Session Information
Date: Sunday, November 5, 2017
Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster I
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Annual echocardiograms (echo) are recommended to detect PAH at an early stage, potentially improving outcome. We aimed to evaluate the potential of baseline echo parameters to predict PAH progression and all-cause mortality.
Methods: The study cohort included all SSc patients from the prospective Oslo University Hospital (OUH) cohort who had an evaluable baseline protocol echo performed between 2003 and 2016. This echo was analyzed regarding left- and right-sided systolic and diastolic function. Right heart catheterization (RHC) was performed in patients suspected of PH. PAH diagnosis was made by an experienced cardiologist with mean pulmonary artery pressure ≥ 25 mmHg, pulmonary capillary wedge pressure <15mmHg, stable forced vital capacity >70% and <10% lung fibrosis on HRCT. Vital status was available for all patients and PAH progression, defined by occurrence of new PAH related events were calculated. Cox regression analyzes were conducted.
Results: In total, 337 SSc patients with baseline echo were included. RHC was conducted in 149 (44.2%) patients and 48 (14.2%) were diagnosed with PAH; of those, 34 patients showed PAH progression. Demographic and clinical characteristics are summarized in Table 1. Significant results from univariable and multivariable cox analyses of death are shown in Table 2, and for PAH progression in Table 3. In the final cox regression model for death, age (HR 1.06, 1.03-1.10 95%CI , p<0.001), DLCO (HR 0.97, 0.96-0.98 95%CI , p=0.001), pericardial effusion (HR 2.24, 1.37-3.63 95%CI , p<0.001), left atrium area (HR 1.07, 1.01-1.14 95% CI, p=0.024) and tricuspid annular plane systolic excursion (TAPSE) (HR 0.38, 0.19-0.74 95% CI , p=0.004) were associated (c-index 0.82). PAH progression was associated with age (HR 1.06, 1.02-1.11 95% CI, p=0.005), DLCO (HR 0.97, 0.95-0.99 95% CI, p=0.002), and right atrium area (HR 1.17, 1.10-1.25 95% CI, p<0.001) (c-index 0.86).
Conclusion: In our large and unselected SSc cohort, impaired baseline right heart function, but not left ventricular systolic or diastolic parameters, was predictive for both death and PAH progression. Echo may serve as a tool to stratify patients at risk for PAH and death.
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Table 1: Demographic and clinical characteristics of all SSc patients, SSc-PAH patients and deceased patients.
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All cases (n=337) |
Deceased patients (n=101) |
PAH (n=48) |
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Male, no (%) |
57 (17) |
25 (25) |
8 (17) |
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ACA, no (%) |
172 (55) |
39 (39) |
38 (83) |
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lcSSc, no (%) |
224 (68) |
65 (64) |
44 (92) |
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Death, no (%) |
101 (30) |
– |
25 (52) |
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Age at echo, mean (SD) |
58 (14) |
64 (12) |
64 (11) |
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Observation period, years, median (min,max) |
5.1 (0-14) |
3.0 (0-12) |
5.3 (0.1-12.3) |
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TAPSE, cm (SD) |
2.3 (0.5) |
2.0 (0.6) |
2.0 (0.6) |
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Right ventricular strain % (SD) |
23.0 (6.1) |
20.3 (7.1) |
19.3 (7.3) |
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Left atrium area, cm2 (SD) |
18 (5) |
19 (5) |
17 (4) |
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LV Global longitudinal strain, % (SD) |
18.4 (3.0) |
17.6 (3.5) |
18.4 (2.2) |
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LVEF, % (SD) |
58 (8) |
56 (10) |
59 (7) |
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E-wave, m/s (SD) |
0.72 (0.21) |
0.72 (0.29) |
0.67(0.24) |
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A-wave, m/s (SD) |
0.70 (0.22) |
0.77 (0.26) |
0.72(0.21) |
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e’ septal, cm/s (SD) |
7.5 (2.6) |
5.9 (2.1) |
5.7 (2.1) |
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E/e’ ratio (SD) |
10.6 (4.5) |
13.0 (6.3) |
12.7 (6.1) |
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Tricuspid regurgitant pressure (TRp) , mmHg (SD) |
34 (21) |
44 (25) |
50 (26) |
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Right atrium, area, cm2 (SD) |
17 (6) |
19 (7) |
21 (8) |
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Fractional area change (FAC), % (SD) |
39 (10) |
35 (10) |
32 (11) |
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Pericardial effusion, no (%) |
42 (13) |
22 (22) |
9 (19) |
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Table 2: Univariable and multivariable cox regression analyses associated with death
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Univarable |
Multivariable |
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HR |
95% CI |
p-value |
HR |
95% CI |
p-value |
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Pericardial effusion |
2.09 |
1.49-2.94 |
<0.001 |
1.86 |
1.18-2.95 |
0.008 |
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Left atrium, area |
1.05 |
1.01-1.05 |
0.017 |
1.08 |
1.02-1.14 |
0.008 |
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TAPSE |
0.29 |
0.19-0.45 |
<0.001 |
0.44 |
0.23-0.83 |
0.011 |
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Right ventricular strain |
0.93 |
0.89-0.98 |
0.002 |
0.94 |
0.88-0.99 |
0.045 |
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Right atrium, area |
1.08 |
1.05-1.12 |
<0.001 |
1.1 |
1.05-1.15 |
<0.001 |
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TRp |
1.02 |
1.01-1.03 |
<0.001 |
1.02 |
1.002-1.02 |
0.019 |
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Table 3: Univariable and multivariable cox regression analyses (adjusted for age and gender) associated with PAH progression
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Univariable |
Multivariable |
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HR |
95% CI |
p-value |
HR |
95% CI |
p-value |
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Pericardial effusion |
2.9 |
1.76-4.69 |
<0.001 |
3.2 |
1.83-5.55 |
<0.001 |
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TAPSE |
0.17 |
0.09-0.34 |
<0.001 |
0.24 |
0.12-0.47 |
<0.001 |
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Right ventricular strain |
0.89 |
0.85-0.95 |
0.001 |
0.89 |
0.84-0.95 |
<0.001 |
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Right atrium, area |
1.17 |
1.11-1.22 |
<0.001 |
1.14 |
1.08-1.20 |
<0.001 |
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FAC |
0.93 |
0.89-0.96 |
<0.001 |
0.94 |
0.90-0.97 |
0.001 |
To cite this abstract in AMA style:
Heiervang Tennøe A, Hoffmann-Vold AM, Molberg Ø, Fretheim H, Garen T, Gude E, Andreassen AK, Murbræch K, Aakhus S, Andreassen J, Midtvedt O. Prediction of All-Cause Mortality and Pulmonary Arterial Hypertension (PAH) Progression in Systemic Sclerosis (SSc), an Echocardiography Study [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/prediction-of-all-cause-mortality-and-pulmonary-arterial-hypertension-pah-progression-in-systemic-sclerosis-ssc-an-echocardiography-study/. Accessed .ACR Meeting Abstracts - https://acrabstracts.org/abstract/prediction-of-all-cause-mortality-and-pulmonary-arterial-hypertension-pah-progression-in-systemic-sclerosis-ssc-an-echocardiography-study/