Rangarajan V, Matiasz R, Freed BH (2017) Cardiac complications of systemic sclerosis and management: recent progress. Curr Opin Rheumatol 29(6):574–584. https://doi.org/10.1097/BOR.0000000000000439

Article  PubMed  Google Scholar 

Champion HC (2008) The heart in scleroderma. Rheum Dis Clin North Am 34(1):181–190; viii. https://doi.org/10.1016/j.rdc.2007.12.002

Ross L, Prior D, Proudman S, Vacca A, Baron M, Nikpour M (2019) Defining primary systemic sclerosis heart involvement: a scoping literature review. Semin Arthritis Rheum 48(5):874–887. https://doi.org/10.1016/j.semarthrit.2018.07.008

Article  PubMed  Google Scholar 

Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, Riemekasten G, Airo P, Joven B, Vettori S et al (2017) Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 76(11):1897–1905. https://doi.org/10.1136/annrheumdis-2017-211448

Article  PubMed  Google Scholar 

Dandel M, Lehmkuhl H, Knosalla C, Suramelashvili N, Hetzer R (2009) Strain and strain rate imaging by echocardiography - basic concepts and clinical applicability. Curr Cardiol Rev 5(2):133–148. https://doi.org/10.2174/157340309788166642

Article  PubMed Central  Google Scholar 

Mele D, Censi S, La Corte R, Merli E, Lo Monaco A, Locaputo A, Ceconi C, Trotta F, Ferrari R (2008) Abnormalities of left ventricular function in asymptomatic patients with systemic sclerosis using doppler measures of myocardial strain. J Am Soc Echocardiogr 21(11):1257–1264. https://doi.org/10.1016/j.echo.2008.08.004

Article  PubMed  Google Scholar 

Karadag DTSTTSI, Yazici OO, Eraldemir A, Cefle FC (2020) Evaluation of left and right ventricle by two-dimensional speckle tracking echocardiography in systemic sclerosis patients without overt cardiac disease. Clin Rheumatol 39(1):37–48. https://doi.org/10.1007/s10067-019-04604-3

Article  PubMed  Google Scholar 

Thomas L, Marwick TH, Popescu BA, Donal E, Badano LP (2019) Left atrial structure and function, and left ventricular diastolic dysfunction: JACC state-of-the-art review. J Am Coll Cardiol 73(15):1961–1977. https://doi.org/10.1016/j.jacc.2019.01.059

Article  PubMed  Google Scholar 

Agoston G, Gargani L, Miglioranza MH, Caputo M, Badano LP, Moreo A, Muraru D, Mondillo S, Moggi Pignone A, Matucci Cerinic M et al (2014) Left atrial dysfunction detected by speckle tracking in patients with systemic sclerosis. Cardiovasc Ultrasound 12:30. https://doi.org/10.1186/1476-7120-12-30

Article  PubMed  PubMed Central  Google Scholar 

Porpaczy A, Nogradi A, Kehl D, Strenner M, Minier T, Czirjak L, Komocsi A, Faludi R (2018) Impairment of Left Atrial mechanics is an early sign of myocardial involvement in systemic sclerosis. J Card Fail 24(4):234–242. https://doi.org/10.1016/j.cardfail.2018.02.012

Article  PubMed  Google Scholar 

Mukherjee MM, Tedford V, Shah RJ, Hsu AA, Mullin S, Sato CJ, Damico T, Kolb R, Mathai TM, Hassoun SC (2017) Right ventricular longitudinal strain is diminished in systemic sclerosis compared with idiopathic pulmonary arterial hypertension. Eur Respir J 50(5). https://doi.org/10.1183/13993003.01436-2017

Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J, Craig R, Hirano I, Marshall K, Ruderman E et al (2011) Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet 378(9790):498–506. https://doi.org/10.1016/S0140-6736(11)60982-3

Article  CAS  PubMed  Google Scholar 

van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, Schuerwegh AJ, Marijt EW, Vonk MC, Schattenberg AV et al (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311(24):2490–2498. https://doi.org/10.1001/jama.2014.6368

Article  CAS  PubMed  Google Scholar 

Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B, Mayes MD, Nash RA, Crofford LJ, Eggleston B et al (2018) Myeloablative autologous stem-cell transplantation for severe Scleroderma. N Engl J Med 378(1):35–47. https://doi.org/10.1056/nejmoa1703327

Article  PubMed  PubMed Central  Google Scholar 

Burt RK, Han X, Quigley K, Arnautovic I, Shah SJ, Lee DC, Freed BH, Jovanovic B, Helenowski IB (2021) Cardiac safe hematopoietic stem cell transplantation for systemic sclerosis with poor cardiac function: a pilot safety study that decreases neutropenic interval to 5 days. Bone Marrow Transpl 56(1):50–59. https://doi.org/10.1038/s41409-020-0978-2

