Figures
- FIGURE 1
a) Histogram reporting per cent changes in ESC/ERS score from diagnosis to last observation, according to groups based on the number of cardiovascular comorbidities. b) Histogram reporting per cent changes in REVEAL 2.0 score from diagnosis to last observation, according to groups based on the number of cardiovascular comorbidities. Group A: patients without cardiovascular comorbidities; Group B: patients with one cardiovascular comorbidity; Group C: patients with at least two cardiovascular comorbidities. Low risk: green; intermediate risk: yellow; high risk: red. ESC: European Society of Cardiology; ERS: European Respiratory Society, REVEAL: United States Registry to Evaluate Early and Long-Term PAH Disease Management registry.
- FIGURE 2
Boxplots of PVR reduction (%) with double oral initial therapy according to the number of cardiovascular comorbidities. PVR: pulmonary vascular resistance.
- FIGURE 3
Boxplots of PVR reduction (%) with double oral initial therapy in patients with one comorbidity. Patients with a) hypercholesterolaemia, b) systemic hypertension, c) obesity, d) diabetes mellitus, e) previous coronary angioplasty, f) thyroid diseases, g) depression and h) other non-cardiac comorbidities. PVR: pulmonary vascular resistance.
- FIGURE 4
a) Histogram reporting per cent changes in ESC/ERS score from diagnosis to last observation, according to groups based on the presence of comorbidities. b) Histogram reporting per cent changes in REVEAL 2.0 score from diagnosis to last observation, according to groups based on the presence of comorbidities. Group A: patients without cardiovascular comorbidities; No-co: patients without comorbidities. Non-cardiac-co: patients with non-cardiac comorbidities. Cardiac-co: patients with cardiac comorbidities. Low risk: green; intermediate risk: yellow; high risk: red. ESC: European Society of Cardiology; ERS: European Respiratory Society, REVEAL: United States Registry to Evaluate Early and Long-Term PAH Disease Management registry.
- FIGURE 5
a) Boxplots of PVR reduction (%) versus number of cardiovascular comorbidities, based on the presence of an ERS/ESC low-risk status at last observation (II obs.). b) Boxplots of PVR reduction (%) versus number of cardiovascular comorbidities, based on the presence of a REVEAL 2.0 low-risk status at last observation. Box edges represent the 25th (Q1) and 75th (Q3) quantiles, respectively. Outliers are defined as values >1.5 times the interquartile range above Q3 or below Q1. Therefore, the upper whisker is drawn at the greatest value smaller than 1.5 IQR above the third quartile, while the lower whisker is drawn at the smallest value >1.5 IQR below the first quartile. ESC: European Society of Cardiology; ERS: European Respiratory Society, PVR: pulmonary vascular resistance, REVEAL: United States Registry to Evaluate Early and Long-Term PAH Disease Management registry.
Tables
- TABLE 1
Clinical, haemodynamic and echocardiographic characteristics of the study population based on the number of cardiovascular comorbidities
