Abstract
Background and aims: Monitoring of electrical activity of the diaphragm (EAdi) is a minimally invasive bedside technology assessing ventilatory drive, which indirectly reflects respiratory efforts. In patients with neuromuscular disease, EAdi may help to characterize and evaluate the progression of the respiratory disease. While in normally breathing infants, peak EAdi is usually below 20 mcV, EAdi values have not been described in congenital myotonic dystrophy. Aims: To describe EAdi in infants with congenital myotonic dystrophy. Methods: Retrospective study of 3 ventilatory assist dependent infants with congenital myotonic dystrophy. EAdi was recorded using a specific nasogastric catheter (Maquet). To assess work of breathing, esophageal pressure was recorded in case 3 with an esophageal balloon. The parents gave their consent, local IRB agreement is not necessary for case-report study. Results:Table: No title available.In all cases, signs of respiratory distress were absent. In case 3, esophageal pressure-time product was 13 cmH2O.s.min-1, confirming a severely diminished work of breathing. Conclusions: Infants with congenital myotonic dystrophy appear to have particularly high EAdi. This does not reflect a high work of breathing but rather a response to low ventilatory efficiency due to muscle weakness. Neuro-ventilatory coupling should be taken into account while interpreting EAdi value.
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