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. 2022 Jul 21;24(1):173.
doi: 10.1186/s13075-022-02863-1.

Prognostic meaning of right ventricular function and output reserve in patients with systemic sclerosis

Panagiota Xanthouli  1   2   3   4 , Julia Miazgowski  1 , Nicola Benjamin  1   2 , Ojan Gordjani  1 , Benjamin Egenlauf  1   2   3 , Satenik Harutyunova  1   2   3 , Rebekka Seeger  1   2   3 , Alberto M Marra  1   2   5 , Norbert Blank  4 , Hanns-Martin Lorenz  4 , Ekkehard Grünig  1   2   3 , Christina A Eichstaedt  6   7   8
Affiliations

Prognostic meaning of right ventricular function and output reserve in patients with systemic sclerosis

Panagiota Xanthouli et al. Arthritis Res Ther. .

Abstract

Background: The objective of this study was to investigate the prognostic impact of right ventricular (RV) function at rest and during exercise in patients with systemic sclerosis (SSc) presenting for a screening for pulmonary hypertension (PH).

Methods: In this study, data from SSc patients who underwent routinely performed examinations for PH screening including echocardiography and right heart catheterization at rest and during exercise were analysed. Uni- and multivariable analyses were performed to identify prognostic parameters.

Results: Out of 280 SSc patients screened for PH, 225 were included in the analysis (81.3% female, mean age 58.1±13.0 years, 68% limited cutaneous SSc, WHO-FC II-III 74%, 24 manifest PH). During the observation period of 3.2±2.7 (median 2.6) years 35 patients died. Tricuspid annular plane systolic excursion (TAPSE) at rest <18 mm (p=0.001), RV output reserve as increase of cardiac index (CI) during exercise <2 l/min (p<0.0001), RV pulmonary vascular reserve (Δ mean pulmonary artery pressure/Δ cardiac output) ≥3 mmHg/l/min (p<0.0001), peak CI <5.5 l/min/m2 (p=0.001), pulmonary arterial compliance <2 ml/mmHg (p=0.002), TAPSE/systolic pulmonary arterial pressure (sPAP) ratio ≤0.6 ml/mmHg (p<0.0001) and echocardiographic qualitative RV function at rest (p<0.0001) significantly predicted worse survival. In the multivariable analysis TAPSE/sPAP ratio and diffusion capacity for carbon monoxide ≤65% were identified as independent prognostic predictors and had 75% sensitivity and 69% specificity to predict future development of pulmonary vascular disease (PVD) during follow-up.

Conclusions: This study demonstrates that assessment of RV function at rest and during exercise may provide crucial information to identify SSc patients who are at a high risk of poor outcome and for the development of PH and/or PVD.

Keywords: Echocardiography; Pulmonary hypertension; Right heart catheterisation; Right ventricular reserve; Screening; Systemic sclerosis.

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Conflict of interest statement

PX: has received personal fees from MSD and OMT outside the submitted work.

JM, OJ, RS, CAE, NBe: nothing to declare.

BE: received travel fees, consulting fees, speaking fees, and/or honoraria from Actelion, MSD, Bayer and OMT, outside the submitted work.

SH: received honoraria for lectures, consultancy, or both from Actelion, AOP, Bayer/MSD, GSK, Novartis, OMT, Pfizer, and United Therapeutics out-side the submitted work.

AMM: AMM has received personal fees from Bayer, outside the submitted work.

NBl: has received consulting fees, speaking fees and/or honoraria and unallocated funds from Roche out-side the submitted work.

HML: has received consulting fees, speaking fees and/or honoraria and unallocated funds for scientific talks and consultations from Abbvie, Astra-Zeneca, Actelion, Alexion, Amgen, Bayer Vital, Baxter, Biogen, Boehringer Ingelheim, BMS, Celgene, Fresenius, Genzyme, GSK, Gilead/Galapagos, Hexal, Janssen-Cilag, Lilly, Medac, MSD, Mundipharm, Mylan, Novartis, octapharm, Pfizer, Roche/Chugai, Sandoz, Sanofi, Shire, SOBI, Thermo Fisher, UCB.

EG: has received grants and personal fees from Actelion, Bayer AG, and MSD; grants from GSK, Novartis, and United Therapeutics and personal fees from SCOPE, OrPha Swiss GmbH, and Zurich Heart House, outside the submitted work. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Fig. 1
Fig. 1
Study flow-chart. The graph provides information on patient-flow, baseline haemodynamics and follow-up. mPAP, mean pulmonary artery pressure; P(A)H, pulmonary (arterial) hypertension; PAWP, pulmonary arterial wedge pressure; PVD, pulmonary vascular disease; PVR, pulmonary vascular resistance
Fig. 2
Fig. 2
Kaplan-Meier survival analysis of echocardiographic right ventricular function. Patients with a tricuspid annular plane systolic excursion <18 mm assessed by echocardiography, b any impairment of RV function or with c TAPSE/sPAP ratio ≤0.6 mm/mmHg had significantly worse survival than patients with tricuspid annular plane systolic excursion ≥18 mm, normal RV function, or TAPSE/sPAP ratio >0.6 mm/mmHg
Fig. 3
Fig. 3
Kaplan-Meier analysis of invasively determined right ventricular function. Patients with a pulmonary vascular resistance ≥2 Wood Units, b pulmonary artery compliance <2 ml/mmHg, c cardiac index increase <2 l/min/m2, d peak cardiac index <5.5 l/min/m2 and/or e RV pulmonary vascular reserve (defined as the increase of mean pulmonary artery pressure/increase of cardiac output during exercise) ≥3 mmHg/(l/min) showed worse survival than SSc patients above the respective thresholds
Fig. 4
Fig. 4
Kaplan-Meier analysis of multivariable risk set. Multivariable Cox regression analysis identified TAPSE/sPAP ratio ≤0.6 mm/mmHg and diffusion capacity for carbon monoxide of the lung (DLCO) ≤65% predicted as independent prognostic predictors of survival. Patients with none of these risk factors had significantly better survival than patients with one or two risk factors
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