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

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

		No increased probability^a			Low probability of PAH^a			Moderate probability of PAH^a			High probability of PAH^a			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 screen^c, 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	25^b (1.5%)	6^a (24%)	1142	53^b (4.6%)	17^a (32.7%)	386	60^b (15.5%)	36^a (60%)	186	110^b (59%)	87^a (79.1%)	942	38^b (4%)	14^a (36.8%)	160 (11.7%)

^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.
^bPercentage of patients who were detected by transthoracic echocardiogram (TTE) to be in a risk category and underwent right-heart catheterisation (RHC)
^cPercentage of patients who underwent RHC and were diagnosed with World Health Organisation (WHO) Group I PAH

ISSN: 1478-6362