Cardiogenic shock in systemic sclerosis: a retrospective study of acute ventricular dysfunction

Abstract

Cardiac involvement in systemic sclerosis (SSc) is a major cause of morbidity and mortality, with ventricular dysfunction and cardiogenic shock being among the most severe complications. The underlying causes of acute ventricular dysfunction in these patients remain unclear. This observational study included 10 SSc patients admitted with cardiogenic shock and acute ventricular dysfunction between 2010 and 2023, excluding those with prior heart disease. Clinical, laboratory, imaging, and pathological data were analyzed, with outcomes assessed at six months. The cohort was 90% female, with a mean age of 58.8 ± 3.8 years. Most had diffuse cutaneous SSc (70%) and musculoskeletal involvement (50%), with an average disease duration of 4.8 ± 5.2 years. All patients presented with severe hemodynamic instability, with a mean systolic blood pressure of 78.4 ± 6.7 mmHg and elevated troponin levels (2077 ± 3379 ng/L). Pericardial effusion was observed in all, and 30% required pericardiocentesis. CMR showed presence of late gadolinium enhancement and prolonged T2 relaxation time and reduced ventricular function (LVEF 31 ± 8%). Biopsies revealed myocarditis with T lymphocyte and macrophage infiltration. In-hospital mortality was 60%. Among survivors, partial ventricular recovery was seen at six months, with an average LVEF improvement of 10 ± 10%. SSc patients with cardiogenic shock and acute ventricular dysfunction face high mortality and limited recovery. The phenotype is associated with diffuse cutaneous SSc and musculoskeletal involvement, likely driven by myocarditis, highlighting the need for improved cardiac-focused treatments in SSc.

Keywords: Cardiac magnetic resonance; Cardiogenic shock; Endomyocardial biopsy; Heart failure; Myocarditis; Systemic sclerosis.