Pulmonary Circulation on the Crossroads Between the Left and Right Heart in Systemic Sclerosis: A Clinical Challenge for Cardiologists and Rheumatologists

Heart Fail Clin. 2018 Jul;14(3):271-281. doi: 10.1016/j.hfc.2018.02.004.

Abstract

Involvement of the right heart-pulmonary circulation system in systemic sclerosis is a typical feature, with critical prognostic implications. Pulmonary hypertension may occur in association with interstitial lung disease or as a result of an isolated pulmonary vascular disease that may affect both the precapillary arterioles and the postcapillary venules, as well as a consequence of left heart involvement. These apparently different phenotypes often underlie a significant pathophysiologic overlap, which makes the diagnosis and management of these patients highly complex and uncertain.

Keywords: Pulmonary circulation; Pulmonary hypertension; Right heart; Systemic sclerosis.

Publication types

  • Review

MeSH terms

  • Cardiologists
  • Heart / physiopathology*
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / therapy
  • Mass Screening / methods*
  • Pulmonary Circulation / physiology*
  • Rheumatologists
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / therapy