Role of Left Ventricular Dysfunction in Systemic Sclerosis-Related Pulmonary Hypertension

Abstract

In systemic sclerosis (SSc), pulmonary hypertension remains a significant cause of morbidity and mortality. Although conventionally classified as group 1 pulmonary arterial hypertension, systemic sclerosis-related pulmonary hypertension (SSc-PH) is a heterogeneous disease. The contribution of left-sided cardiac disease in SSc-PH remains poorly understood. How often does left ventricular (LV) dysfunction occur in SSc among patients undergoing right heart catheterization and how does coexistent LV dysfunction with SSc-PH affect all-cause mortality in this patient population? We conducted a retrospective, observational study of 165 patients with SSc who underwent both echocardiography and right heart catheterization. LV dysfunction was identified using LV global longitudinal strain (GLS) on speckle-tracking echocardiography based on a defined threshold of >-18%. SSc-PH was defined by a mean pulmonary artery pressure > 20mmHg. Among patients with SSc who have undergone right heart catheterization, LV dysfunction occurred in 74.2%with SSc-PH and 51.2%without SSc-PH. The median survival of patients with SSc-PH and LV dysfunction was 67.9 (95%CI, 38.3-102.0) months, with a hazard ratio of 12.64 (95%CI, 1.73-92.60) for all-cause mortality when adjusted for age, sex, SSc disease duration, and FVC compared with patients with SSc without pulmonary hypertension with normal LV function. LV dysfunction is common in SSc-PH. Patients with SSc-PH and LV dysfunction by LV GLS have increased all-cause mortality. This suggests that LV GLS may be helpful in identifying underlying LV dysfunction and in risk assessment of patients with SSc-PH.