Top

Published in:

Open Access 01-11-2013 | Original Paper

Exaggerated increase of exercise-induced pulmonary artery pressure in systemic sclerosis patients predominantly results from left ventricular diastolic dysfunction

Authors: Michał Ciurzyński, Piotr Bienias, Katarzyna Irzyk, Maciej Kostrubiec, Zbigniew Bartoszewicz, Maria Siwicka, Marcin Kurzyna, Urszula Demkow, Piotr Pruszczyk

Published in: Clinical Research in Cardiology | Issue 11/2013

Abstract

Objective

High prevalence of exaggerated pulmonary artery pressure response to exercise (EPAPR) was reported in patients with systemic sclerosis (SSc). However, pathophysiology of this phenomenon has not been well defined. Therefore, we evaluated the frequency and potential aetiology of EPAPR in SSc patients.

Methods

We included 85 patients (79 female, 6 male, mean age 54.3 ± 13.9 years) with SSc. Transthoracic echocardiography followed by exercise Doppler echocardiography (EDE) were performed. A positive EDE was defined when at least 20 mmHg increase of tricuspid regurgitation peak gradient (TRPG) was recorded. Right heart catheterization (RHC) with exercise was performed in positive EDE patients and in subjects with resting TRPG >31 mmHg.

Results

Resting TRPG >31 mmHg and/or positive EDE was found in 30 patients and they were referred to RHC. Finally, RHC was performed in 20 patients (16 pts resting TRPG >31 mmHg and 4 others normal resting TRPG and positive EDE). In 12 (60 %) of them an EPAPR with elevated pulmonary capillary wedge pressure (PCWP) was observed. Interestingly, mean left atrium (LA) diameter was greater in an EPAPR with elevated PCWP patients than in subjects with normal exercise response (39.36 ± 5.6 vs. 35.53 ± 3.48, p = 0.03). In EPAPR with elevated PCWP group greater mean value of E/E′ of mitral lateral annulus was observed (7.98 ± 3.35 vs. 6.27 ± 1.94, p = 0.03). In the univariate logistic regression analysis increased LA diameter was significant predictor of EPAPR with elevated PCWP (OR 1.199, 95 % CI 1.029–1.396, p = 0.019).

Conclusions

Despite very well-known risk of PAH in systemic sclerosis patients, the excessive increase of PAP during exercise is more commonly caused by left ventricular diastolic dysfunction than pulmonary arterial vasculopathy.

Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52(12):3792–3800PubMedCrossRef

Mukerjee D, St GD, Coleiro B, Knight C, Denton CP, Davar J et al (2003) Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 62(11):1088–1093PubMedCrossRef

Chung L, Liu J, Parsons L, Hassoun PM, McGoon M, Badesch DB et al (2010) Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 138(6):1383–1394PubMedCrossRef

Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L et al (2006) Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 54(9):3043–3050PubMedCrossRef

Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA 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(20):2493–2537PubMedCrossRef

McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR et al (2009) ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American college of cardiology foundation task force on expert consensus documents and the American heart association developed in collaboration with the American college of chest physicians; American thoracic society, Inc. and the pulmonary hypertension association. J Am Coll Cardiol 53(17):1573–1619PubMedCrossRef

Galie N, Rubin L, Hoeper M, Jansa P, Al-Hiti H, Meyer G et al (2008) Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 371(9630):2093–2100PubMedCrossRef

D’Alto M, Ghio S, D’Andrea A, Pazzano AS, Argiento P, Camporotondo R et al (2011) Inappropriate exercise-induced increase in pulmonary artery pressure in patients with systemic sclerosis. Heart 97(2):112–117PubMedCrossRef

Kovacs G, Maier R, Aberer E, Brodmann M, Scheidl S, Hesse C et al (2010) Assessment of pulmonary arterial pressure during exercise in collagen vascular disease: echocardiography versus right-sided heart catheterization. Chest 138(2):270–278PubMedCrossRef

10.

Reichenberger F, Voswinckel R, Schulz R, Mensch O, Ghofrani HA, Olschewski H et al (2009) Non-invasive detection of early pulmonary vascular dysfunction in scleroderma. Respir Med 103(11):1713–1718PubMedCrossRef

11.

Callejas-Rubio JL, Moreno-Escobar E, de la Fuente PM, Perez LL, Fernandez RR, Sanchez-Cano D et al (2008) Prevalence of exercise pulmonary arterial hypertension in scleroderma. J Rheumatol 35(9):1812–1816PubMed

12.

Gargani L, Pignone A, Agoston G, Moreo A, Capati E, Badano LP et al (2013) Clinical and echocardiographic correlations of exercise-induced pulmonary hypertension in systemic sclerosis: a multicenter study. Am Heart J 165(2):200–207PubMedCrossRef

13.

Steen V, Chou M, Shanmugam V, Mathias M, Kuru T, Morrissey R (2008) Exercise-induced pulmonary arterial hypertension in patients with systemic sclerosis. Chest 134(1):146–151PubMedCrossRef

14.

Hachulla AL, Launay D, Gaxotte V, de GP, Lamblin N, Devos P et al (2009) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis 68(12):1878–1884PubMedCrossRef

15.

Meune C, Avouac J, Wahbi K, Cabanes L, Wipff J, Mouthon L et al (2008) Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: a controlled study of 100 consecutive patients. Arthritis Rheum 58(6):1803–1809PubMedCrossRef

16.

(1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma) Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 23(5):581–90

17.

