P2727Hemodynamic profiles in patients with cardiac amyloidosis
Authors: Duca F , Snidat A , Aschauer S , Rettl R , Binder C , Agis H , Kain R , Hengstenberg C , Mascherbauer J , Bonderman D 
Published: October 2019

Abstract

Background
Intracardiac filling and pulmonary arterial pressures play a central role in various heart failure entities, as they are strong predictors of outcome. However, their role in patients with cardiac amyloidosis (CA) is less clear.

Purpose
We aimed to characterize hemodynamic profiles of CA patients and assess their association with outcomes.

Methods
The present study was conducted within a prospective, national CA registry.
Patients underwent invasive hemodynamic, clinical, laboratory, and echocardiography assessment, as well cardiac magnetic resonance imaging with T1-mapping. The main outcome measure was a combined endpoint consisting of hospitalization for heart failure or cardiovascular death.

Results
Between March 2012 and October 2018, 63 consecutively recruited CA patients underwent invasive hemodynamic profiling. Of those, 36 had cardiac transthyretin amyloidosis (ATTR) and 25 cardiac light-chain amyloidosis (AL). In two patients amyloid subtyping was not possible.
Median age of the study population was 74.0 years and the majority were male (61.9%). Almost half of the patients were in New York Heart Association (NYHA) class ≥III (47.6%) and showed elevated N-terminal prohormone of brain natriuretic peptides (NT-proBNP) with a median of 3222 pg/mL. In comparison to AL, cardiac TTR patients were older (75.0 years versus 69.0 years, p=0.004), more often male (80.6% versus 40.0%, p=0.001), less symptomatic (NYHA class ≥III: 38.9% versus 64.0%, p=0.021), and had lower NT-proBNP values (2324pg/mL versus 5151pg/mL, p=0.004).
Hemodynamic profiling revealed significantly increased intracardiac as well as pulmonary arterial pressures (PAP). On an average, pulmonary artery wedge pressure was 20.0mmHg [interquartile range (IQR): 17.0–25.0], mean PAP (mPAP) was 30.0mmHg (IQR: 25.0–37.0), and mean right atrial pressure (mRAP) was 11.0mmHg (IQR: 7.0–16.0). No differences between ATTR and AL patients could be detected (p=0.148, p=0.398, p=0.620). During a median follow-up of 63.0 weeks, 28 study participants (44.4%) reached the combined endpoint. Moreover, cardiac AL patients had significantly more events as their ATTR counterparts (72.0% versus 27.8%, p=0.001). In cardiac ATTR patients, mPAP was significantly associated with outcome [hazard ratio (HR): 1.083, p=0.034, Figure 1A], which was not the case in the AL group (HR: 1.024, p=0.186, Figure 1B). Cardiac output and pulmonary vascular resistance were not associated with outcome. Neither in the ATTR (p=0.144; p=0.063) nor in AL cohort (p=0.420; p=0.115).
Figure 1

Conclusion
Despite differences in the severity of symptoms between cardiac AL and cardiac ATTR patients, no differences with regards to hemodynamic profiles could be detected.
Furthermore, intracardiac filling and pulmonary arterial pressures seem to be of greater clinical importance in cardiac ATTR as compared to cardiac AL, as these parameters were associated with outcome in the first, but not the latter group.