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Updated: Jul 16, 2026
Assessment of Right Ventricular Structure and Function in Mouse Model of Pulmonary Artery Constriction by Transthoracic Echocardiography
Published on: February 3, 2014
Three-Dimensional Echocardiographic Assessment of Right Ventricular Global Myocardial Work and Ventricular-Pulmonary
Ashwin Venkateshvaran1, Fredrik Edbom2, Sandra Arvidsson2
1Clinical Physiology, Department of Clinical Sciences Lund, Lund University, Skåne University Hospital, 22185 Lund, Sweden.
Insights
Transthyretin amyloid cardiomyopathy (ATTR-CM) significantly impairs right ventricular (RV) function, showing reduced performance and altered myocardial work. Novel echocardiographic indices reveal distinct RV dysfunction phenotypes in ATTR-CM compared to pulmonary artery hypertension.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Cardiac Physiology
Background:
- Right ventricular (RV) function is often overlooked in transthyretin amyloid cardiomyopathy (ATTR-CM).
- Distinguishing intrinsic RV myocardial disease from RV overload is crucial for understanding ATTR-CM pathophysiology.
- Novel imaging techniques may elucidate RV dysfunction in ATTR-CM.
Purpose of the Study:
- To investigate RV performance in ATTR-CM using comprehensive 2D and 3D echocardiography.
- To compare RV indices in ATTR-CM with those in pulmonary artery hypertension (PAH) and healthy controls.
- To explore novel echocardiographic measures of RV myocardial work and ventricular-vascular coupling.
Main Methods:
- 21 ATTR-CM patients, 10 PAH patients, and 12 healthy controls were studied.
- Conventional and novel 2D/3D echocardiographic indices of RV function, myocardial work, and ventricular-vascular coupling were assessed.
- RV elastance (Ees), afterload (Ea), and RV-pulmonary artery (PA) coupling (Ees/Ea) were estimated using the single-beat method.
Main Results:
- ATTR-CM patients exhibited lower RV ejection fraction (RVEF), global longitudinal strain (GLS), and RV elastance (Ees) compared to controls.
- ATTR-CM showed higher RV global myocardial work index (GWI) and constructive work (GCW), along with increased afterload (Ea) and reduced RV-PA coupling.
- While RV EF, stroke volume, and GLS did not differ between ATTR-CM and PAH, myocardial work indices and RV-PA coupling were distinct.
Conclusions:
- ATTR-CM is characterized by diminished RV performance, elevated myocardial work, and impaired RV-PA coupling.
- Novel myocardial work indices (Ees, Ea) serve as important differentiators of RV dysfunction phenotypes.
- Further research is needed to establish the clinical and prognostic significance of these novel echocardiographic variables in ATTR-CM.
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 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.
