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Original Research |

Idiopathic and Systemic Sclerosis associated Pulmonary Arterial Hypertension: A Comparison of Demographic, Haemodynamic and Magnetic Resonance Imaging Characteristics and Outcomes

Sheila Ramjug, MBChB; Nehal Hussain, MBChB; Judith Hurdman, MD; Catherine Billings, PhD; Athanasios Charampopoulos, MD; Charlie A. Elliot, MD; David G. Kiely, MD; Ian Sabroe, PhD; Smitha Rajaram, MD; Andrew J. Swift, PhD; Robin Condliffe, MD
Author and Funding Information

Conflicts of Interest:

SR No conflicts to declare

NH No conflicts to declare

JH JH was part funded as a clinical research fellow by an unrestricted educational grant from Actelion and has received funding to attend conferences from Actelion, GSK and Pfizer

CB Received grant from Actelion to attend educational meeting

AC Actelion, GSK, Servier: consultancy fees, honoraria and support for attendance to conferences

CAE Honoraraia for lecturing and advisory boards from Actelion, GSK, Bayer

DGK Honoraraia for lecturing and advisory boards from Actelion, GSK, Bayer. Research funding from Actelion, GSK and Bayer

IS No conflicts to declare

SR No conflicts to declare

AJS No conflicts to declare

RC Honoraraia for lecturing and advisory boards from Actelion, GSK, Bayer

1Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield

2Department of Infection and Immunity, University of Sheffield

3Department of Radiology, Sheffield Teaching Hospitals NHS Trust

4Academic Department of Radiology, University of Sheffield, Sheffield, United Kingdom

Correspondence to: Dr Robin Condliffe Sheffield Pulmonary Vascular Disease Unit Sheffield United Kingdom S10 2JF.


Copyright 2017, . All Rights Reserved.


Chest. 2017. doi:10.1016/j.chest.2017.02.010
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Abstract

Background  Previous studies have identified survival in systemic sclerosis (SSc)-associated pulmonary arterial hypertension (SSc-PAH) to be worse than in idiopathic pulmonary arterial hypertension (IPAH). We investigated differences between these conditions by comparing demographic, haemodynamic and radiological characteristics and outcomes in a large cohort of incident patients.

Methods  651 patients diagnosed with IPAH or SSc-associated pre-capillary pulmonary hypertension were included. Patients with lung disease or ≥2 risk factors for left heart disease were identified leaving a primary analysis set of 375. Sub-group analysis of cardiac magnetic resonance imaging was performed.

Results  Median survival was 7.8 years in IPAH and 3 years in SSc-PAH (p<0.001). SSc-PAH patients were older with less severe haemodynamics but lower gas transfer (DLCO). Independent prognostic factors were age, SSc, DLCO, pulmonary artery saturation and stroke volume. After excluding patients with normal or only mildly elevated resistance there was no difference in the relationship between pulmonary vascular resistance and compliance in IPAH and SSc-PAH. The relationship between mean pulmonary arterial pressure (mPAP) and systolic pulmonary arterial pressure (sPAP) in IPAH was identical to that previously reported (mPAP = 0.61sPAP + 2mmHg). The relationship in SSc-PAH was similar: mPAP = 0.58sPAP + 2mmHg (p-value for difference with IPAH=0.095). The correlation between ventricular mass index assessed at cardiac magnetic resonance imaging and pulmonary vascular resistance was stronger in SSc-PAH.

Conclusion  The reasons for poorer outcomes in SSc-PAH are likely to be multifactorial including, but not limited to, older age and reduced gas transfer.


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