Group A Group B Group C Baseline 6 months FU Baseline 6 months FU Δ p-value Baseline 6 months FU Δ p-value Baseline 6 months FU Δ p-value 1 versus 2 1 versus 3 2 versus 3 1 versus 2 1 versus 3 2 versus 3 p-value p-value p-value p-value p-value p-value Age years 49.7±15 59.9±14 61.8±13 0.000 0.000 ns Sex, female, n (%) 65 (67.7) 37 (68.5) 18 (58.0) ns <0.01 <0.01 DLCO % pred 54±11 53±13 51±3 ns ns ns NYHA 2.8±0.4 2.2±0.6 −0.5±0.6 0.0001 2.9±0.3 2.4±0.6 −0.4±06 0.0001 2.6±0.5 2.5±0.6 −0.1±0.4 ns ns ns 0.02 ns ns ns Class I–II, n (%) 20 (20.8) 65 (67.7) 9 (16.7) 31 (57.4) 8 (25.8) 11 (35.5) Class III, n (%) 67 (69.8) 31 (32.3) 40 (74.0) 21 (38.9) 20 (64.5) 13 (41.9) Class IV, n (%) 9 (9.4) 0 (0) 5 (9.3) 2 (3.7) 3 (9.7) 7 (22.6) 6MWT m 337±96 392±103 +55±71 0.0001 313±108 352±129 +39±104 0.005 321±103 343±109 +22±88 0.04 ns ns ns ns ns ns Heart rate, beats·min−1 78±17 73±13 −5±16 0.001 79±19 76±12 −3±20 ns 78±13 71±25 −7±24 ns ns ns ns ns ns ns NT-proBNP pg·mL−1 944±708 465±359 −478±517 0.0001 824±574 391±198 −433±521 0.003 1268±846 579±501 −689±839 0.01 ns ns 0.009 ns ns ns Haemodynamics RAP mmHg 9.0±4.4 6.8±3.8 −2.1±4.3 0.0001 8.3±4.9 7.6±4.2 −0.7±4.5 ns 8.0±5.5 5.9±4.9 −2.2±3.2 0.02 ns ns ns ns ns ns mPAP mmHg 51±12 43±16 −7.5±13 0.0001 49±12 43±9 −6.5±10 0.0001 49±11 44±13 −4.9±9 ns ns ns ns ns ns ns WP mmHg 9.7±3.2 8.8±3.8 10.9±3.2 10.2±3.5 10±3.5 10±3.3 ns ns ns ns ns ns CI L·min·m−2 2.3±0.9 3.1±0.9 +0.8±0.9 0.0001 2.3±0.6 2.8±0.6 +0.5±0.6 0.0001 2.3±0.6 2.8±0.9 +0.5±0.6 0.002 ns ns ns ns ns ns PVR WU 12.4±6.5 7.4±4.2 −4.8±5.6 0.0001 10.3±4.4 7.2±2.8 −3.1±3.4 0.0001 10.6±4.9 8.7±3.9 −2.0±2.6 0.01 ns ns ns ns ns ns Echocardiography RV/LV ratio <0.01 <0.01 <0.01 ns ns ns <0.01 <0.01 ns <1, n (%) 3 (3.1) 22 (22.9) 3 (5.6) 8 (14.8) 0 (0) 4 (12.9) =1, n (%) 15 (15.6) 18 (18.7) 7 (13) 9 (16.7) 5 (16.1) 4 (12.9) >1, n (%) 78 (81.3) 56 (58.2) 44 (81.5) 37 (68.5) 26 (83.9) 23 (74.2) TAPSE, mm 16.8±4 19.5±4 +2.7±3 0.0001 16.2±4 17.6±4.7 +1.4±4 0.01 16.7±3 18.2±4 +1.5±3 0.01 ns ns ns 0.04 ns ns TR, grade <0.01 <0.01 <0.01 ns <0.01 ns ns <0.01 <0.01 Mild, n (%) 48 (50.0) 59 (61.5) 25 (46.3) 28 (51.8) 12 (38.7) 12 (38.7) Moderate, n (%) 33 (34.3) 29 (30.2) 18 (33.3) 21 (38.9) 10 (32.3) 15 (48.3) Severe, n (%) 15 (15.7) 8 (8.3) 11 (20.3) 5 (9.3) 9 (29.0) 4 (13.0) Pericardial effusion, n (%) 20 (20.8) 9 (9.3) 12 (22.2) 5 (9.2) 6 (19.3) 5 (16.1) Group A: patients without cardiovascular comorbidities; Group B: patients with one cardiovascular comorbidity; Group C: patients with at least two cardiovascular comorbidities. Results are expressed as mean±sd unless indicated otherwise. FU: follow-up; ns: nonsignificant; DLCO: diffusing capacity of the lung for carbon monoxide; NYHA: New York Heart Association; 6MWT: non-encouraged 6-min walk test; RAP: mean right atrial pressure; mPAP: mean pulmonary arterial pressure; WP: wedge pressure; CI: cardiac index; PVR: pulmonary vascular resistance; RV: right ventricular end-diastolic area; LV: left ventricular end-diastolic area; TAPSE: tricupid anular plane systolic excursion; TR: tricuspid regurgitation.
- TABLE 2
Patients’ risk distribution at the time of diagnosis
Overall Group A Group B Group C p-value Patients n 181 96 54 31 ERS/ESC score, n (%) Low 27 (14.9) 15 (15.6) 6 (11.1) 6 (19.3) NS Intermediate 130 (71.8) 70 (72.9) 41 (75.9) 19 (61.4) High 24 (13.3) 11 (11.5) 7 (13.0) 6 (19.3) REVEAL 2.0, n (%) Low (<7) 19 (10.5) 9 (9.4) 7 (13.0) 3 (9.7) NS Intermediate (7–8) 100 (55.2) 56 (58.3) 27 (50.0) 17 (54.8) High (>8) 62 (34.3) 31 (32.3) 20 (37.0) 11 (35.5) Group A: patients without cardiovascular comorbidities; Group B: patients with one cardiovascular comorbidity; Group C: patients with at least two cardiovascular comorbidities. Results are expressed as n (%). ERS/ESC: European Respiratory Society/European Society of Cardiology; ns: nonsignificant.
