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Table 3 Diagnosis of WHO Group I PAH by risk stratification using the screening algorithm

From: Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme

  No increased
probabilitya
Low probability of PAHa Moderate probability of PAHa High probability of PAHa Missing sPAP Total with PAH, n
Review number Screened, n Normal TTE, n RHC,
n
PAH,
n
Moderate TTE, n RHC,
n
PAH,
n
High suspicion TTE, n RHC,
n
No. with PAH High probability TTE, n RHC,
n
PAH,
n
Positive screenc,
n
RHC, n PAH,
n
 
1st screen 1363 470 19 4 358 38 11 121 36 25 101 83 72 313 24 10 122 (8.9%)
2nd screen 896 355 6 2 229 5 3 78 10 4 32 11 5 202 8 3 17 (1.9%)
3rd screen 679 272 0 0 167 4 1 65 5 3 20 7 6 155 3 1 11 (1.6%)
4th screen 482 199 0 0 125 3 0 42 7 2 13 5 3 103 1 0 5 (1.0%)
5th screen 361 149 0 0 104 0 0 25 1 1 9 4 1 74 1 0 2 (0.6%)
6th screen 235 91 0 0 72 1 1 25 1 1 6 0 0 41 0 0 2 (0.9%)
7th screen 162 66 0 0 48 0 0 14 0 0 3 0 0 31 1 0 0 (0.0%)
8th screen 104 48 0 0 27 1 1 10 0 0 1 0 0 18 0 0 0 (0.0%)
9th screen 43 20 0 0 11 1 1 6 0 0 1 0 0 5 0 0 1 (2.3%)
10th screen 1 0 0 0 1 0 0 0 0 0 0 0 0 0 0 0 0 (0.0%)
Total 4326 1670 25b (1.5%) 6a (24%) 1142 53b (4.6%) 17a (32.7%) 386 60b (15.5%) 36a (60%) 186 110b (59%) 87a (79.1%) 942 38b (4%) 14a (36.8%) 160 (11.7%)
  1. aProbability of pulmonary arterial hypertension (PAH): no increased probability of PAH was indicated by systolic pulmonary arterial pressure (sPAP) <30 and diffusing capacity of the lung for carbon monoxide corrected for haemoglobin (DLCO) >50%, low probability of PAH was indicated by 30 ≤ sPAP <40 mmHg with normal forced vital capacity (FVC) and high-resolution computed tomography (HRCT), moderate probability of PAH was 40 ≤ sPAP <50 mmHg OR DLCO ≤50% and FVC <85% or annual fall in DLCO ≥20% or unexplained dyspnea and high probability of PAH was indicated by sPAP ≥50 mmHg.
  2. bPercentage of patients who were detected by transthoracic echocardiogram (TTE) to be in a risk category and underwent right-heart catheterisation (RHC)
  3. cPercentage of patients who underwent RHC and were diagnosed with World Health Organisation (WHO) Group I PAH