicted 1-year survival rate of <90% (REVEAL 2.0 risk score ⩾9), the intermediate-risk group was defined as patients with a predicted 1-year survival rate of 90–<95% (REVEAL 2.0 risk score = 7 or 8), and the low-risk group was defined as patients with a predicted 1-year survival rate of ⩾95% (REVEAL 2.0 risk score ⩽6).
Among 26 patients, 21 were genetically tested and 15 had no genetic mutations related to PAH.
6MWD, 6-minute walk distance; ACVRL1, activin A receptor-like kinase 1; BMPR2, bone morphogenetic protein receptor type 2; BNP, B-type natriuretic peptide; CHD, congenital heart disease; CTD, connective tissue disease; HPAH, heritable pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; PAH, pulmonary arterial hypertension; RNF213, ring finger protein 213; WHO-FC, World Health Organization functional class.