Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Comparison of Demographic, Hemodynamic, and MRI Characteristics and Outcomes

Sheila Ramjug; Nehal Hussain; Judith Hurdman; Catherine Billings; Athanasios Charalampopoulos; Charlie A Elliot; David G Kiely; Ian Sabroe; Smitha Rajaram; Andrew J Swift; Robin Condliffe

doi:10.1016/j.chest.2017.02.010

Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Comparison of Demographic, Hemodynamic, and MRI Characteristics and Outcomes

Chest. 2017 Jul;152(1):92-102. doi: 10.1016/j.chest.2017.02.010. Epub 2017 Feb 20.

Affiliations

¹ Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, England.
² Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, England; Department of Infection and Immunity, University of Sheffield, Sheffield, England.
³ Department of Radiology, Sheffield Teaching Hospitals NHS Trust, Sheffield, England.
⁴ Academic Department of Radiology, University of Sheffield, Sheffield, England; Department of Radiology, Sheffield Teaching Hospitals NHS Trust, Sheffield, England.
⁵ Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, England. Electronic address: robin.condliffe@sth.nhs.uk.

PMID: 28223154
DOI: 10.1016/j.chest.2017.02.010

Abstract

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

Methods: Six hundred fifty-one patients diagnosed with IPAH or SSc-associated precapillary pulmonary hypertension were included. Patients with pulmonary disease or two or more risk factors for left heart disease were identified, leaving a primary analysis set of 375 subjects. Subgroup analysis using cardiac magnetic resonance (CMR) imaging was performed.

Results: Median survival was 7.8 years in IPAH and 3 years in SSc-PAH (P < .001). Patients with SSc-PAH were older with less severe hemodynamics but lower gas transfer (diffusing capacity for carbon monoxide [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 (PVR) 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 = 0.61 sPAP + 2 mm Hg). The relationship in SSc-PAH was similar: mPAP = 0.58 sPAP + 2 mm Hg (P value for difference with IPAH = 0.095). The correlation between ventricular mass index assessed at CMR imaging and PVR was stronger in SSc-PAH.

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

Keywords: connective tissue disease; idiopathic; pulmonary hypertension; systemic sclerosis.

Publication types

Comparative Study

MeSH terms

Adult
Age Factors
Aged
Demography
England / epidemiology
Familial Primary Pulmonary Hypertension* / diagnosis
Familial Primary Pulmonary Hypertension* / epidemiology
Familial Primary Pulmonary Hypertension* / physiopathology
Female
Hemodynamics
Humans
Hypertension, Pulmonary* / diagnosis
Hypertension, Pulmonary* / epidemiology
Hypertension, Pulmonary* / etiology
Hypertension, Pulmonary* / physiopathology
Magnetic Resonance Imaging / methods
Male
Middle Aged
Outcome Assessment, Health Care
Respiratory Function Tests / methods
Scleroderma, Systemic / complications*
Severity of Illness Index
Survival Analysis
Vascular Resistance