Skip to main content
Top
Published in: Clinical Rheumatology 2/2014

01-02-2014 | Original Article

Impact of cardiac involvement on the risk of mortality among patients with systemic sclerosis: a 5-year follow-up of a single-center cohort

Authors: Gyöngyvér Költő, Réka Faludi, Dániel Aradi, Barbara Bartos, Gábor Kumánovics, Tünde Minier, László Czirják, András Komócsi

Published in: Clinical Rheumatology | Issue 2/2014

Login to get access

Abstract

Cardiac involvement is among the leading causes of mortality in patients with systemic sclerosis (SSc). Previously, we demonstrated in a single-center, cross-sectional study the frequent coexistence of different forms of cardiac involvement in systemic sclerosis including pulmonary arterial hypertension (PAH), coronary artery disease (CAD), and microvascular dysfunction (MVD). The aim of the present study was to investigate the prognostic significance of cardiac involvement. One hundred twenty patients with SSc were enrolled. All cases underwent a non-invasive cardiovascular protocol. In 30 patients with suspected cardiac involvement, right heart catheterization and intra-coronary pressure–wire-supplemented coronary angiography were performed. Clinical follow-up was 5 years. Patients with CAD at the baseline showed a trend for higher cardiovascular mortality while in patients with MVD this difference was significant (26.7 % versus 9.5 %, p = 0.077 and 30 % versus 10.1 %, p < 0.05, respectively). Cardiovascular mortality of PAH cases was higher but, however, did not reach statistical significance 21.4 % versus 10.4 %, p = 0.261. Cardiovascular event-free survival was significantly lower among patients with combinations of two or three disorders (p < 0.05). Multivariate analysis of organ involvements and comorbidities showed that the diffuse cutaneous subset, the presence of kidney involvement, the velocity of the tricuspid regurgitation, as well as diabetes mellitus were independent predictors of overall mortality. MVD and CAD alone or in combination with PAH significantly affected the 5-year cardiovascular mortality. These findings highlight the prognostic importance of coronary disease in patients with SSc [www.​clinicaltrials.​gov (Reg. Nr.: NCT00843102)].
Literature
1.
go back to reference Komocsi A, Vorobcsuk A, Faludi R et al (2012) The impact of cardio-pulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 51:1027–1036CrossRef Komocsi A, Vorobcsuk A, Faludi R et al (2012) The impact of cardio-pulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 51:1027–1036CrossRef
2.
go back to reference Czirjak L, Pakozdi A, Kumanovics G et al (2008) Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis 67:59–63PubMedCrossRef Czirjak L, Pakozdi A, Kumanovics G et al (2008) Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis 67:59–63PubMedCrossRef
3.
go back to reference Trad S, Amoura Z, Beigelman C et al (2006) Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease. Arthritis Rheum 54:184–191PubMedCrossRef Trad S, Amoura Z, Beigelman C et al (2006) Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease. Arthritis Rheum 54:184–191PubMedCrossRef
4.
go back to reference Komocsi A, Pinter T, Faludi R et al (2010) Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis. Ann Rheum Dis 69:202–205PubMedCrossRef Komocsi A, Pinter T, Faludi R et al (2010) Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis. Ann Rheum Dis 69:202–205PubMedCrossRef
5.
go back to reference LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205PubMed LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205PubMed
6.
go back to reference Hatano S and Strasser T (1975) Primary pulmonary hypertension: report on a WHO meeting, Geneva, 15–17 October 1973 Hatano S and Strasser T (1975) Primary pulmonary hypertension: report on a WHO meeting, Geneva, 15–17 October 1973
7.
go back to reference Simonneau G, Robbins IM, Beghetti M et al (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54:S43–S54PubMedCrossRef Simonneau G, Robbins IM, Beghetti M et al (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54:S43–S54PubMedCrossRef
8.
go back to reference Galie N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537PubMedCrossRef Galie N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537PubMedCrossRef
9.
go back to reference Kern MJ (2000) Coronary physiology revisited: practical insights from the cardiac catheterization laboratory. Circulation 101:1344–1351PubMedCrossRef Kern MJ (2000) Coronary physiology revisited: practical insights from the cardiac catheterization laboratory. Circulation 101:1344–1351PubMedCrossRef
10.
go back to reference Meune C, Vignaux O, Kahan A, Allanore Y (2010) Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis 103:46–52PubMedCrossRef Meune C, Vignaux O, Kahan A, Allanore Y (2010) Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis 103:46–52PubMedCrossRef
