Table 4.
Haemodynamic characteristics at diagnosis in patients with confirmed pulmonary hypertension (PH confirmed), according to pulmonary hypertension groups and subgroups
| All patients with PH confirmed | PH group 3 | PH group 1
|
||||||
|---|---|---|---|---|---|---|---|---|
| IPAH or FPAH | APAH with CHD
|
APAH excluding APAH with CHD | ||||||
| All CHD | Unrepaired shunt* | Repaired shunt | Never shunt | |||||
| Patients with heart catheterisation at diagnosis | 357 (99%) | 42 (100%) | 178 (98%) | 115 (100%) | 61 (100%) | 45 (100%) | 9 (100%) | 19 (95%) |
|
| ||||||||
| mRAP | 345 (95%) | 41 (98%) | 172 (95%) | 110 (96%) | 59 (97%) | 43 (96%) | 8 (89%) | 19 (95%) |
| Mean mm Hg (95% CI) | 7 (7–8) | 7 (6–8) | 7 (6–8) | 7 (7–8) | 7 (6–8) | 8 (7–9) | 10 (5–14) | 8 (6–9) |
| p value† | ·· | ·· | 0·73 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| mPAP | 357 (99%) | 42 (100%) | 178 (98%) | 115 (100%) | 61 (100%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Mean mm Hg (95% CI) | 58 (56–59) | 44 (39–48) | 59 (57–62)§ | 61 (57–64)§ | 64 (59–69) | 56 (49–62) | 62 (49–75) | 53 (45–61) |
| p value† | ·· | ·· | <0·0001 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| mPCWP | 357 (99%) | 42 (100%) | 178 (98%) | 115 (100%) | 61 (100%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Mean mm Hg (95% CI) | 8 (8–9) | 9 (8–9) | 8 (8–9) | 9 (8–9) | 8 (8–9) | 9 (8–10) | 8 (6–11) | 8 (7–9) |
| p value† | ·· | ·· | 0·99 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| mSAP | 353 (98%) | 42 (100%) | 176 (97%) | 113 (98%) | 59 (97%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Mean mm Hg (95% CI) | 68 (66–69) | 62 (58–67) | 68 (66–70)§ | 68 (65–70)§ | 66 (62–70) | 68 (64–72) | 74 (66–81) | 74 (66–82) |
| p value† | ·· | ·· | 0·04 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| Cardiac index | 348 (96%) | 41 (98%) | 175 (96%) | 110 (96%) | 56 (92%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Mean L/min/m2 (95% CI) | 3·7 (3·5–3·8) | 4·2 (3·6–4·8) | 3·4 (3·2–3·6)§ | 3·9 (3·5–4·3) | 3·8 (3·3–4·3) | 3·7 (3·2–4·2) | 5·4 (2·6–8·3) | 4·0 (2·8–5·2) |
| p value‡ | ·· | ·· | 0·02 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| Qp:Qs | 357 (99%) | 42 (100%) | 182 (100%) | 110 (96%) | 56 (92%) | 45 (100%) | 9 (100%) | 20 (100%) |
| Ratio median (IQR) | 1·00 (1·0–1·0) | 1·00 (1·0–1·0) | 1·00 (1·0–1·0) | 1·00 (1·0–1·1) | 1·03 (0·8–1·5) | 1·00 (1·0–1·0) | 1·00 (1·0–1·0) | 1·00 (1·0–1·0) |
| p value‡ | ·· | ·· | 0·92 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| mPAP:mSAP | 353 (98%) | 42 (100%) | 176 (97%) | 113 (98%) | 59 (97%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Ratio median (IQR) | 0·86 (0·7–1·0) | 0·69 (0·6–1·0) | 0·87 (0·7–1·0)§ | 0·92 (0·8–1·0)§ | 0·96 (0·9–1·1) | 0·84 (0·6–1·0) | 0·85 (0·8–1·0) | 0·64 (0·5–1·1) |
| p value† | ·· | ·· | 0·0001 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| PVRi | 357 (99%) | 42 (100%) | 178 (98%) | 115 (100%) | 61 (100%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Mean WU/m−2 (95% CI) | 16·0 (14·9–17·0) | 9·8 (8·1–11·5) | 17·2 (15·8–18·6)§ | 16·3 (14·3–18·3)§ | 16·8 (14·6–19·0) | 15·0 (11·7–18·3) | 19·3 (4·0–34·7) | 14·9 (11·1–18·7) |
