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Aims  Pre‐capillary pulmonary hypertension (PH pre‐cap ) has a poor prognosis, especially when caused by pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc‐PAH). Whether cardiac magnetic resonance (CMR)‐based quantification of atrial volumes in PH pre‐cap  is beneficial in risk assessment is unknown. The aims were to investigate if (i) atrial volumes using CMR are associated with death or lung transplantation in PH pre‐cap , (ii) atrial volumes differ among four unmatched major PH pre‐cap  subgroups, and (iii) atrial volumes differ between SSc‐PAH and idiopathic/familial PAH (IPAH/FPAH) when matched for pulmonary vascular resistance (PVR).    Methods and results  Seventy‐five PH pre‐cap  patients (57 ± 19 years, 53 female, 43 de novo) with CMR and right heart catheterization were retrospectively included. Short‐axis stacks of cine images were analysed, and right and left atrial maximum (RAV max  and LAV max ) and minimum volume (RAV min  and LAV min ) were indexed for body surface area. Increased (mean + 2 SD) and reduced (mean – 2 SD) volumes were predefined from CMR normal values.  Transplantation‐free survival was lower in patients with increased RAV max  than in those with normal [hazard ratio (HR) = 2.1, 95% confidence interval (CI) 1.1–4.0] but did not differ between those with reduced LAV max  and normal (HR 2.0, 95% CI 0.8–5.1). RAV max  and RAV min  showed no differences among unmatched or matched groups ( P  = ns). When matched for PVR, LAV max , LAV min , and pulmonary artery wedge pressure were reduced in SSc‐PAH compared with IPAH/FPAH (95% CI 0.3–21.4, 95% CI 0.8–19.6, and 95% CI 2–7, respectively).    Conclusions  Patients with PH pre‐cap  and increased right atrial volume measured with CMR had worse clinical outcome. When matched for PVR, left atrial volume was lower in SSc‐PAH than in IPAH/FPAH, consistent with left‐sided underfilling, indicating a potential differentiator between the groups.

esc heart failure

Increased right atrial volume measured with cardiac magnetic resonance is associated with worse clinical outcome in patients with pre‐capillary pulmonary hypertension