Cross‐sectional baseline analysis of electrocardiography in a large cohort of patients with untreated Fabry disease. Issue 5 (11th October 2012)

• Record Type: Journal Article
• Authors: Niemann, Markus; Hartmann, Tanja; Namdar, Mehdi; Breunig, Frank; Beer, Meinrad; Machann, Wolfram; Herrmann, Sebastian; Ertl, Georg; Wanner, Christoph; Weidemann, Frank
• Is Part Of: Journal of inherited metabolic disease. Volume 36:Issue 5(2013)
• Page Start: 873
• Page End: 879
• Publication Date: 2012-10-11
• Subjects: Metabolism, Inborn errors of -- Periodicals; Metabolism -- Disorders -- Periodicals; 616.39042
• DOI: 10.1007/s10545-012-9540-8
• Languages: English
• ISSNs: 0141-8955

Abstract

Abstract: Background: Morphology and function of Fabry cardiomyopathy has been previously studied by echocardiography and cardiac magnetic resonance (CMR). However, the value of electrocardiography (ECG) in relation to these two techniques remains largely unknown. Methods: One hundred fifty genetically confirmed Fabry patients were investigated using a comprehensive clinical workup comprising 12‐lead ECG, echocardiography, and CMR. Results: ECG parameters at rest [PR, P wave, QT, QTc, QT dispersion and time interval from the peak to the end of the T wave (Tpeak to Tend )] were normal in the entire cohort and did not distinguish between males and females or stages of cardiomyopathy. A significant positive correlation was found between left ventricular (LV) mass on CMR and both the QRS duration and the LV Sokolow index, with the highest values in male patients with an advanced cardiomyopathy stage. No prediction of late enhancement on CMR (a sign for replacement fibrosis) was possible by a single ECG parameter. However, the absence of ST or T alterations (in 37 of 38 patients) specifically excluded late enhancement on CMR. Conclusion: Our data in a large cohort of Fabry patients, including all cardiomyopathy stages, show, in contrast to former assumptions, that ECG parameters are not suitable to stage Fabry cardiomoypathy. Most ECG parameters were normal in the complete cohort. However, the absence of ST or T alterations seems to almost exclude late enhancement on CMR in these patients.