Electrocardiographic Evaluation in Patients With Spinal Muscular Atrophy: A Case-Control Study.

BACKGROUND

This study aimed to show the impairment of autonomic cardiac conduction causing bradycardia and/or electrocardiographic alterations in children affected by spinal muscular atrophy type 1 and 2 (SMA 1 and 2).

METHODS

We included 25 spinal muscular atrophy patients, admitted from November 2016 to May 2017. All patients underwent an electrocardiographic examination and we studied PR and QRS intervals, P-waves and QRS amplitudes, and heart rate in spinal muscular atrophy patients compared to a control group.

RESULTS

In all patients, we found longer PRi and QRSi ( P < .05), lower P-wave and QRS complex amplitudes ( P < .01), and a decreased heart rate ( P < .01) with respect to controls. When we divided our patients into SMA1 and SMA2 subgroups, we found that statistical differences were maintained for P-wave and QRS complex amplitudes and heart rate, but not for PRi and QRSi with respect to controls.

CONCLUSION

We suggest the hypothesis of SMN expression on cardiac tissue condition and/or autonomic cardiac conduction.