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Related Experiment Video

Updated: May 31, 2026

In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease

In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease

Published on: December 20, 2017

Tei index in fabry disease.

Markus Niemann¹, Frank Breunig, Meinrad Beer

¹Department of Internal Medicine, University of Würzburg, Würzburg, Germany.

Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography

|July 2, 2011

Summary

The Tei index detects left ventricular (LV) functional reduction in Fabry disease patients, reflecting cardiomyopathy progression. However, it has limited value for detecting fibrosis or monitoring enzyme replacement therapy.

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Area of Science:

Cardiology
Genetics
Biomarkers

Background:

Fabry disease (FD) is a genetic disorder causing left ventricular (LV) systolic and diastolic dysfunction.
Cardiac involvement in FD necessitates reliable markers for early detection and monitoring.

Purpose of the Study:

To evaluate the diagnostic utility of the Tei index, a measure of global cardiac function, in patients with Fabry disease.
To assess the Tei index's ability to detect cardiac involvement, including hypertrophy and fibrosis, and monitor disease progression.

Main Methods:

Sixty-six genetically confirmed Fabry disease patients underwent echocardiography (including Tei index) and cardiac MRI.
Patients were categorized into three groups based on cardiac involvement: none, LV hypertrophy without fibrosis, and LV hypertrophy with fibrosis.
Follow-up included assessment of enzyme replacement therapy (ERT) and natural history.

Main Results:

The Tei index was significantly elevated in patients with LV hypertrophy and/or fibrosis compared to those without cardiac involvement (P < .001).
Ejection fraction remained normal across all groups, rendering it ineffective for detecting cardiac involvement.
A positive correlation existed between LV wall thickness and Tei index; ROC analysis showed high accuracy for Tei index in detecting hypertrophy but low accuracy for fibrosis.

Conclusions:

The Tei index effectively detects global LV functional impairment and LV hypertrophy progression in Fabry disease, outperforming ejection fraction.
The Tei index has limited value in detecting myocardial fibrosis or monitoring the efficacy of enzyme replacement therapy.
The Tei index serves as a valuable global functional parameter for assessing cardiac involvement in Fabry disease.