3D echocardiography may be useful for assessing heart damage in Fabry

Imaging can help check common Fabry complication of heart disease

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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An imaging technology called 3D echocardiography, which allows clinicians to visualize the heart in three dimensions and measure the strain on heart tissue when the heart beats, may be useful for assessing heart damage among people with Fabry disease.

That’s according to the study, “Three-dimensional echocardiographic left ventricular strain analysis in Fabry disease: correlation with heart failure severity, myocardial scar, and impact on long-term prognosis,” which was published in the European Heart Journal Cardiovascular Imaging.

Heart disease is a common complication of Fabry disease. Usually, the left ventricle (LV) — the part of the heart responsible for pumping oxygen-rich blood out to the body — is most affected.

In this study, scientists in Czechia used an imaging technique called 3D echocardiography to analyze the hearts of Fabry patients. As its name implies, 3D echocardiography allows clinicians to visualize the heart in three dimensions.

“While 2D analysis of myocardial [heart tissue] deformation and its clinical impact on [Fabry disease] have been evaluated before, no data on echocardiographic 3D LV deformation have been published previously,” the scientists wrote. “Accordingly, our aim was to assess the feasibility of 3D echocardiographic LV strain, its relation to heart failure severity, and its impact on the long-term prognosis of patients with” Fabry disease.

The analysis included 3D echocardiography imaging from 75 people with Fabry disease. Just over half of the patients were women and the average age was in the late 40s.

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Researchers compare strain measurements with NTproBNP levels

From the 3D visualizations of patients’ hearts, the researchers derived measurements reflecting the mechanical strain placed on the heart as it beats to pump blood. The team compared how these measurements compared against levels of N-terminal pro-BNP (NTproBNP), a well-established marker of heart damage.

Results showed statistically significant correlations between NTproBNP levels and measures of LV global area strain, global longitudinal strain, and radial strain. In other words, patients with higher measures of strain (as assessed by 3D echocardiography) also tended to have higher levels of this heart damage marker.

Analyses of patients who also had heart MRI data available showed significant associations between measures of heart strain and the presence of scar tissue in the heart.

Over a median follow-up of about three years, three of the patients died, seven experienced worsening heart failure, and 22 were hospitalized due to heart-related complaints. Statistical analyses showed that several echocardiography-based measures of heart strain — particularly global longitudinal strain, which reflects how much the left ventricle needs to compress itself each time the heart pumps — were significantly associated with an increased risk of these poor outcomes.

The team noted that these analyses were limited by the small number of patients and clinical events, noting a need to verify the results in further larger studies. They also acknowledged that, while 3D echocardiography might be a valuable tool, it can be technically challenging to use, and they were not able to get usable images in about a quarter of patients who underwent evaluation.

“3D echocardiography might be used for a comprehensive mechanical assessment of LV in patients with [Fabry disease], but technological advancements in image acquisition and analysis are needed,” the team concluded.