Abstract
This study sought to characterize cardiac amyloidosis (CA) patients with respect to hemodynamic parameters and asses their prognostic impact in different CA cohorts. Intracardiac and pulmonary arterial pressures (PAPs) are among the strongest predictors of outcomes in patients with heart failure (HF). Despite that, the hemodynamic profiles of patients with CA and their relation to prognosis have rarely been investigated. Invasive hemodynamic, clinical, and laboratory assessment, as well as cardiac magnetic resonance imaging were performed in our CA cohort. A total of 61 patients, 35 (57.4%) with wild-type transthyretin amyloidosis (ATTRwt) and 26 (42.6%) with light-chain amyloidosis (AL) were enrolled. ATTRwt patients had lower N-terminal prohormone of brain natriuretic peptide values and were less frequently in New York Heart Association class ≥ III. Intracardiac and PAPs were elevated, but hemodynamic parameters did not differ between CA groups. Whereas in ATTRwt, the median mean PAP (hazard ratio (HR): 1.130, p = 0.040) and pulmonary vascular resistance (HR: 1.010, p = 0.046) were independent predictors of outcome, no hemodynamic parameter was associated with outcome in the AL group. Cardiac ATTRwt and AL patients feature elevated intracardiac and PAPs and show similar hemodynamic profiles. However, hemodynamic parameters are of greater prognostic relevance in ATTRwt, potentially providing a new therapeutic target.
Highlights
Once deemed an ultra-rare disease, cardiac amyloidosis (CA) is considered an important etiology in the diagnostic work-up of heart failure (HF) and an increasing number of cardiologists are confronted with the difficult task of treating these patients [1,2]
Supporting the importance of hemodynamics in HF, the recently published phase III VerICiguaT Global Study in Subjects with Heart Failure With Reduced Ejection Fraction (VICTORIA) trial, which tested vericiguat, a soluble guanylate cyclase stimulator that acts as a vasodilator in the pulmonary and systemic circulation, has met the primary endpoint i.e., the time to the first occurrence of cardiovascular death or HF hospitalization [5]
Further research is warranted to investigate hemodynamic profiles in different CA subgroups and to assess whether intracardiac filling pressures and pulmonary arterial pressures (PAPs) are associated with patient outcomes, providing potential new therapeutic targets in CA
Summary
Once deemed an ultra-rare disease, cardiac amyloidosis (CA) is considered an important etiology in the diagnostic work-up of heart failure (HF) and an increasing number of cardiologists are confronted with the difficult task of treating these patients [1,2]. In HF, irrespective of left ventricular ejection fraction (LVEF), intracardiac filling pressures and pulmonary arterial pressures (PAPs) play a central pathophysiological role and are among the strongest predictors of adverse outcomes [3,4]. Supporting the importance of hemodynamics in HF, the recently published phase III VerICiguaT Global Study in Subjects with Heart Failure With Reduced Ejection Fraction (VICTORIA) trial, which tested vericiguat, a soluble guanylate cyclase stimulator that acts as a vasodilator in the pulmonary and systemic circulation, has met the primary endpoint i.e., the time to the first occurrence of cardiovascular death or HF hospitalization [5]. Further research is warranted to investigate hemodynamic profiles in different CA subgroups and to assess whether intracardiac filling pressures and PAPs are associated with patient outcomes, providing potential new therapeutic targets in CA. We aimed to characterize CA patients with respect to invasively measured hemodynamic parameters and assess their prognostic relevance in a prospectively followed cohort consisting of wild-type transthyretin amyloidosis (ATTRwt) and light-chain amyloidosis (AL) patients
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