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Updated: Feb 14, 2026
Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
Published on: October 28, 2020
Comparison of left atrial size and function in hypertrophic cardiomyopathy and in Fabry disease with left ventricular
María Cristina Saccheri1, Tomás Francisco Cianciulli1,2, Wilde Challapa Licidio1
1Echocardiography Laboratory, Division of Cardiology, Hospital General de Agudos del Gobierno de la Ciudad de Buenos Aires "Dr. Cosme Argerich, Buenos Aires, Argentina.
Insights
Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) both cause left ventricular hypertrophy, but FD is associated with a smaller left atrial volume. Both conditions severely impair left atrial function, indicating atrial cardiomyopathy.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) are distinct conditions causing left ventricular hypertrophy and fibrosis.
- While left atrial (LA) remodeling in HCM is linked to atrial fibrillation, LA changes in FD remain understudied.
Purpose of the Study:
- To compare left atrial size and mechanical function in patients with non-obstructive HCM and Fabry cardiomyopathy.
- To investigate the presence of atrial cardiomyopathy in FD using speckle tracking echocardiography.
Main Methods:
- Prospective study comparing 19 HCM patients, 20 FD patients, and 20 controls.
- Echocardiography used to measure left ventricular mass, LA volume, and LA mechanical function (strain and strain rate).
- Speckle tracking assessed reservoir, conduit, and contraction phases of LA function.
Main Results:
- Patients with HCM had significantly larger LA volumes than those with FD (48.16 vs. 38.9 mL/m²).
- Both HCM and FD groups exhibited severe reductions in LA reservoir strain and strain rate during all three functional phases.
- No significant differences in LA function parameters were observed between the HCM and FD groups.
Conclusions:
- Fabry disease is associated with atrial cardiomyopathy, impacting all three phases of LA mechanical function.
- Despite larger LA volumes in HCM, both conditions demonstrate similarly impaired LA function.
- These findings highlight the significant atrial involvement in FD and HCM, with potential clinical implications.
Background:
Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) are two diseases with a different pathophysiology, both cause left ventricular hypertrophy (LVH) and myocardial fibrosis. Although remodeling and systolic dysfunction of the left atrium (LA) are associated with atrial fibrillation and stroke in HCM, changes in the size and function of the LA have not been well studied in FD with LVH.
Methods:
The following groups were studied prospectively, and their respective findings compared: 19 patients with non-obstructive HCM (Group I), 20 patients with a diagnosis of Fabry cardiomyopathy (Group II), and 20 normal subjects matched for sex and age (Group III). Left ventricular mass index was measured using Devereux' formula, left atrial volume with Simpson's biplane method and left atrial mechanical function, including strain and strain rate, was measured using the speckle tracking technique. Strain and strain rate of the reservoir were measured during the three phases: reservoir (SR S), passive conduit (SR E) and atrial contraction (SR A).
Results:
Patients with HCM had a larger left atrial volume than patients with FD (48.16 ± 14.3 mL/m2 vs 38.9 ± 14.9 mL/m2 respectively, P < .001), but in both disorders there was a severe decrease in left atrial function: reservoir strain in the apical four-chamber view: 17.47% in HCM vs 22.5% in FD, P = .24), strain rate in the apical chamber view: SR A: -0.80/seconds in HCM vs -1.04/seconds in FD (P = .88), SR S: 0.69/seconds in HCM vs 0.93 in FD (P = .12), SR E: -0.80 seconds in HCM vs -0.97/seconds in FD (P = .18).
Conclusions:
In this echocardiographic study we used speckle tracking to assess left atrial mechanical function and showed that FD is associated to an atrial cardiomyopathy, affecting the three phasic functions of the LA. Although in patients with HCM left atrial volume is larger than in patients with FD, both disorders exhibit severe decrease in left atrial function. These findings should be considered, given the potentially serious complications that can occur with the two diseases.
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