Prevalence and haemodynamic profiles of pulmonary hypertension in cardiac amyloidosis

Figure 1. Study schematic. Figure depicting the schematic for inclusion and exclusion of patients. Patients with cardiac amyloidosis (CA) who had undergone cardiovascular magnetic resonance (CMR) and right heart catheterisation (RHC) within 1 year were included. Of the 94 patients who underwent CMR, 52 were ultimately included in the analysis.

Figure 2. Correlation between right ventricular function and amyloid burden. This figure depicts the relationship between amyloid burden as assessed with extracellular volume (ECV) and cardiovascular magnetic resonance (CMR)-derived measurements of right ventricular function. (A) right ventricular ejection fraction, peak right ventricular (B) radial, (C) circumferential and (D) longitudinal strain. Correlation was determined using Pearson’s (r) or Spearman’s rank (rs) correlation coefficients.

Figure 3. Major adverse events across haemodynamic PH profiles. This figure depicts Kaplan-Meier curves demonstrating time to (A) major adverse cardiovascular events—defined as death or heart failure hospitalisation—and (B) death among the four haemodynamic PH profiles. There were no significant differences in outcomes between groups using log-rank sum test (p values denoted in upper right hand corner). IPC, isolated postcapillary; PH, pulmonary hypertension.

Figure 4. Importance of haemodynamic and cardiac imaging predictors of MACE: Importance of RHC and CMR imaging parameters in predicting the combined endpoint of death or heart failure hospitalisation. The relative importance was determined using Random Forest algorithm and is depicted in descending order of importance. Central Illustration: summary of key findings from our analysis. (A) PH is highly prevalent in cardiac amyloidosis (CA); while isolated post-capillary PH (IPC-PH) is most common, a substantial proportion of patients have combined post and pre-capillary PH (CPCPH). (B) Right ventricular dysfunction, as defined using RV ejection fraction and strain, correlates with amyloid burden as assessed by extracellular volume (ECV). (C) In Random Forest modelling, TPG, LVEF and RVEF were the most important variables in predicting major adverse cardiovascular events (MACE). CMR, cardiac magnetic resonance; DPG, diastolic pulmonary gradient; ECV, extracellular vol; EF, ejection fraction; LV, left ventricle; PA, pulmonary artery; PVR, pulmonary vascular resistance; RHC, right heart catheterisation; RV, right ventricle; TPG, transpulmonary gradient.

Figure 5. Summary of key findings from our analysis. (A) PH is highly prevalent in cardiac amyloidosis (CA); while isolated post-capillary PH (IPC-PH) is most common, a substantial proportion of patients have combined post and pre-capillary PH (CPCPH). (B) Right ventricular dysfunction, as defined using RV ejection fraction and strain, correlates with amyloid burden as assessed by extracellular volume (ECV). (C) In Random Forest modelling, TPG, LVEF and RVEF were the most important variables in predicting major adverse cardiovascular events (MACE). DPG, diastolic pulmonary gradient; ECV, extracellular vol; EF, ejection fraction; LV, left ventricle; PA, pulmonary artery; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RV, right ventricle; TPG, transpulmonary gradient.