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Three-Dimensional Echocardiographic Assessment of Right Ventricular Global Myocardial Work and Ventricular–Pulmonary Coupling in ATTR Cardiac Amyloidosis

Venkateshvaran, Ashwin LU orcid ; Edbom, Fredrik ; Arvidsson, Sandra ; Kovacs, Attila and Lindqvist, Per (2025) In Journal of Clinical Medicine 14(3).
Abstract

Background: Right ventricular (RV) function is inadequately investigated and routinely overlooked in transthyretin amyloid cardiomyopathy (ATTR-CM). Novel imaging distinguishers between intrinsic RV myocardial disease in ATTR-CM and primary RV overload disorder phenotypes may enhance mechanistic and pathophysiological understanding of RV dysfunction. We aimed to investigate RV performance in ATTR-CM employing comprehensive 2D and 3D echocardiography, and to compare these indices with primary RV afterload disease. Methods: We investigated conventional and novel indices of RV contractile function, myocardial work and ventricular–vascular coupling in 21 well-characterized ATTR-CM patients, 10 PAH patients and 12 healthy controls. RV long... (More)

Background: Right ventricular (RV) function is inadequately investigated and routinely overlooked in transthyretin amyloid cardiomyopathy (ATTR-CM). Novel imaging distinguishers between intrinsic RV myocardial disease in ATTR-CM and primary RV overload disorder phenotypes may enhance mechanistic and pathophysiological understanding of RV dysfunction. We aimed to investigate RV performance in ATTR-CM employing comprehensive 2D and 3D echocardiography, and to compare these indices with primary RV afterload disease. Methods: We investigated conventional and novel indices of RV contractile function, myocardial work and ventricular–vascular coupling in 21 well-characterized ATTR-CM patients, 10 PAH patients and 12 healthy controls. RV long axis function and pulmonary artery (PA) systolic pressure were evaluated using 2D Doppler echocardiography. RV ejection fraction (RVEF), volumes, global longitudinal strain (GLS) and novel myocardial work indices were analyzed by 3D echocardiography. RV elastance (Ees), afterload (Ea) and RV-PA coupling (Ees/Ea) were estimated using the single-beat volume method. Results: ATTR-CM showed lower RVEF, GLS and Ees, and a higher RV global myocardial work index (GWI), constructive work (GCW), Ea and reduced RV-PA coupling compared with controls. RV EF, stroke volume, GLS and circumferential strain did not differ between ATTR-CM and PAH. However, GWI, GCW, Ees and Ea were lower in ATTR-CM. RV–pulmonary coupling displayed strong association with RV 3D strain (r = 0.84, p < 0.001), whereas RV Ees (contractility) was related to RV GWI (r = 0.54, p < 0.001). Conclusions: ATTR-CM displayed lower RV performance, higher GMW and reduced RV-PA coupling. Myocardial work indices Ees and Ea are novel distinguishers of RV dysfunction phenotypes. The clinical and prognostic value of these novel variables warrant further investigation.

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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
cardiac amyloidosis, echocardiography, right ventricular function, ventricular–arterial coupling
in
Journal of Clinical Medicine
volume
14
issue
3
article number
668
publisher
MDPI AG
external identifiers
  • pmid:39941339
  • scopus:85217662151
ISSN
2077-0383
DOI
10.3390/jcm14030668
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2025 by the authors.
id
4209a6bc-3696-4470-8df4-e6f7959fc8d9
date added to LUP
2025-06-23 13:40:40
date last changed
2025-07-07 14:13:10
@article{4209a6bc-3696-4470-8df4-e6f7959fc8d9,
  abstract     = {{<p>Background: Right ventricular (RV) function is inadequately investigated and routinely overlooked in transthyretin amyloid cardiomyopathy (ATTR-CM). Novel imaging distinguishers between intrinsic RV myocardial disease in ATTR-CM and primary RV overload disorder phenotypes may enhance mechanistic and pathophysiological understanding of RV dysfunction. We aimed to investigate RV performance in ATTR-CM employing comprehensive 2D and 3D echocardiography, and to compare these indices with primary RV afterload disease. Methods: We investigated conventional and novel indices of RV contractile function, myocardial work and ventricular–vascular coupling in 21 well-characterized ATTR-CM patients, 10 PAH patients and 12 healthy controls. RV long axis function and pulmonary artery (PA) systolic pressure were evaluated using 2D Doppler echocardiography. RV ejection fraction (RVEF), volumes, global longitudinal strain (GLS) and novel myocardial work indices were analyzed by 3D echocardiography. RV elastance (E<sub>es</sub>), afterload (E<sub>a</sub>) and RV-PA coupling (E<sub>es</sub>/E<sub>a</sub>) were estimated using the single-beat volume method. Results: ATTR-CM showed lower RVEF, GLS and E<sub>es</sub>, and a higher RV global myocardial work index (GWI), constructive work (GCW), E<sub>a</sub> and reduced RV-PA coupling compared with controls. RV EF, stroke volume, GLS and circumferential strain did not differ between ATTR-CM and PAH. However, GWI, GCW, E<sub>es</sub> and E<sub>a</sub> were lower in ATTR-CM. RV–pulmonary coupling displayed strong association with RV 3D strain (r = 0.84, p &lt; 0.001), whereas RV E<sub>es</sub> (contractility) was related to RV GWI (r = 0.54, p &lt; 0.001). Conclusions: ATTR-CM displayed lower RV performance, higher GMW and reduced RV-PA coupling. Myocardial work indices E<sub>es</sub> and E<sub>a</sub> are novel distinguishers of RV dysfunction phenotypes. The clinical and prognostic value of these novel variables warrant further investigation.</p>}},
  author       = {{Venkateshvaran, Ashwin and Edbom, Fredrik and Arvidsson, Sandra and Kovacs, Attila and Lindqvist, Per}},
  issn         = {{2077-0383}},
  keywords     = {{cardiac amyloidosis; echocardiography; right ventricular function; ventricular–arterial coupling}},
  language     = {{eng}},
  number       = {{3}},
  publisher    = {{MDPI AG}},
  series       = {{Journal of Clinical Medicine}},
  title        = {{Three-Dimensional Echocardiographic Assessment of Right Ventricular Global Myocardial Work and Ventricular–Pulmonary Coupling in ATTR Cardiac Amyloidosis}},
  url          = {{http://dx.doi.org/10.3390/jcm14030668}},
  doi          = {{10.3390/jcm14030668}},
  volume       = {{14}},
  year         = {{2025}},
}