11.
go back to reference Kahan A, Nitenberg A, Foult JM et al (1985) Decreased coronary reserve in primary scleroderma myocardial disease. Arthritis Rheum 28:637–646PubMedCrossRef Kahan A, Nitenberg A, Foult JM et al (1985) Decreased coronary reserve in primary scleroderma myocardial disease. Arthritis Rheum 28:637–646PubMedCrossRef
12.
go back to reference Pinter T, Faludi R, Magyari B et al (2011) Mechanism of coronary flow reserve reduction in systemic sclerosis: insight from intracoronary pressure wire studies. Rheumatology (Oxford) 50:781–788CrossRef Pinter T, Faludi R, Magyari B et al (2011) Mechanism of coronary flow reserve reduction in systemic sclerosis: insight from intracoronary pressure wire studies. Rheumatology (Oxford) 50:781–788CrossRef
13.
go back to reference Pinter T, Faludi R, Magyari B et al (2010) Mechanism of coronary flow reserve reduction in systemic sclerosis, insight from intracoronary pressure-wire studies. Rheumatology (Oxford). doi:10.1093/rheumatology/keq402 Pinter T, Faludi R, Magyari B et al (2010) Mechanism of coronary flow reserve reduction in systemic sclerosis, insight from intracoronary pressure-wire studies. Rheumatology (Oxford). doi:10.​1093/​rheumatology/​keq402
14.
go back to reference Steen VD, Follansbee WP, Conte CG, Medsger TA Jr (1996) Thallium perfusion defects predict subsequent cardiac dysfunction in patients with systemic sclerosis. Arthritis Rheum 39:677–681PubMedCrossRef Steen VD, Follansbee WP, Conte CG, Medsger TA Jr (1996) Thallium perfusion defects predict subsequent cardiac dysfunction in patients with systemic sclerosis. Arthritis Rheum 39:677–681PubMedCrossRef
15.
go back to reference Kahan A, Devaux JY, Amor B et al (1990) The effect of captopril on thallium 201 myocardial perfusion in systemic sclerosis. Clin Pharmacol Ther 47:483–489PubMedCrossRef Kahan A, Devaux JY, Amor B et al (1990) The effect of captopril on thallium 201 myocardial perfusion in systemic sclerosis. Clin Pharmacol Ther 47:483–489PubMedCrossRef
16.
go back to reference Kahan A, Devaux JY, Amor B et al (1986) Pharmacodynamic effect of dipyridamole on thallium-201 myocardial perfusion in progressive systemic sclerosis with diffuse scleroderma. Ann Rheum Dis 45:718–725PubMedCrossRef Kahan A, Devaux JY, Amor B et al (1986) Pharmacodynamic effect of dipyridamole on thallium-201 myocardial perfusion in progressive systemic sclerosis with diffuse scleroderma. Ann Rheum Dis 45:718–725PubMedCrossRef
17.
go back to reference Kahan A, Devaux JY, Amor B et al (1986) Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis. N Engl J Med 314:1397–1402PubMedCrossRef Kahan A, Devaux JY, Amor B et al (1986) Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis. N Engl J Med 314:1397–1402PubMedCrossRef
18.
go back to reference Allanore Y, Meune C, Vignaux O et al (2006) Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and tissue-Doppler echography study. J Rheumatol 33:2464–2469PubMed Allanore Y, Meune C, Vignaux O et al (2006) Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and tissue-Doppler echography study. J Rheumatol 33:2464–2469PubMed
19.
go back to reference Allanore Y, Meune C, Vonk MC et al (2010) Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. Ann Rheum Dis 69:218–221PubMedCrossRef Allanore Y, Meune C, Vonk MC et al (2010) Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. Ann Rheum Dis 69:218–221PubMedCrossRef
20.
go back to reference Caiati C, Montaldo C, Zedda N et al (1999) Validation of a new noninvasive method (contrast-enhanced transthoracic second harmonic echo Doppler) for the evaluation of coronary flow reserve: comparison with intracoronary Doppler flow wire. J Am Coll Cardiol 34:1193–1200PubMedCrossRef Caiati C, Montaldo C, Zedda N et al (1999) Validation of a new noninvasive method (contrast-enhanced transthoracic second harmonic echo Doppler) for the evaluation of coronary flow reserve: comparison with intracoronary Doppler flow wire. J Am Coll Cardiol 34:1193–1200PubMedCrossRef
21.
go back to reference Hachulla AL, Launay D, Gaxotte V et al (2009) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis 68:1878–1884PubMedCentralPubMedCrossRef Hachulla AL, Launay D, Gaxotte V et al (2009) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis 68:1878–1884PubMedCentralPubMedCrossRef
22.
go back to reference Allanore Y, Wahbi K, Borderie D et al (2009) N-terminal pro-brain natriuretic peptide in systemic sclerosis: a new cornerstone of cardiovascular assessment? Ann Rheum Dis 68:1885–1889PubMedCrossRef Allanore Y, Wahbi K, Borderie D et al (2009) N-terminal pro-brain natriuretic peptide in systemic sclerosis: a new cornerstone of cardiovascular assessment? Ann Rheum Dis 68:1885–1889PubMedCrossRef
23.
24.
