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Interact CardioVasc Thorac Surg 2009;8:171-172. doi:10.1510/icvts.2008.187096
© 2009 European Association of Cardio-Thoracic Surgery

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Case report - Congenital

Non-invasive positive pressure ventilation for bilateral diaphragm paralysis after pediatric cardiac surgery

Lubica Kovacikova*, Dusan Dobos and Martin Zahorec

Intensive Care Unit, Pediatric Cardiac Center, Limbova 1, 833 51 Bratislava, Slovakia

Received 26 June 2008; received in revised form 12 September 2008; accepted 17 September 2008

*Corresponding author. Tel.: +421-2-59371729; fax: +421-2-54792317.

E-mail address: lkovacikova{at}yahoo.com (L. Kovacikova).


    Abstract
 Top
 Abstract
 1. Introduction
 2. Case presentation
 3. Discussion
 4. Conclusion
 References
 
We present the case histories of two children having respiratory failure due to bilateral diaphragm paralysis after cardiac surgery. In both children non-invasive positive pressure ventilation alleviated respiratory distress, improved gas exchange, and prevented the need for endotracheal intubation. Following unilateral recovery of diaphragmatic function both children were successfully weaned from non-invasive positive pressure ventilation.

Key Words: Ventilation; Congenital heart disease; Diaphragm; Complications


    1. Introduction
 Top
 Abstract
 1. Introduction
 2. Case presentation
 3. Discussion
 4. Conclusion
 References
 
Bilateral diaphragm paralysis (BDP) is a very rare complication of pediatric cardiac surgery causing thoracoabdominal asynchrony. In infants and small children who breathe mainly with the diaphragm, BDP results in severe respiratory distress. These children usually require tracheal intubation and prolonged mechanical ventilation while awaiting for the diaphragm recovery [1–4].

This report documents the use of non-invasive positive pressure ventilation (NPPV) in the management of respiratory failure resulting from BDP after cardiac surgery.


    2. Case presentation
 Top
 Abstract
 1. Introduction
 2. Case presentation
 3. Discussion
 4. Conclusion
 References
 
2.1. Case 1

An infant with complete atrio-ventricular septal defect and congestive heart failure requiring mechanical ventilation underwent pulmonary artery banding at the age of six weeks. The postoperative mediastinitis required mediastinal debridement. Following complete correction of congenital heart defect at the age of 11 months, the patient failed two extubations. Ultrasonography and fluoroscopy revealed BDP. On the 16th postoperative day, the patient self-extubated and was placed on nasal continuous positive airway pressure (N-CPAP) of 10 cm H2O. Because of worsening respiratory distress the patient was placed on a flow-triggered pressure assist-control ventilator (Servo-i, Maquet Inc., Solna, Sweden). The device allows a pressure support and/or pressure control mode. Respiratory distress was relieved at Pressure Control mode with respiratory rate of 20 breaths/min, peak inspiratory pressure of 20 cm H2O, and positive end-expiratory pressure of 10 cm H2O. Because of an improperly fitting oronasal mask a nasopharyngeal tube was used as an interface for NPPV. With this therapy, breath rate decreased from 70 to 50 breaths per min, arterial partial carbon dioxide tension from 6.35 to 5.47 kPa, heart rate from 155 to 130 beats per min, and arterial partial oxygen tension increased from 12.9 to 17.6 kPa. Six weeks after surgery, the patient started to be successfully weaned from NPPV and seven weeks after surgery NPPV was no longer needed. Spontaneous movement of the right hemidiaphragm was demonstrated by ultrasonography and fluoroscopy. During the period of NPPV the patient had one episode of infection with blood and throat cultures positive for Klebsiella pneumoniae. The patient was fed by a nasogastric tube without problems with abdominal distension.

2.2. Case 2

A 3.5-year-old boy with heterotaxy, interrupted inferior caval vein with azygos continuation, unbalanced atrioventricular septal defect with dominant right ventricle, and pulmonary stenosis had a history of modified Blalock-Taussig shunt placement and bilateral bidirectional Glenn anastomosis with azygos vein left in the atrial end of the right superior caval vein. Following median sternotomy for bidirectional Glenn procedure the patient had left-sided diaphragmatic paralysis. The function of diaphragm was not assessed before Fontan procedure. The recent surgery included fenestrated Fontan procedure, connection of hepatic veins to extracardiac conduit and anastomosis of the superior caval vein on the right pulmonary artery. The patient was extubated 17 h after surgery. Because of increased respiratory distress he was placed on N-CPAP. Ultrasonography and fluoroscopy demonstrated BDP. On the fifth postoperative day, the patient was in severe respiratory distress. Non-invasive ventilation was applied through the anesthesia oronasal mask using a pressure control mode (a ventilator Servo-i). Initially, peak inspiratory positive airway pressure was set at 20 cm H2O, end-expiratory positive airway pressure at 10 cm H2O, and respiratory rate at 20 breaths per min. Respiratory distress abated, the respirations dropped from 50 to 25 per min, and arterial partial oxygen tension increased from 8.6 to 11.1 kPa.

Use of an oronasal mask was complicated with skin breakdown over the nasal bridge and cheeks and secondary herpetic infection requiring antiviral therapy. During the period of NPPV the patient had two episodes of respiratory tract infection. Because of chylothorax the patient had been receiving parenteral nutrition followed by a medium-chain triglyceride diet through a nasogastric tube. After a six-week period of non-invasive ventilation, diaphragmatic movement of the left hemidiaphragm was detected ultrasonographically and fluoroscopically. As a weaning attempt had failed, plication of the right side of the diaphragm was performed. Sixteen hours after diaphragm plication, the boy was extubated and NPPV was reinstituted. Weaning process lasted one week. The patient was completely liberated from NPPV on the 56th postoperative day.


