How to Assess Pulmonary Circulation and Right Heart Chambers in Systemic Sclerosis Patients?

TL;DR

This paper reviews diagnostic tools for detecting heart and lung issues in patients with systemic sclerosis, a rare autoimmune disease.

Contribution

The paper evaluates diagnostic methods for early detection of right heart and pulmonary issues specific to systemic sclerosis patients.

Findings

Various diagnostic tools are analyzed for identifying right ventricle impairment in systemic sclerosis patients.

The paper highlights the need for distinct outcome measures and cut-off values specific to systemic sclerosis-related pulmonary hypertension.

Abstract

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by a widespread accumulation of extracellular matrix components leading to fibrosis of the skin and internal organs. Vascular changes occur in all involved tissues and are responsible for several distinctive clinical manifestations of the disease. This review focuses on the usefulness of various diagnostic tools in clinical practice for the early identification of clinical, functional, and/or structural RV impairment in SSc patients at risk of PH. It aims to identify specific causes of RV dysfunction, describe potential differences in outcome measures, and, ultimately, determine different cut-off values compared to subjects with PH not related to SSc.

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