Decreased biventricular longitudinal strain in patients with systemic sclerosis is mainly caused by pulmonary hypertension and not by systemic sclerosis per se.

Anthony Lindholm,Ellen Ostenfeld,Håkan Arheden,Roger Hesselstrand,Göran Rådegran

doi:10.1111/cpf.12561

Anthony Lindholm, Ellen Ostenfeld + Show 3 more

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https://doi.org/10.1111/cpf.12561

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Abstract

SummaryPurposePatients with pulmonary arterial hypertension (PAH) due to systemic sclerosis (SSc) have high mortality. Left ventricular (LV) peak global longitudinal strain (GLS) is decreased in SSc. It is unknown whether low GLS is due to SSc or PAH. Therefore, our primary aim was to evaluate both LV and right ventricular free wall GLS (RVFW GLS) in SSc, with and without PAH, using cardiac magnetic resonance with feature tracking. Secondary aim was to relate GLS to invasive mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR).MethodsThirty‐eight patients with SSc, 19 patients with SSc‐PAH and 19 healthy controls for comparison, were included. Endocardial and epicardial borders were delineated in cine images (short‐axis stack and three long‐axis views) for volumetric and strain calculations.ResultsSystemic sclerosis‐PAH had lower LV and RVFW GLS than SSc (LV: P = 0·01, RV: P<0·001) and controls (LV: P = 0·02; RV: P<0·001), with no difference between SSc and controls. LV strain correlated with mPAP (R = 0·42, P = 0·03) and PVR (R = 0·52, P = 0·006). RVFW GLS correlated with mPAP (R = 0·68, P<0·001) and PVR (R = 0·59, P = 0·001). ROC curves for predicting PAH had AUC 0·73 for LV strain (P = 0·003) and 0·86 for RVFW GLS (P<0·001).ConclusionsLower GLS is mainly determined by increased pulmonary pressure and not by SSc per se. Low LV and RVFW GLS are indicative of increased mPAP and PVR, which opens for improved non‐invasive methods to select patients eligible for right heart catheterization and to monitor the effects of PAH therapy.

Highlights

Systemic sclerosis (SSc) is the connective tissue disease with the highest mortality rates, with more than half of the patients dying from disease-related complications (Denton, 2015)
Pulmonary arterial hypertension (PAH) was defined as mean pulmonary arterial pressure ≥25 mmHg and pulmonary arterial wedge pressure ≤15 mmHg at normal to low cardiac output measured with right heart catheterization (RHC) (Galie et al, 2016)
Peak global longitudinal left ventricular (LV) strain was lower in SSc-associated PAH (SSc-PAH) (À18 Æ 3%) compared to both SSc (À20 Æ 3%, P = 0Á01) and controls (À20 Æ 2%, P = 0Á02)

Summary

Introduction

Systemic sclerosis (SSc) is the connective tissue disease with the highest mortality rates, with more than half of the patients dying from disease-related complications (Denton, 2015). PAH is a rare and complex vascular disease (Peacock et al, 2007) with poor prognosis and high mortality rate, characterized by progressive increase in pulmonary vascular resistance (PVR) and loss of pulmonary vascular compliance (McLaughlin & McGoon, 2006; Galie et al, 2016). These hemodynamic changes lead to right ventricular (RV) failure and rapid progression to death (Lai et al, 2014). Left ventricular (LV) GLS has been shown to be low in patients with PAH as well as in some patients with SSc, with higher risk of poor outcome

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