Prognostic Value of Right Heart Involvement in Systemic Sclerosis:Not Only Pulmonary Arterial Hypertension

Luna Gargani¹, Cosimo Bruni², Antonella Moreo³, Laura Belloli³, Silvia Bellando-Randone², Oscar Massimiliano Epis³, Francesco Ferrara⁴, Olga Vriz⁵, Eduardo Bossone⁶, Petar M. Seferovic⁷, Daniel E. Furst⁸ and Marco Matucci-Cerinic⁹, ¹Institute of Clinical Physiology, Consiglio Nazionale delle Ricerche (CNR), Pisa, Italy, ²Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Firenze, Italy, ³Niguarda Ca’ Granda Hospital, Milan, Italy, ⁴"Cava de' Tirreni and Amalfi Coast", University Hospital, Salerno, Italy, ⁵King Faisal Hospital, Riyadh, Saudi Arabia, ⁶Cardarelli Hospital, Naples, Italy, ⁷Department of Cardiology, , Clinical Center of Serbia and Belgrade University School of Medicine, Belgrade, Serbia, ⁸University of California Los Angeles, Los Angeles, CA, ⁹Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Cardiovascular disease, systemic sclerosis and ultrasound

Session Information

Date: Monday, October 22, 2018

Title: Systemic Sclerosis and Related Disorders – Clinical Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: In SSc, cardiac dysfunction is associated with poor prognosis. Right

ventricular involvement is frequently secondary to PAH. Echocardiography is the routine imaging tool

to detect cardiac involvement and pulmonary hemodynamic. Our aim was to evaluate right ventricular

function and non-invasive hemodynamic by standard trans-thoracic Doppler echocardiography in SSc

patients without known cardiac involvementand PAH, compared to healthy controls.

Methods: 343 SSc patients (mean age, 54.2±14.8 years) and 340 healthy age-matched controls

(mean age 52.0±16.9 years, p=ns) prospectively underwent a comprehensive trans-thoracic 2D and

Doppler echocardiography, including tissue Doppler imaging analysis (TDI) of both the right and left

heart. Patients with known cardiac involvement and/or a diagnosis of PAH were excluded. Patients

were followed-up and cardiac events were recorded as new onset of heart failure (HF), development

of PAH, significant ventricular or supra-ventricular arrhythmias requiring therapy or implantable

cardioverter-defibrillator (ICD).

Results: Compared to controls, SSc patients did not show differences in systolic left ventricular

function, while a worse diastolic left ventricular function, as well as worse right ventricular function and

pulmonary circulation parameters were found(table). Mean follow-up was 32 ± 28 months. During the

follow-up a total of 52 events occurred. Echocardiographic predictors of cardiac events at univariate

analysis were E/e’ (e’ as mean of TDI lateral and septal values), right ventricular (RV) end-diastolic

diameter, pulmonary artery systolic pressure (PASP), and presence of even trivial pericardial effusion.

At multivariate analysis, only PASP was an independent predictor (hazard ratio 1.05, C.I. 1.01-1.10,

p<0.0001).

Conclusion: SSc patients without overt cardiac dysfunction and no PAH show worse parameters of

right ventricular function and pulmonary hemodynamic compared to controls, although still within

normal values. These indexes of subclinical right heart involvement provide prognostic information and

are independent predictors of further cardiac events, even in patients without PAH.

Disclosure: L. Gargani, Geeneral Electronics, 5,GlaxoSmithKline, 5; C. Bruni, None; A. Moreo, None; L. Belloli, None; S. Bellando-Randone, None; O. M. Epis, None; F. Ferrara, None; O. Vriz, None; E. Bossone, None; P. M. Seferovic, None; D. E. Furst, None; M. Matucci-Cerinic, None.

To cite this abstract in AMA style:

Gargani L, Bruni C, Moreo A, Belloli L, Bellando-Randone S, Epis OM, Ferrara F, Vriz O, Bossone E, Seferovic PM, Furst DE, Matucci-Cerinic M. Prognostic Value of Right Heart Involvement in Systemic Sclerosis:Not Only Pulmonary Arterial Hypertension [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/prognostic-value-of-right-heart-involvement-in-systemic-sclerosisnot-only-pulmonary-arterial-hypertension/. Accessed .

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