Clinicians in the Paris region, some of whom practice at the Institute, report on a study of a new ratio of echocardiographic parameters in patients with various forms of muscular dystrophy (primarily Becker or Duchenne muscular dystrophy and sarcoglycanopathies):
- the assessment of coupling between the pulmonary artery and the right ventricle provides a good indication of the impact of the underlying myopathy on cardiac function;
- in echocardiography, the ratio of pulmonary artery systolic pressure (sPAP) to tricuspid valve plane systolic excursion (TAPSE) is a good indicator of this coupling,
- 113 adults (including 20 with sarcoglycanopathy) participated in the study,
- 46% of whom were on mechanical ventilation and more than two-thirds were wheelchair-bound,
- a high TAPSE/sPAP ratio was found to have predictive value for the risk of cardiac involvement, with a cutoff of 5.3 mm Hg.
This non-invasive method and the calculation of this ratio may be useful for the cardiological follow-up of these potentially at-risk patients.
