• Anna Bredfelt, Göran Rådegran, Roger Hesselstrand, Håkan Arheden, and Ellen Ostenfeld.
• Department of Clinical Sciences Lund, Clinical Physiology, Lund University, Lund, Sweden.
• ESC Heart Fail. 2018 Oct 1; 5 (5): 864-875.

AimsPre-capillary pulmonary hypertension (PHpre-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 PHpre-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 PHpre-cap , (ii) atrial volumes differ among four unmatched major PHpre-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 ResultsSeventy-five PHpre-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 (RAVmax and LAVmax ) and minimum volume (RAVmin and LAVmin ) 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 RAVmax 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 LAVmax and normal (HR 2.0, 95% CI 0.8-5.1). RAVmax and RAVmin showed no differences among unmatched or matched groups (P = ns). When matched for PVR, LAVmax , LAVmin , 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).ConclusionsPatients with PHpre-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.© 2018 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.

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