Role of exercise stress echocardiography in systemic sclerosis: pathophysiological and prognostic significance of the systemic sclerosis with a heart failure and preserved ejection fraction phenotype

Aims: Left ventricular (LV) diastolic dysfunction and heart failure with preserved ejection fraction (HFpEF) are common cardiac complications of patients with systemic sclerosis (SSc). Exercise stress echocardiography is often used in symptomatic patients with SSc to detect abnormal increases in pulmonary pressures during exercise, but the pathophysiologic and prognostic significance of exercise stress echocardiography to assess the presence of HFpEF in these patients is unclear. Methods and results: Patients with SSc (n = 140) underwent ergometry exercise stress echocardiography with simultaneous expired gas analysis. The HFA-PEFF score ≥ 5 points was used to diagnose HFpEF. Thirty-five patients met the HFpEF criteria (prevalence 25%). Compared with patients with SSc-non-HFpEF, those with SSc-HFpEF were older and had a higher prevalence of coronary artery disease, more severe LV diastolic dysfunction (by definition), depressed right ventricular systolic function, reduced exercise capacity (lower peak oxygen consumption), and poorer ventilatory efficiency. Exercise right heart catheterization was performed in 25 patients and it confirmed elevated pulmonary capillary wedge pressure during peak exercise in patients with SSc-HFpEF. Participants were followed up to assess the primary composite endpoint: all-cause mortality, HF hospitalization, unplanned hospital visits requiring intravenous diuretics, or oral diuretic intensification. Compared with SSc-non-HFpEF, SSc-HFpEF had a 5.3-fold increased risk of the composite outcomes (hazard ratio 5.29, confidence intervals 2.06-13.5, P = 0.0005). Conclusion: In addition to pulmonary haemodynamics, exercise stress echocardiography may be useful to identify the HFpEF phenotype that has different pathophysiology and clinical outcomes in patients with SSc.