Valentini G, Bencivelli W, Bombardieri S, D’Angelo S, Della RA, Silman AJ et al (2003) European scleroderma study group to define disease activity criteria for systemic sclerosis III. Assessment of the construct validity of the preliminary activity criteria. Ann Rheum Dis 62(9):901–903PubMedCrossRef

18.

Nagueh SF, Appleton CP, Gillebert TC, Marino PN, Oh JK, Smiseth OA et al (2009) Recommendations for the evaluation of left ventricular diastolic function by echocardiography. Eur J Echocardiogr 10(2):165–193PubMedCrossRef

19.

Feigenbaum H, Armstrong WF, Ryan T (2005) Feigenbaum’s echocardiography, 6th edn. Lippincott, Williams and Wilkins, Philadelphia

20.

Ciurzynski M, Bienias P, Irzyk K, Rymarczyk Z, Kostrubiec M, Szewczyk A et al (2011) Usefulness of echocardiography in the identification of an excessive increase in pulmonary arterial pressure in patients with systemic sclerosis. Kardiol Pol 69(1):9–15PubMed

21.

Naeije R, Melot C, Niset G, Delcroix M, Wagner PD (1993) Mechanisms of improved arterial oxygenation after peripheral chemoreceptor stimulation during hypoxic exercise. J Appl Physiol 74(4):1666–1671PubMed

22.

Bae S, Saggar R, Bolster MB, Chung L, Csuka ME, Derk C et al. (2012) Baseline characteristics and follow-up in patients with normal hemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry. Ann Rheum Dis 71(8):1335–1342

23.

Tolle JJ, Waxman AB, Van Horn TL, Pappagianopoulos PP, Systrom DM (2008) Exercise-induced pulmonary arterial hypertension. Circulation 118(21):2183–2189PubMedCrossRef

24.

Yagi S, Akaike M, Iwase T, Kusunose K, Niki T, Yamaguchi K et al (2010) Bosentan ameliorated exercise-induced pulmonary arterial hypertension complicated with systemic sclerosis. Intern Med 49(21):2309–2312PubMedCrossRef

25.

Alkotob ML, Soltani P, Sheatt MA, Katsetos MC, Rothfield N, Hager WD et al (2006) Reduced exercise capacity and stress-induced pulmonary hypertension in patients with scleroderma. Chest 130(1):176–181PubMedCrossRef

26.

Pignone A, Mori F, Pieri F, Oddo A, Galeota G, Fiori G et al (2007) Exercise doppler echocardiography identifies pre clinic asymptomatic pulmonary hypertension in systemic sclerosis. Ann N Y Acad Sci 1108:291–304PubMedCrossRef

27.

Ha JW, Choi D, Park S, Shim CY, Kim JM, Moon SH et al (2009) Determinants of exercise-induced pulmonary hypertension in patients with normal left ventricular ejection fraction. Heart 95(6):490–494PubMedCrossRef

28.

Saggar R, Khanna D, Furst DE, Shapiro S, Maranian P, Belperio JA et al (2010) Exercise-induced pulmonary hypertension associated with systemic sclerosis: four distinct entities. Arthritis Rheum 62(12):3741–3750PubMedCrossRef

29.

Hager WD, Collins I, Tate JP, Azrin M, Foley R, Lakshminarayanan S et al. (2012) Exercise during cardiac catheterization distinguishes between pulmonary and left ventricular causes of dyspnea in systemic sclerosis patients. Clin Respir J [Epub ahead of print]

30.

Tschope C, Kasner M, Westermann D, Gaub R, Poller WC, Schultheiss HP (2005) The role of NT-proBNP in the diagnostics of isolated diastolic dysfunction: correlation with echocardiographic and invasive measurements. Eur Heart J 26(21):2277–2284PubMedCrossRef

Title: Exaggerated increase of exercise-induced pulmonary artery pressure in systemic sclerosis patients predominantly results from left ventricular diastolic dysfunction
Authors: Michał Ciurzyński
Piotr Bienias
Katarzyna Irzyk
Maciej Kostrubiec
Zbigniew Bartoszewicz
Maria Siwicka
Marcin Kurzyna
Urszula Demkow
Piotr Pruszczyk
Publication date: 01-11-2013
Publisher: Springer Berlin Heidelberg
Published in: Clinical Research in Cardiology / Issue 11/2013
Print ISSN: 1861-0684
Electronic ISSN: 1861-0692
DOI: https://doi.org/10.1007/s00392-013-0594-x

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Heart Failure Video

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.

Watch now

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Exaggerated increase of exercise-induced pulmonary artery pressure in systemic sclerosis patients predominantly results from left ventricular diastolic dysfunction

Abstract

Objective

Methods

Results

Conclusions

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusions

Please log in to get access to this content

Other articles of this Issue 11/2013

Drug-coated balloons for treatment of coronary artery disease: updated recommendations from a consensus group

Reperfusion therapy and mortality in octogenarian STEMI patients: results from the Belgian STEMI registry

Treatment of coronary artery disease via MIDCAB in a patient with severe thalidomide phocomelia

Circulating cells as predictors of secondary manifestations of cardiovascular disease: design of the CIRCULATING CELLS study

The revised EuroSCORE II for the prediction of mortality in patients undergoing transcatheter aortic valve implantation

Long-term safety and efficacy of dual therapy with oral anticoagulation and clopidogrel in patients with atrial fibrillation treated with drug-eluting stents

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page