Arrhythmic Burden, Myocardial Markers, and Long-term Survival in Distinct Cardiac Magnetic Resonance Subsets of Systemic Sclerosis

Silvia Laura Bosello¹, Enrico De Lorenzis², Giacomo De Luca³, Antonio Tonutti⁴, veronica Batani⁵, Pier Giacomo Cerasuolo⁶, Gerlando Natalello⁷, Lucia Lanzo⁶, Gabriella Alonzi⁶, Silvia Fiore⁶, Stefano Di Murro⁶, Andrea Zoli⁶, Valentina Boni⁶, Riccardo Marano⁸, Francesca Augusta Gabrielli⁹, Francesco Del Galdo¹⁰, Dan Knight¹¹, Vivek Muthurangu¹¹, Christopher Denton¹², Maria De Santis⁴, Marco Matucci Cerinic⁵ and Maria Antonietta D'Agostino¹³, ¹Unit of Rheumatology, Catholic University of the Sacred Heart, Rome, Italy, ²Catholic University of the Sacred Heart, Roma, Rome, Italy, ³Vita-Salute San Raffaele University & IRCCS San Raffaele Hospital, Milan, Milan, Italy, ⁴Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy, ⁵Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, ⁶Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ⁷Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, ⁸Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Diagnostic Imaging Area, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, Rome, Italy, ⁹Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ¹⁰University of Leeds, Leeds, United Kingdom, ¹¹UCL Department of Cardiac MRI, University College London (Royal Free Campus), London, United Kingdom, ¹²University College London, Northwood, United Kingdom, ¹³Division of Rheumatology, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Università Cattolica del Sacro Cuore, Rome, Italy

Meeting: ACR Convergence 2024

Keywords: Heart disease, Magnetic resonance imaging (MRI), Mortality, Scleroderma

Session Information

Date: Saturday, November 16, 2024

Title: Systemic Sclerosis & Related Disorders – Clinical Poster I

Session Type: Poster Session A

Session Time: 10:30AM-12:30PM

Background/Purpose: Cardiac involvement in Systemic Sclerosis (SSc) is widely recognized as heterogeneous and, when clinically evident, it is associated with a poor prognosis. Recently, five cardiac magnetic resonance (CMR) phenotypes in SSc have been identified (Knight DS et al. European Heart Journal 2023). These phenotypes do not align with the existing clinical subgroup classifications or autoantibody statuses, yet each has a distinct 5-year prognosis. Our objective is to test the long-term prognostic significance of this classification system in an external cohort and to compare ECG Holter monitor parameters, NT-proBNP, and troponin T levels across these groups.

Methods: CMR assessments were conducted in three Italian tertiary centers on consecutive SSc patients who presented symptoms of dyspnoea, palpitations, or chest pain. Based on the CMR findings, patients were categorized into five distinct groups: those with dilated right hearts with right ventricular failure (RVF); biventricular failure with dilatation and dysfunction (BVF); and those with normal function, categorized further based on cavity sizes as average cavity (NF-AC), small cavity (NF-SC), and large cavity (NF-LC). The study focused on comparing clinical characteristics, serum troponin and NT-proBNP levels, ECG Holter monitor parameters, and 10-year survival outcomes across these groups.

Results: Table 1 presents the clinical characteristics and assessed parameters of the 143 enrolled patients across the CMR subsets. The distributions for NF-AC, NF-SC, NF-LC, BVF, and RVF were 46.2%, 22.4%, 14.0%, 14.0%, and 3.5%, respectively. Proportions of male patients and pulmonary function tests showed statistically significant differences across the subsets. Troponin T and NT-proBNP values were similar across all subsets. The NF-LC and RVF groups exhibited Left Bundle Branch Block (LBB) more frequently and more ventricular ectopic beats (VEB) compared to other groups. There was a variation in 10-year survival rates across the groups, with patients in the RVF, NF-LC, and BVF categories showing poorer prognosis (Figure 1).

Conclusion: This data confirms the prognostic value of the proposed CMR subsets in an another European SSc cohort, highlighting that subsets with poorer prognosis are associated with a higher arrhythmic burden.

Table 1. Comparison of clinical characteristics, serum troponin, serum NTproBNP and Holter ECG across five cardiac MRI patterns.

Figure 1. Survival comparison across five cardiac MRI patterns.

Disclosures: S. Bosello: None; E. De Lorenzis: None; G. De Luca: Boehringer-Ingelheim, 6; A. Tonutti: None; v. Batani: None; P. Cerasuolo: None; G. Natalello: None; L. Lanzo: None; G. Alonzi: None; S. Fiore: None; S. Di Murro: None; A. Zoli: None; V. Boni: None; R. Marano: None; F. Gabrielli: None; F. Del Galdo: AbbVie, 2, 5, Argenx, 2, Arxx, 2, 5, AstraZeneca, 2, 5, Boehringer Ingelheim, 2, 5, Chemomab, 5, Deepcure, 2, GlaxoSmithKline (GSK), 2, Janssen, 2, Mitsubishi Tanabe, 5, Novartis, 2, Ventus, 2; D. Knight: None; V. Muthurangu: None; C. Denton: AbbVie, 2, 5, Acceleron, 2, Arxx Therapeutics, 2, 5, Bayer, 2, Boehringer Ingelheim, 2, 6, Certa, 2, Corbus, 2, CSL Behring, 2, 5, Galapagos, 2, GlaxoSmithKline, 2, 5, 6, Horizon, 2, 5, Inventiva, 2, Janssen, 2, 6, Lilly, 2, Novartis, 2, Roche, 2, Sanofi-Aventis, 2, Servier, 5, Zurabio, 2; M. De Santis: None; M. Matucci Cerinic: None; M. D'Agostino: AbbVie, 2, 6, Amgen, 2, 6, BMS, 2, 6, Boehringer Ingelheim, 2, 6, Eli Lilly, 2, 6, Galapagos, 2, 6, Janssen, 2, 6, Novartis, 2, 6, Pfizer, 2, 6, UCB Pharma, 2, 6.

To cite this abstract in AMA style:

Bosello S, De Lorenzis E, De Luca G, Tonutti A, Batani v, Cerasuolo P, Natalello G, Lanzo L, Alonzi G, Fiore S, Di Murro S, Zoli A, Boni V, Marano R, Gabrielli F, Del Galdo F, Knight D, Muthurangu V, Denton C, De Santis M, Matucci Cerinic M, D'Agostino M. Arrhythmic Burden, Myocardial Markers, and Long-term Survival in Distinct Cardiac Magnetic Resonance Subsets of Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/arrhythmic-burden-myocardial-markers-and-long-term-survival-in-distinct-cardiac-magnetic-resonance-subsets-of-systemic-sclerosis/. Accessed .

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/arrhythmic-burden-myocardial-markers-and-long-term-survival-in-distinct-cardiac-magnetic-resonance-subsets-of-systemic-sclerosis/