Article  CAS  Google Scholar 

Burt RK, Oliveira MC, Shah SJ, Moraes DA, Simoes B, Gheorghiade M, Schroeder J, Ruderman E, Farge D, Chai ZJ et al (2013) Cardiac involvement and treatment-related mortality after non-myeloablative haemopoietic stem-cell transplantation with unselected autologous peripheral blood for patients with systemic sclerosis: a retrospective analysis. Lancet 381(9872):1116–1124. https://doi.org/10.1016/S0140-6736(12)62114-X

Article  PubMed  Google Scholar 

Farge D, Burt RK, Oliveira MC, Mousseaux E, Rovira M, Marjanovic Z, de Vries-Bouwstra J, Del Papa N, Saccardi R, Shah SJ et al (2017) Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners. Bone Marrow Transpl 52(11):1495–1503. https://doi.org/10.1038/bmt.2017.56

Article  CAS  Google Scholar 

Preliminary criteria for the classification of systemic sclerosis (scleroderma) (1980) Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 23(5):581–590. https://doi.org/10.1002/art.1780230510

Article  Google Scholar 

van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr., Carreira PE et al (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747. https://doi.org/10.1002/art.38098

Article  PubMed  PubMed Central  Google Scholar 

Clements PJ, Lachenbruch PA, Seibold JR, Zee B, Steen VD, Brennan P, Silman AJ, Allegar N, Varga J, Massa M et al (1993) Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol 20(11):1892–1896

CAS  PubMed  Google Scholar 

Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, Flachskampf FA, Foster E, Goldstein SA, Kuznetsova T et al (2015) Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr 28(1):1–39e14. https://doi.org/10.1016/j.echo.2014.10.003

Article  PubMed  Google Scholar 

Badano LP, Kolias TJ, Muraru D, Abraham TP, Aurigemma G, Edvardsen T, D’Hooge J, Donal E, Fraser AG, Marwick T et al (2018) Standardization of left atrial, right ventricular, and right atrial deformation imaging using two-dimensional speckle tracking echocardiography: a consensus document of the EACVI/ASE/Industry Task Force to standardize deformation imaging. Eur Heart J Cardiovasc Imaging 19(6):591–600. https://doi.org/10.1093/ehjci/jey042

Article  PubMed  Google Scholar 

Kurt M, Wang J, Torre-Amione G, Nagueh SF (2009) Left atrial function in diastolic heart failure. Circ Cardiovasc Imaging 2(1):10–15. https://doi.org/10.1161/CIRCIMAGING.108.813071

Article  PubMed  Google Scholar 

Pathan F, D’Elia N, Nolan MT, Marwick TH, Negishi K Normal ranges of left atrial strain by Speckle-Tracking Echocardiography: a systematic review and Meta-analysis. J Am Soc Echocardiogr 2017, 30(1):59–70e58. https://doi.org/10.1016/j.echo.2016.09.007

Sugimoto T, Dulgheru R, Bernard A, Ilardi F, Contu L, Addetia K, Caballero L, Akhaladze N, Athanassopoulos GD, Barone D et al (2017) Echocardiographic reference ranges for normal left ventricular 2D strain: results from the EACVI NORRE study. Eur Heart J Cardiovasc Imaging 18(8):833–840. https://doi.org/10.1093/ehjci/jex140

Article  PubMed  Google Scholar 

Tedford RJ, Mudd JO, Girgis RE, Mathai SC, Zaiman AL, Housten-Harris T, Boyce D, Kelemen BW, Bacher AC, Shah AA et al (2013) Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. Circ Heart Fail 6(5):953–963. https://doi.org/10.1161/CIRCHEARTFAILURE.112.000008

Article  CAS  PubMed  Google Scholar 

van Rhijn-Brouwer FCC, Spierings J, van Laar JM (2018) Autologous hematopoietic stem cell transplantation in systemic sclerosis: a reset to tolerance? Immunol Lett 195:88–96. https://doi.org/10.1016/j.imlet.2017.11.005

Article  CAS  PubMed  Google Scholar 

Mukherjee M, Mercurio V, Hsu S, Mayer SA, Mathai SC, Hummers LK, Kass DA, Hassoun PM, Wigley FM, Tedford RJ et al (2021) Assessment of right ventricular reserve utilizing exercise provocation in systemic sclerosis. Int J Cardiovasc Imaging 37(7):2137–2147. https://doi.org/10.1007/s10554-021-02237-9

Article  PubMed  PubMed Central  Google Scholar 

Tzelepis GE, Kelekis NL, Plastiras SC, Mitseas P, Economopoulos N, Kampolis C, Gialafos EJ, Moyssakis I, Moutsopoulos HM (2007) Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum 56(11):3827–3836. https://doi.org/10.1002/art.22971

Article  PubMed  Google Scholar 

Hsu S, Kokkonen-Simon KM, Kirk JA, Kolb TM, Damico RL, Mathai SC, Mukherjee M, Shah AA, Wigley FM, Margulies KB et al (2018) Right ventricular myofilament functional differences in humans with systemic sclerosis-Associated Versus Idiopathic Pulmonary arterial hypertension. Circulation 137(22):2360–2370. https://doi.org/10.1161/CIRCULATIONAHA.117.033147

Article  PubMed