Mechanics and prognostic value of left and right ventricular dysfunction in patients with systemic sclerosis.

AIMS: Impairment of myocardial function is an important potential complication of systemic sclerosis (SSc) and associated with poor prognosis. The detection of left (LV) and right ventricular (RV) dysfunction may prompt specific therapeutic interventions. We sought to investigate the prognostic value of both LV and RV deformation in patients with SSc.

METHODS AND RESULTS: Speckle tracking LV strain parameters [global longitudinal strain (GLS) and global circumferential strain (GCS)] and tricuspid annular peak systolic velocity (Ts') were measured in 103 patients with SSc and 103 age- and gender-matched controls. Subjects were followed for a median of 3.4 years for heart failure-specific admission or death, and the association of the study parameters with outcome was assessed using Cox proportional hazards models. GLS, GCS, and Ts' were significantly impaired in patients with SSc, even without pulmonary hypertension, compared with controls. GCS (r2 = 0.07, P = 0.03) but not GLS (r2 = 0.04, P = 0.11) was associated with systolic pulmonary artery pressure. During follow-up, SSc patients (n = 17, 17%) had more events than controls (n = 7, 7%, P = 0.04). In SSc patients, GCS (but not GLS), Ts', and 6-minute walk distance (6MWD) were significantly associated with outcome. 6MWD and Ts' (but not GCS) were independently associated with outcome. A model based on age, gender, 6MWD, and GCS was significantly improved by adding by adding Ts' (P = 0.02).

CONCLUSION: RV dysfunction was associated with adverse outcome, independent of and incremental to clinical and LV deformation parameters in SSc. LV dysfunction appears to have less prognostic relevance than RV dysfunction.