go back to reference Poms AM, Turner M, Farber HW, Meltzer LA, and McGoon MD (2013) Comorbid conditions and outcomes in patients with pulmonary arterial hypertension: a REVEAL registry analysis. Chest Poms AM, Turner M, Farber HW, Meltzer LA, and McGoon MD (2013) Comorbid conditions and outcomes in patients with pulmonary arterial hypertension: a REVEAL registry analysis. Chest
25.
go back to reference Perk J, De Backer G, Gohlke H et al (2012) European Guidelines on cardiovascular disease prevention in clinical practice (version 2012). The Fifth Joint Task Force of the European Society of Cardiology and Other Societies on Cardiovascular Disease Prevention in Clinical Practice (constituted by representatives of nine societies and by invited experts). Developed with the special contribution of the European Association for Cardiovascular Prevention & Rehabilitation (EACPR). Eur Heart J 33:1635–1701PubMedCrossRef Perk J, De Backer G, Gohlke H et al (2012) European Guidelines on cardiovascular disease prevention in clinical practice (version 2012). The Fifth Joint Task Force of the European Society of Cardiology and Other Societies on Cardiovascular Disease Prevention in Clinical Practice (constituted by representatives of nine societies and by invited experts). Developed with the special contribution of the European Association for Cardiovascular Prevention & Rehabilitation (EACPR). Eur Heart J 33:1635–1701PubMedCrossRef
26.
go back to reference Hachulla E, Gressin V, Guillevin L et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52:3792–3800PubMedCrossRef Hachulla E, Gressin V, Guillevin L et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52:3792–3800PubMedCrossRef
27.
go back to reference McGoon M, Gutterman D, Steen V et al (2004) Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 126:14S–34SPubMedCrossRef McGoon M, Gutterman D, Steen V et al (2004) Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 126:14S–34SPubMedCrossRef
28.
go back to reference Humbert M, Yaici A, de Groote P et al (2011) Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 63:3522–3530PubMedCrossRef Humbert M, Yaici A, de Groote P et al (2011) Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 63:3522–3530PubMedCrossRef
29.
go back to reference Kovacs G, Maier R, Aberer E et al (2012) Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure. Arthritis Rheum 64:1257–1262PubMedCrossRef Kovacs G, Maier R, Aberer E et al (2012) Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure. Arthritis Rheum 64:1257–1262PubMedCrossRef
30.
go back to reference Avouac J, Wipff J, Kahan A, Allanore Y (2008) Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials. Ann Rheum Dis 67:808–814PubMedCrossRef Avouac J, Wipff J, Kahan A, Allanore Y (2008) Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials. Ann Rheum Dis 67:808–814PubMedCrossRef
31.
go back to reference Ryerson CJ, Nayar S, Swiston JR, Sin DD (2010) Pharmacotherapy in pulmonary arterial hypertension: a systematic review and meta-analysis. Respir Res 11:12PubMedCentralPubMedCrossRef Ryerson CJ, Nayar S, Swiston JR, Sin DD (2010) Pharmacotherapy in pulmonary arterial hypertension: a systematic review and meta-analysis. Respir Res 11:12PubMedCentralPubMedCrossRef
32.
go back to reference Galie N, Manes A, Negro L et al (2009) A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 30:394–403PubMedCrossRef Galie N, Manes A, Negro L et al (2009) A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 30:394–403PubMedCrossRef
33.
go back to reference Williams MH, Das C, Handler CE et al (2006) Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 92:926–932PubMedCrossRef Williams MH, Das C, Handler CE et al (2006) Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 92:926–932PubMedCrossRef
34.
go back to reference Hachulla E, Carpentier P, Gressin V et al (2009) Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study. Rheumatology (Oxford) 48:304–308CrossRef Hachulla E, Carpentier P, Gressin V et al (2009) Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study. Rheumatology (Oxford) 48:304–308CrossRef
35.
go back to reference Badesch DB, Champion HC, Sanchez MA et al (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54:S55–S66PubMedCrossRef Badesch DB, Champion HC, Sanchez MA et al (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54:S55–S66PubMedCrossRef
Metadata
Title
Impact of cardiac involvement on the risk of mortality among patients with systemic sclerosis: a 5-year follow-up of a single-center cohort
Authors
Gyöngyvér Költő
Réka Faludi
Dániel Aradi
Barbara Bartos
Gábor Kumánovics
Tünde Minier
László Czirják
András Komócsi
Publication date
01-02-2014
Publisher
Springer London
Published in
Clinical Rheumatology / Issue 2/2014
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-013-2358-4

Other articles of this Issue 2/2014

Clinical Rheumatology 2/2014 Go to the issue