Left Ventricular Diastolic Dysfunction Predicts Mortality in Patients With Systemic Sclerosis

J Am Coll Cardiol. 2018 Oct 9;72(15):1804-1813. doi: 10.1016/j.jacc.2018.07.068.

Abstract

Background: Primary cardiac affection is common and is a major cause of death in systemic sclerosis (SSc), but there are knowledge gaps regarding the effect of cardiac dysfunction on mortality.

Objectives: The purpose of this study was to evaluate diastolic function in a large, unselected SSc cohort and assess the effect of diastolic dysfunction (DD) on mortality.

Methods: SSc patients followed prospectively at the Oslo University Hospital from 2003 to 2016 with available echocardiographies and matched control subjects were included. DD was assessed by echocardiography according to the 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging guidelines. Pulmonary hypertension (PH) was diagnosed by right heart catheterization. Vital status was available for all patients. Cox regression analyses with hazards ratios (HRs) were conducted.

Results: Diastolic function was assessed in 275 SSc patients at baseline and in 186 patients at follow-up. At baseline, 46 of the 275 SSc patients (17%) were diagnosed with DD and 195 (71%) had normal diastolic function. After a median follow-up of 3.4 years (interquartile range: 1.6 to 6.2 years), the proportion of DD increased from 17% to 29%. During follow-up, 57% of patients with DD at baseline died, compared with 13% of patients with normal diastolic function. At baseline, 86 patients had performed right heart catheterization, and 43 were diagnosed with PH; of these 60% deceased. In multivariable Cox regression analyses, DD was a stronger predictor of death (HR: 3.7; 95% CI: 1.69 to 8.14; c-index 0.89) than PH (HR: 2.0; 95% CI: 1.1 to 3.9; c-index 0.84).

Conclusions: DD is frequent in SSc, and the presence of DD is associated with high mortality. DD exceeds PH with respect to predicting mortality.

Keywords: diastolic dysfunction; echocardiography; mortality; systemic sclerosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cardiac Catheterization / methods
  • Echocardiography, Doppler / methods
  • Female
  • Follow-Up Studies
  • Heart Failure, Diastolic* / diagnosis
  • Heart Failure, Diastolic* / etiology
  • Heart Failure, Diastolic* / mortality
  • Humans
  • Hypertension, Pulmonary* / diagnosis
  • Hypertension, Pulmonary* / etiology
  • Male
  • Middle Aged
  • Mortality
  • Norway / epidemiology
  • Proportional Hazards Models
  • Scleroderma, Systemic* / complications
  • Scleroderma, Systemic* / mortality
  • Ventricular Dysfunction, Left* / diagnosis
  • Ventricular Dysfunction, Left* / etiology
  • Ventricular Dysfunction, Left* / mortality