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

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 (pConclusion

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