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Updated: Aug 20, 2026

Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Comparison of ventricular long-axis function in patients with cardiac amyloidosis versus idiopathic restrictive
Enrica Perugini1, Claudio Rapezzi, Letizia Bacchi Reggiani
1The Royal Brompton Hospital, London, United Kingdom. e_perugini@hotmail.com <e_perugini@hotmail.com>
Insights
Cardiac amyloidosis (CA) significantly impairs ventricular long-axis function, unlike idiopathic restrictive cardiomyopathy (IRC). Longitudinal function is a sensitive marker for early systolic dysfunction in these conditions.
Area of Science:
- Cardiology
- Cardiovascular Physiology
Background:
- Cardiac amyloidosis (CA) and idiopathic restrictive cardiomyopathy (IRC) are distinct conditions affecting heart function.
- Understanding their differential impact on ventricular mechanics is crucial for diagnosis and management.
Purpose of the Study:
- To compare ventricular long-axis function in patients with CA and IRC.
- To assess the utility of ventricular long-axis function as an early marker of systolic dysfunction.
Main Methods:
- Studied 16 patients with CA and 14 patients with IRC.
- Assessed left ventricular (LV) long-axis function, fractional shortening, and LV filling.
Main Results:
- All patients with CA exhibited depressed LV long-axis function.
- Only 36% of IRC patients showed impaired longitudinal function.
- Longitudinal function impairment was detected even with normal fractional shortening and LV filling.
Conclusions:
- Ventricular long-axis function is a sensitive indicator of early systolic dysfunction in CA and IRC.
- CA and IRC possess distinct pathophysiologic profiles, questioning their classification as subtypes of a single entity.
Abstract:
To investigate ventricular long-axis function in cardiac amyloidosis (CA) and idiopathic restrictive cardiomyopathy (IRC), 16 patients with CA and 14 with IRC were studied. Left ventricular (LV) long-axis function was depressed in all patients with CA compared with only 36% of patients with IRC. Impairment in longitudinal function was clearly evident, even if fractional shortening and LV filling were normal. Ventricular long-axis function may be used as a sensitive marker of early systolic dysfunction. CA and IRC have quite distinct pathophysiologic profiles, raising some concerns about the appropriateness of considering them as 2 subtypes of a single nosographic entity.
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