Pneumologie 2018; 72(S 01): S7
DOI: 10.1055/s-0037-1619133
Sektion 6 – Kardiorespiratorische Interaktion
Freie Vorträge – Titel: Kardiale Funktion bei pneumologischen Patienten, neue Messmethoden und Frühdiagnostik
Georg Thieme Verlag KG Stuttgart · New York

Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis after negative right heart catheterisation

G Coghlan
1   Cardiology Department, Royal Free Hospital, London
,
M Wolf
2   Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg
,
O Distler
3   Department of Rheumatology, Faculty of Medicine, University of Zurich
,
CP Denton
4   Centre of Rheumatology, Royal Free Hospital, London
,
M Doelberg
5   Actelion Pharmaceuticals Ltd., Switzerland
,
S Harutyunova
2   Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg
,
A Marra
2   Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg
,
N Benjamin
2   Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg
,
C Fischer
6   Institute of Biostatistics Heidelberg
,
E Grünig
7   Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg; Translational Lung Research Center (TLRC) of the German Center for Lung Research (DZL), Heidelberg
› Author Affiliations
Further Information

Publication History

Publication Date:
21 February 2018 (online)

Also available at
 

Background:

Pulmonary hypertension (PH) is a common complication of systemic sclerosis (SSc) which can occur at any stage of the disease and has been observed in 15 – 27% of symptomatic patients and 8 – 12% of asymptomatic patients using right heart catheterization (RHC) for screening. The diagnosis of PAH is defined by a mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, a pulmonary arterial wedge pressure ≤15 mmHg and a pulmonary vascular resistance > 3 wood units, measured by RHC. According to the current guidelines, the clinical significance of a mPAP between 21 and 24 is not known. The objective of this study was to evaluate the incidence of PH and determining factors in patients with SSc.

Methods:

In this bi-centric, prospective cohort study, patients with SSc were assessed at baseline and after 3 years clinically including (RHC). Analysis of determining factors for development of PH was performed using univariate and multivariate analysis. Results:

Ninety-six patients with (mPAP) < 25 mmHg at baseline were followed 2.95 ± 0.7 (median 3) years, 71 had a second RHC, 18 of the 71 patients (25.3%) developed PH, 5 (7%) a SSc-associated pulmonary arterial hypertension. Patients with mPAP between 21 and 24 mmHg at baseline significantly more often presented PH or “borderline” pressures during follow-up (p < 0.001) than patients with normal pressures. Pulmonary vascular resistance, tricuspid regurgitation velocity, diffusion capacity and size of inferior vena cava at baseline were independent predictive for development of PH during follow-up. Conclusions:

In SSc-patients pulmonary pressures appear to rise progressively during follow-up. Using RHC during follow-up it was possible to identify manifest PH in almost 25% of patients. This is the first prospective study indicating that borderline elevation of mPAP is associated with higher incidence of PH in high risk SSc-patients using systematic recatheterisation. Therefore, regular clinical assessment including RHC might be useful in SSc-patients.