Right ventricular remodelling in pulmonary arterial hypertension predicts treatment response

Heart. 2022 Aug 11;108(17):1392-1400. doi: 10.1136/heartjnl-2021-320733.

Abstract

Objectives: To determine the prognostic value of patterns of right ventricular adaptation in patients with pulmonary arterial hypertension (PAH), assessed using cardiac magnetic resonance (CMR) imaging at baseline and follow-up.

Methods: Patients attending the Sheffield Pulmonary Vascular Disease Unit with suspected pulmonary hypertension were recruited into the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral Centre) Registry. With exclusion of congenital heart disease, consecutive patients with PAH were followed up until the date of census or death. Right ventricular end-systolic volume index adjusted for age and sex and ventricular mass index were used to categorise patients into four different volume/mass groups: low-volume-low-mass, low-volume-high-mass, high-volume-low-mass and high-volume-high-mass. The prognostic value of the groups was assessed with one-way analysis of variance and Kaplan-Meier plots. Transition of the groups was studied.

Results: A total of 505 patients with PAH were identified, 239 (47.3%) of whom have died at follow-up (median 4.85 years, IQR 4.05). The mean age of the patients was 59±16 and 161 (32.7%) were male. Low-volume-low-mass was associated with CMR and right heart catheterisation metrics predictive of improved prognosis. There were 124 patients who underwent follow-up CMR (median 1.11 years, IQR 0.78). At both baseline and follow-up, the high-volume-low-mass group had worse prognosis than the low-volume-low-mass group (p<0.001). With PAH therapy, 73.5% of low-volume-low-mass patients remained in this group, whereas only 17.4% of high-volume-low-mass patients transitioned into low-volume-low-mass.

Conclusions: Right ventricular adaptation assessed using CMR has prognostic value in patients with PAH. Patients with maladaptive remodelling (high-volume-low-mass) are at high risk of treatment failure.

Keywords: Heart Failure; Magnetic Resonance Imaging; Pulmonary Arterial Hypertension.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Familial Primary Pulmonary Hypertension
  • Female
  • Humans
  • Hypertension, Pulmonary* / complications
  • Hypertension, Pulmonary* / diagnostic imaging
  • Male
  • Predictive Value of Tests
  • Prognosis
  • Pulmonary Arterial Hypertension* / diagnostic imaging
  • Stroke Volume
  • Ventricular Dysfunction, Right* / complications
  • Ventricular Dysfunction, Right* / etiology
  • Ventricular Function, Right
  • Ventricular Remodeling