Springer Nature is making SARS-CoV-2 and COVID-19 research free. View research | View latest news | Sign up for updates

Mechanisms of ventilator dependence in children with neuromuscular and respiratory control disorders identified by monitoring diaphragm electrical activity



To report on the monitoring of diaphragm electrical activity (Edi) using neurally adjusted ventilator assist (NAVA) technology to investigate the mechanisms of ventilator dependence in children with neuromuscular and respiratory control disorders.

Patients and methods

Using NAVA technology, electrical activity of the diaphragm (Edi) was monitored at the lowest achievable level of respiratory support in six ventilator-dependent patients with neuromuscular and respiratory control disorders, aged 6 weeks to 12 years, admitted to a tertiary paediatric intensive care unit between 2009 and 2011.


Edi monitoring identified markedly abnormal respiratory dynamic patterns that were not always apparent clinically. These were associated with disorders of central respiratory control, muscle weakness and diaphragm pathology.


Edi monitoring using NAVA technology is a valuable, minimally invasive, diagnostic adjunct in children with neuromuscular and respiratory control disorders who are ventilator-dependent.

This is a preview of subscription content, log in to check access.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6


  1. 1.

    Goldstone J (2002) The pulmonary physician in critical care. 10: difficult weaning. Thorax 57:986–991

  2. 2.

    Sinderby C, Navalesi P, Beck J, Skrobik Y, Comtois N, Friberg S, Gottfried SB, Lindström L (1999) Neural control of mechanical ventilation in respiratory failure. Nat Med 5:1433–1436

  3. 3.

    Sinderby C, Saphija J, Beck J (2004) Neurally adjusted ventilatory assist. In: Slutksy AS, Brochard L (eds) Mechanical ventilation. Update in Intensive Care Medicine, Springer, pp 125–134

  4. 4.

  5. 5.

    Barwing J, Ambold M, Linden N, Quintel M, Moerer O (2009) Evaluation of the catheter positioning for neurally adjusted ventilatory assist. Intensive Care Med 35:1809–1814

  6. 6.

    Ogier M, Katz DM (2008) Breathing dysfunction in Rett syndrome: understanding epigenetic regulation of the respiratory network. Respir Physiol Neurobiol 164:55–63

  7. 7.

    Julu PO, Kerr AM, Apartopoulos F, Al-Rawas S, Engerström IW, Engerström L, Jamal GA, Hansen S (2001) Characterisation of breathing and associated central autonomic dysfunction in the Rett disorder. Arch Dis Child 85:29–37

  8. 8.

    Emeriaud G, Beck J, Tucci M, Lacroix J, Sinderby C (2006) Diaphragm electrical activity during expiration in mechanically ventilated infants. Pediatr Res 59:705–710

  9. 9.

    Forrest KM, Al-Sarraj S, Sewry C, Buk S, Tan SV, Pitt M, Durward A, McDougall M, Irving M, Hanna MG, Matthews E, Sarkozy A, Hudson J, Barresi R, Bushby K, Jungbluth H, Wraige E (2011) Infantile onset myofibrillar myopathy due to recessive CRYAB mutations. Neuromuscul Disord 21:37–40

  10. 10.

    Goldfarb LG, Vicart P, Goebel HH, Dalakas MC (2004) Desmin myopathy. Brain 127:723–734

  11. 11.

    Dubowitz V (1999) Very severe spinal muscular atrophy (SMA type 0): an expanding clinical phenotype. Eur J Paediatr Neurol 3:49–51

  12. 12.

    Bordessoule A, Emeriaud G, Dlenard N, Beck J, Jouvet P (2010) Recording diaphragm activity by an oesophageal probe: a new tool to evaluate the recovery of diaphragmatic paralysis. Intensive Care Med 36:1978–1979

Download references

Author information

Correspondence to Miriam R. Fine-Goulden.

Appendix: NAVA catheter positioning

Appendix: NAVA catheter positioning

As referenced in the main text, the NAVA catheter was positioned as previously described. The appropriately sized catheter was selected by the child’s age, as recommended by the manufacturer. The ‘NEX’ (nose-ear-xiphisternum) technique was used to estimate catheter length, and the correct position was confirmed by inspection of the four oesophageal ECG tracings, with the blue markers present in the two middle tracings [4, 5].

See Figs. 7, 8, 9 and 10.

Fig. 7

(Case 3). Twelve-hour ventilatory trend of peak inspired airway pressure (PIP) (cm H2O), respiratory rate (per min), inspired tidal volume (TV) and peak Edi (μV) in a patient with repaired diaphragm hernia on NAVA mode with support of 2 cm H2O/μV. Median (IQR) for peak Edi was 5.3 μV (3.0–9.6) and inspired tidal volume 4.3 ml/kg (3.5–5.3). Edi was >40 μV for 8 % of the time and between 20 and 40 μV for 8 % of the time

Fig. 8

Edi in a spontaneously ventilating neonate with spinal muscular atrophy (SMA) and end-stage respiratory failure demonstrating a similar pattern to case 5, with high Edi signals (25–50 μV) and tonic activity of the diaphragm. The lower figure is 2 h after the first 20-s recording. Prior to extubation the median Edi was 38 μV (IQR 30–45, max 58) on a pressure support of 10 cm H2O above a PEEP of 5 cm H2O. Respiratory failure proved fatal following a palliative care pathway

Fig. 9

Volume (ml), pressure (cm H2O), flow (l/s) and Edi (in μV) in a 2-year-old ventilator-dependent patient with Pompe’s disease and severe myopathy. The patient is breathing spontaneously via an endotracheal tube at 8 cm H2O of CPAP with an end tidal CO2 of 9 kPa. Only four breaths demonstrate a prompt return to baseline Edi on expiration (arrows). We include this case as a comparison with the children with SMA (Figs. 5, 8), in which there is sparing of the diaphragm motor units until later on in the disease process. In Pompe’s, the diaphragm itself is myopathic at an earlier stage of the disease. In both cases, tonic activity of the diaphragm may be present as a means to preserve functional residual capacity [8]

Fig. 10

Second recording of case 6 (diaphragm dystonia) during spontaneous ventilation via an endotacheal tube at zero PEEP. Hypercarbia is mild (end tidal CO2 7 kPa). Arrows indicate diaphragm activity out of synch with visible phasic respiratory efforts (nasal flaring, shoulder and rib cage movement). The patient generates adequate and regular tidal volumes during spontaneous ventilation, but this is uncoupled with the electrical activity of the diaphragm, presumably due to diaphragm dystonia with flow triggering. Hypercarbia ensued rapidly during generalised dystonic movements (Fig. 6)

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Fine-Goulden, M.R., Puppala, N.K. & Durward, A. Mechanisms of ventilator dependence in children with neuromuscular and respiratory control disorders identified by monitoring diaphragm electrical activity. Intensive Care Med 38, 2072–2079 (2012).

Download citation


  • Neurally adjusted ventilator assist (NAVA)
  • Diaphragm
  • Fatigue
  • Children
  • Ventilator asynchrony