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

Presenting Author(s)

• RM

  Riccardo Marano, MD

  Medical Doctor, Associate Professor of Radiology
  Università Cattolica - Fondazione Policlinico Universitario "Agostino Gemelli" - IRCCS, Rome (Italy)
  Rome, Lazio, Italy

Primary Author(s)

• 

  Luigi Natale, MD

  Medical Doctor, Associate Professor of Radiology
  Catholic University - Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome (Italy)
  Rome, Lazio, Italy

Co-Author(s)

• AM

  Alessandro Marchetti, MD

  Radiology Resident
  Catholic University of Rome, “Agostino Gemelli" University Polyclinic Foundation IRCCS - Rome (Italy)
  Roma, Lazio, Italy
• EM

  Eleonora Moliterno, MD

  Radiologist
  San Carlo di Nancy Hospital - Rome (Italy), Italy
• GR

  Giuseppe Rovere, MD

  Medical Doctor, Diagnostic Radiologist
  Fondazione Policlinico Universitario "A.Gemelli" - IRCCS, Rome (Italy)
  Rome, Lazio, Italy
• 

  Francesco Lauriero, MD

  Medical Doctor, Diagnostic Radiologist
  Fondazione Policlinico Universitario "A. Gemelli" - IRCCS, Rome (Italy)
  Rome, Lazio, Italy
• SB

  Silvia Laura Bosello, MD

  Assistant Professor
  Catholic University of Rome, “Agostino Gemelli" University Polyclinic Foundation IRCCS - Rome (Italy), Italy
• ED

  Enrico De Lorenzis, MD

  Rheumatologist
  “Agostino Gemelli" University Polyclinic Foundation IRCCS - Rome (Italy), Italy
• GD

  Giacomo De Luca, MD

  Rheumatologist
  Vita-Salute San Raffaele University & IRCCS San Raffaele Hospital, Milan (Italy), Italy
• MD

  Maria De Santis, MD

  Professor of Rheumatologist
  IRCCS Humanitas Research Hospital, Rozzano (Italy), Italy
• MD

  Maria Antonietta D'Agostino, MD

  Full Professor of Rheumatology - Head of Rheumatology Complex Unit
  Catholic University of Rome, “Agostino Gemelli" University Polyclinic Foundation IRCCS - Rome (Italy), Italy
• AM

  Agostino Meduri, MD

  Assistant Professor
  Catholic University of Rome, “Agostino Gemelli" University Polyclinic Foundation IRCCS - Rome (Italy), Italy

Background: Cardiac involvement in Systemic Sclerosis (SSc) is widely recognized as heterogeneous and, when clinically evident, associated with a poor prognosis. Recently, 5 CMR phenotypes in SSc have been identified, not aligning 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 pulmonary function tests, ECG-Holter monitor parameters, NT-proBNP and troponin T levels and 10-year survival outcomes across these groups.

Methods: CMR assessments were conducted in 3 tertiary centers on 143 consecutive SSc patients, who presented symptoms of dyspnea, palpitations or chest pain. Based on the CMR findings, patients were categorized into 5 distinct groups: dilated right hearts with right ventricular failure (RVF); biventricular failure with dilatation and dysfunction (BVF), normal function with average cavity (NF-AC), small cavity (NF-SC), and large cavity (NF-LC).

Results: 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 NFLC and RVF groups exhibited Left Bundle Branch Block (LBB) and ventricular ectopic beats (VEB) more frequently 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.

Conclusion: These data confirm 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.

Based on the findings (biventricular volumes & function) patients were categorized in to 5 distinct groups. The survival analysis was performed including ECG-Holter, NTpro-BN & TnT assay, pulmonary function tests, and cardiac-MR.