Right Ventricle Remodeling and Function in Scleroderma Patients.

Adriana Mitre,Monica Chitu,Roxana Cucuruzac,Nora Rat,Iolanda Muntean,Andras Mester,Imre Benedek,Theodora Benedek

doi:10.1155/2018/4528148

Abstract

Scleroderma, known also as systemic sclerosis (SSc), is a severe disease associated with high mortality rates, and right ventricular (RV) remodeling and dysfunction, along with pulmonary artery hypertension (PAH), are among the most important internal organ manifestations of this disease. PAH has a higher prevalence in patients with SSc compared to the general population and represents a significant predictor of mortality in SSc. In patients with SSc, the morphological remodeling and alteration of RV function begin even before the setting of PAH and lead to development of a specific adaptive pattern of the RV which is different from the one recorded in patients with IAPH. These alterations cause worse outcomes and increased mortality rates in SSc patients. Early detection of RV dysfunction and remodeling is possible using modern imaging tools currently available and can indicate the initiation of specific therapeutic measures before installation of PAH. The aim of this review is to summarize the current knowledge related to mechanisms involved in the remodeling and functional alteration of the RV in SSc patients.

Highlights

Scleroderma, known as systemic sclerosis (SSc), is a severe disease associated with high mortality rates, and right ventricular (RV) remodeling and dysfunction, along with pulmonary artery hypertension (PAH), are among the most important internal organ manifestations of this disease [1].The mortality of SSc depends primary on the burden of the internal organ involvement
The aim of this review is to summarize the current knowledge related to mechanisms involved in the remodeling and functional alteration of the RV in SSc patients
Unpublished data from this study showed a significant decrease of the capillary density in Systemic Sclerosis-Related Pulmonary Artery Hypertension (SSc-PAH) samples compared to the HFpEF and SSc without

Summary

Introduction

Scleroderma, known as systemic sclerosis (SSc), is a severe disease associated with high mortality rates, and right ventricular (RV) remodeling and dysfunction, along with pulmonary artery hypertension (PAH), are among the most important internal organ manifestations of this disease [1]. SSc is a complex autoimmune disease characterized by a marked chronic inflammation, which leads to increased connective tissue fibrosis and vascular involvement causing severe damage of internal organs (mainly the heart, lungs, gastrointestinal tract, kidneys, and muscles) and the skin [5]. Despite the fact that hemodynamic changes are less pronounced in SScPAH compared to idiopathic PAH (IPAH), SSc-related PAH is associated with twofold to threefold higher mortality risk, due to the systemic nature and complexity of the disease, added to a limited efficiency of the administered therapy [22, 23]. Myocardial capillary density was significantly lower in samples from SSc patients with increased right atrial pressure (RAP), compared with lower RAP SSc samples, supporting the theory of structural RV alteration in these patients [27]

Right Ventricular Involvement in Scleroderma: A Distinctive Pattern

Imaging Tools for Characterization of the RV in Scleroderma

Findings

Conclusions