| p value‡ | ·· | ·· | <0·001 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| SVRi | 340 (94%) | 41 (98%) | 169 (93%) | 108 (94%) | 54 (89%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Mean WU/m−2 (95% CI) | 19·7 (18·7–20·8) | 15·8 (13·2–18·3) | 20·5 (19·2–21·8)§ | 19·0 (17·2–20·8)§ | 19·0 (16·6–21·4) | 18·6 (16·4–20·8) | 20·8 (6·4–35·1) | 23·4 (14·3–32·4) |
| p value‡ | ·· | ·· | 0·002 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| PVRi:SVRi | 340 (94%) | 41 (98%) | 169 (93%) | 108 (94%) | 54 (89%) | 45 (100%) | 9 (100%) | 19 (95%) |
| Ratio median (IQR) | 0·80 (0·6–1·0) | 0·66 (0·5–1·0) | 0·82 (0·6–1·0)§ | 0·79 (0·6–1·0)§ | 0·82 (0·6–1·2) | 0·79 (0·5–1·0) | 0·89 (0·7–1·0) | 0·64 (0·4–1·1) |
| p value‡ | ·· | ·· | 0·02 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| Sys ven SO2 | 202 (56%) | 30 (71%) | 72 (40%) | 88 (77%) | 59 (97%) | 27 (60%) | 2 (22%) | 10 (50%) |
| Mean % (95% CI) | 66% (65–67) | 66% (63–70) | 66% (64–68) | 66% (64–68) | 66% (64–69) | 65% (61–69) | 75% (71–79) | 64% (56–72) |
| p value† | ·· | ·· | 0·96 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| Sys art SO2 | 214 (59%) | 32 (76%) | 78 (43%) | 91 (79%) | 60 (98%) | 29 (64%) | 2 (22%) | 11 (55%) |
| Mean % (95% CI) | 91% (90–92) | 86% (81–91) | 94% (93–95)§ | 90% (88–92)§†¶ | 88% (87–90) | 93% (91–96) | 91% (··) | 88% (81–96) |
| p value† | ·· | ·· | <0·0001 | ·· | ·· | ·· | ·· | ·· |
|
| ||||||||
| Yes responders at AVT | 117 (36) | 21 (51) | 61 (38) | 25 (25) | 12 (22) | 13 (33) | 0 (%) | 8 (42) |
|
| ||||||||
| No responders at AVT | 206 (64) | 20 (49) | 100 (62) | 74 (75) | 42 (78) | 26 (67) | 6 (100) | 11 (58) |
APAH excluding APAH with congenital heart disease and groups 4 and 5 are excluded from the analysis. Only patients in the confirmed pulmonary hypertension population are included in the analysis. Patients from pulmonary hypertension groups 4 and 5 (n=3), included in all patients with confirmed pulmonary hypertension, are not depicted separately in this table. APAH=pulmonary arterial hypertension associated with other disorders. IPAH=idiopathic pulmonary arterial hypertension. FPAH=familial pulmonary arterial hypertension. mRAP=mean right atrial pressure. mPAP=mean pulmonary arterial pressure. mSAP=mean systemic arterial pressure. mPCWP=mean pulmonary capillary wedge pressure. PVRI=pulmonary vascular resistance indexed. SVRI=systemic vascular resistance indexed. Qp=pulmonary blood flow. Qs=systemic blood flow. Sys ven SO2=systemic venous oxygen saturation. Sys art SO2=systemic arterial oxygen saturation. AVT=acute pulmonary vasoresponse test as reported by the treating physician (no predefined criteria were used). Data are number (%) unless otherwise specified.
Or partial repair.
p value from ANOVA with cause subgroup (1=pulmonary hypertension group 3, 2=IPAH or FPAH, 3=all congenital heart disease) included in the model. APAH excluding APAH with congenital heart disease and groups 4 and 5 are excluded from the analysis. Only patients in the confirmed pulmonary hypertension population are included in the analysis.
p value from Kruskal-Wallis test to assess differences across cause subgroups (1=pulmonary hypertension group 3, 2=IPAH or FPAH, 3=all congenital heart disease).
Significantly different from PH Group 3.
Significantly different from IPAH or FPAH.