    3. Discussion
 Top
 Abstract
 1. Introduction
 2. Case presentation
 3. Discussion
 4. Conclusion
 References
 
In contrast to unilateral diaphragm paralysis, the patients with BDP represent the higher risk group of cardiosurgical patients who require more aggressive management. Earlier reports recommend the use of tracheostomy [5, 6], however, a recent report [1] highlights the early recovery of the diaphragm and the effectiveness of conservative management. In the latter report, mechanical ventilation by nasotracheal tube was required for 14–62 days and maximum time to recovery was seven weeks.

Data on early plication of both diaphragms in BDP are lacking. Our management includes mechanical ventilation until one side of the diaphragm recovers. If necessary, plication of the other diaphragm is performed. Diaphragm function is monitored twice a week.

Our traditional approach was to intubate patients and avoid tracheostomy. However, intubation and positive- pressure ventilation increases the risk of infection, lung damage and, if heavy sedation is used, also leads to muscle atrophy and weakness [7].

Recently, non-invasive ventilation has been applied to various types of respiratory failure, but data on the use of non-invasive ventilation in pediatric cardiosurgical patients are limited [8, 9]. In this paper, we describe the efficacy of NPPV as a mode to provide prolonged mechanical ventilation in children with BDP resulting from cardiac surgery.

To avoid tracheal intubation N-CPAP was firstly applied in both children. This ventilatory mode is used in the management of unilateral diaphragmatic paralysis as it stabilizes the diaphragm and increases the functional residual capacity [10]. In our patients N-CPAP ameliorated respiratory distress only temporarily. Thus, NPPV as a ventilatory mode that actively assists inspiration was contemplated. Pressure control mode, delivering and maintaining the preset pressure at a preset respiratory rate showed to be effective for improvement of vital signs and blood gas exchange in both patients. Since diaphragmatic paralysis primarily impairs inspiration, it is very likely that control mode of NPPV in these cases served as a means of compensation for the lack of diaphragmatic contraction.

Non-invasive ventilation was delivered via a nasopharyngeal tube in the 11-month-old infant. Although problems with high leakage were expected with use of the tube, ventilator with non-invasive mode adapted to variations in leakage adequately. In the 3.5-year-old child, use of the anesthesia oronasal mask was complicated with facial skin breakdown and a secondary herpetic infection. Atopic dermatitis, long-term pressure of anesthesia type of oronasal mask, and immunodeficiency resulting from postoperative chylothorax might have played a role in the development of these complications.

Non-invasive ventilation required minimal sedation and allowed the close contact between a child and the parent. Nasogastric tube feeding provided adequate alimentation without abdominal distension.


    4. Conclusion
 Top
 Abstract
 1. Introduction
 2. Case presentation
 3. Discussion
 4. Conclusion
 References
 
Our experience demonstrates that NPPV may be an effective therapeutic approach to relieve respiratory distress and improve blood gas exchange in children with BDP after cardiac surgery.


    References
 Top
 Abstract
 1. Introduction
 2. Case presentation
 3. Discussion
 4. Conclusion
 References
 

  1. Dagan O, Nimri R, Katz Y, Birk E, Vidne B. Bilateral diaphragm paralysis following cardiac surgery in children. 10-years' experience. Intensive Care Med 2006;32:1222–1226.[CrossRef][Medline]
  2. de Leeuw M, Williams JM, Freedom RM, Williams WG, Shemie SD, McCrindle BW. Impact of diaphragmatic paralysis after cardiothoracic surgery in children. J Thorac Cardiovasc Surg 1999;118:510–517.[Abstract/Free Full Text]
  3. Joho-Arreola AL, Bauersfeld U, Stauffer UG, Baenziger O, Bernet V. Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children. Eur J Cardiothorac Surg 2005;27:53–57.[Abstract/Free Full Text]
  4. Akay TH, Ozkan S, Gultekin B, Uguz E, Varan B, Sezgin A, Tokel K, Aslamaci S. Diaphragmatic paralysis after cardiac surgery in children: incidence, prognosis and surgical management. Pediatr Surg Int 2006;22:341–346.[CrossRef][Medline]
  5. Stewart S, Alexson C, Manning J. Bilateral phrenic nerve paralysis after the Mustard procedure. Experience with four cases and recommendations for management. J Thorac Cardiovasc Surg 1986;92:138–141.[Abstract]
  6. Commare MC, Kurstjens SP, Barois A. Diaphragmatic paralysis in children: a review of 11 cases. Pediatr Pulmonol 1994;18:187–193.[Medline]
  7. Ross Russell RI, Helms PJ, Elliott MJ. A prospective study of phrenic nerve damage after cardiac surgery in children. Intensive Care Med 2008;34:728–734.[CrossRef][Medline]
  8. Chin K, Takahashi K, Ohmori K, Toru I, Matsumoto H, Niimi A, Doi H, Ikeda T, Nakahata T, Komeda M, Mishima M. Non-invasive ventilation for pediatric patients under one year of age after cardiac surgery. J Thorac Cardiovasc Surg 2007;134:260–261.[Free Full Text]
  9. Tokuda Y, Matsumoto M, Sugita T, Nishizawa J. Nasal mask bilevel positive airway pressure ventilation for diaphragmatic paralysis after pediatric open-heart surgery. Pediatr Cardiol 2004;25:552–553.[CrossRef][Medline]
  10. Haller JA Jr, Pickard LR, Tepas JJ, Rogers MC, Robotham JL, Shorter N, Shermeta DW. Management of diaphragmatic paralysis in infants with special emphasis on selection of patients for operative plication. J Pediatr Surg 1979;14:779–785.[Medline]




This Article
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