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- ACE, angiotensin converting enzyme
- BMD, Becker muscular dystrophy
- LV, left ventricular
- LVEF, left ventricular ejection fraction
- SRDIA, diastolic strain rate
- SRSYS, systolic strain rate
- TDE, tissue Doppler echocardiography
Becker muscular dystrophy (BMD) is an X linked mutation of the dystrophin gene characterised by skeletal muscle dystrophy and progressive heart failure which frequently leads to a fatal outcome.1 Cardiac involvement is not accurately predicted by gene mutation and may occur in patients without muscle weakness. Consequently, systematic cardiac examinations are required. Tissue Doppler echocardiography (TDE) allows accurate quantification of regional myocardial function and may be a reliable method for early detection of myocardial dysfunction in BMD.2
Twenty four consecutive men with genetically documented BMD were prospectively investigated after informed consent and compared to 17 age matched controls. All patients underwent clinical examination, ECG, radionuclide ventriculography, and conventional echocardiography with TDE imaging.
Radionuclide ventriculography (gamma camera General Electric, Starport 400AT, interfaced with ADAC computer, DPS 3000) was performed after in vitro red cell labelling with 15 mCi of technetium-99m.
Ultrasound examinations (HDI5000, ATL ultrasound, Bothell, Washington DC) were recorded and analysed offline (HDILab Software) by two cardiologists unaware of any information. Radial left ventricular (LV) thickening was interrogated using colour M mode TDE acquisitions in the posterior wall. Myocardial wall motion velocities were extracted in endocardium (EndoV) and epicardium (EpiV) …