Background-There are limited data on the prognostic value of cardiovascular magnetic resonance measurements in idiopathic pulmonary arterial hypertension, with no studies investigating the impact of correction of cardiovascular magnetic resonance indices for age and sex on prognostic value. Methods and Results-Consecutive patients with idiopathic pulmonary arterial hypertension underwent cardiovascular magnetic resonance imaging at 1.5T. Steady-state free precession cardiac volumes and mass measurements were corrected for age, sex, and body surface area according to reference data and prognostic significance assessed. A total of 80 patients with idiopathic pulmonary arterial hypertension were identified, and 23 patients died during the mean follow-up of 32±14 months. Corrected for age, sex, and body surface area, right ventricular end-systolic volume (P=0.004) strongly predicted mortality, independent of World Health Organization functional class, mean right atrial pressure, cardiac index, and mixed venous oxygen saturations. Conclusions-Consideration should be given to correcting cardiovascular magnetic resonance measures for age, sex, and body surface area, particularly given the changing demographics of patients with idiopathic pulmonary arterial hypertension. Corrected right ventricular end-systolic volume is a strong prognostic marker in idiopathic pulmonary arterial hypertension, independent of invasively derived measurements, mean right atrial pressure cardiac index, and mixed venous oxygen saturations. Correspondence to Andrew Swift, PhD, University of Sheffield, Academic Unit of Radiology, C Floor, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2J, UK. E-mail a.j.swift@shef.ac.uk
Methods
PatientsConsecutive treatment-naïve patients with IPAH who underwent MRI and right heart catheterization (RHC), within 48 hours, were identified from a database of a large volume, nationally designated, pulmonary hypertension referral center from January 1, 2008, to November 2011. A census was performed on December 17, 2012, providing a minimum of 1 year follow-up from scan date. Patients referred with suspected pulmonary hypertension underwent systematic evaluation as previously described in the Assessing the Spectrum of Pulmonary Hypertension Identified At a Referral Centre (ASPIRE) registry, 17 including lung function, exercise testing, high resolution computed tomography and computed tomographic pulmonary angiography, CMR and MR angiography, and RHC. This study was approved by our institutional review committee, and informed consent was waived for this retrospective study.
CMR Image AcquisitionCMR imaging was performed using an 8-channel cardiac coil on a GE HDx (GE Healthcare, Milwaukee, WI) whole body scanner at 1.5T. Short-axis cine images were acquired using a cardiac gated multislice balanced steady-state free precession sequence (20 frames per cardiac cycle; field of view, 48; matrix, 256×256; bandwidth, 125 KHz/pixel; repetition time/echo time, 3.7/1.6 ms). A stack of images in the short-axis plane ...