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

Area of Science:

• Cardiology
• Rheumatology
• Pathology

Background:

• Cardiac involvement is a leading cause of death in systemic sclerosis (SSc).
• Acute ventricular dysfunction and cardiogenic shock in SSc patients have unclear underlying causes.
• This study investigates the characteristics and outcomes of SSc patients with acute ventricular dysfunction and cardiogenic shock.

Purpose of the Study:

• To elucidate the clinical, imaging, and pathological features of acute ventricular dysfunction in systemic sclerosis (SSc) patients presenting with cardiogenic shock.
• To assess the in-hospital and six-month outcomes of this patient cohort.
• To identify potential drivers of cardiac dysfunction in SSc.

Main Methods:

• Observational study of 10 SSc patients with cardiogenic shock and acute ventricular dysfunction (2010-2023).
• Analysis of clinical, laboratory, imaging (including cardiac MRI), and pathological data.
• Six-month outcome assessment.

Main Results:

• The cohort was predominantly female (90%) with diffuse cutaneous SSc (70%) and significant hemodynamic instability.
• All patients had pericardial effusion, and cardiac MRI revealed late gadolinium enhancement, prolonged T2 relaxation times, and reduced left ventricular ejection fraction (LVEF).
• Myocarditis with inflammatory cell infiltration was confirmed pathologically; in-hospital mortality was 60%, with limited LVEF recovery at six months in survivors.

Conclusions:

• Acute ventricular dysfunction and cardiogenic shock in SSc are associated with high mortality and poor recovery.
• The phenotype is linked to diffuse cutaneous SSc and musculoskeletal involvement, with myocarditis as a probable underlying mechanism.
• There is an urgent need for enhanced cardiac-focused management strategies for SSc patients.