Successful pregnancy and cesarean delivery via noninvasive ventilation in mitochondrial myopathy

Abstract

We report a case study of a 22-year-old woman with mitochondrial thymidine kinase 2 deficiency and chronic respiratory failure due to severe neuromuscular weakness requiring noninvasive positive pressure ventilation (NIPPV) since 12 years of age. During pregnancy and cesarean delivery, she was successfully supported with NIPPV. A multidisciplinary team approach should be used in pregnant patients with these disorders with specific attention to management of pulmonary complications, selection of route of delivery, anesthesia, and analgesia.

Non-invasive mechanical ventilation with spinal anesthesia for cesarean delivery

“…Non-invasive mechanical ventilation is most commonly performed in ICU, on chest disease wards, and in the ER; 2 however, it can also be used for preoxygenation and postoperative recovery in patients with respiratory issues following major surgery. 2,3 The intraoperative use of NIMV is rarely reported, 2,4,5,11 although BiPAP during vaginal delivery with spinal analgesia has been described. 12 In our case, BiPAP was used preoperatively, and when positioning for spinal anesthesia became difficult due to continued dyspnea and orthopnea.…”

Successful use of noninvasive ventilation in pregnancy

“…With the assistance of NIV, all four females delivered healthy, full-term babies [2]. Further descriptions of NIV use in neuromuscular disease include two cases of successful pregnancies in females with chronic ventilatory insufficiency due to mitochondrial myopathy [4,5].…”

“…Several case reports and small series have shown favourable outcomes with NIV use for respiratory failure in pregnancy (table 1) [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19].…”

“…Patient selection is paramount and indications and contraindications to its use must be considered carefully. To optimise the safety of NIV in this setting, staff should be [15] 2006 Case report 1 Non-cardiogenic pulmonary oedema AL-ANSARI [12] 2007 Case series 4 ARDS, acute chest syndrome PERBET [6] 2008 Case report 1 Non-cardiogenic pulmonary oedema associated with tocolytic agents BANGA [9] 2009 Case report 1 ARDS, community-acquired pneumonia BASSANI [10] 2009 Case report 1 ARDS/ATRA syndrome YUAN [5] 2009 Case report 1 Ventilatory failure, neuromuscular (mitochondrial myopathy) GUTERRES [14] 2010 Case report 1 Ventilatory failure, neuromuscular (high neuraxial blockade) DJIBRÉ [11] 2010 Case report 1 ARDS, H1N1 ERDOGAN [13] 2010 Case report 1 Non-cardiogenic pulmonary oedema, severe preeclampsia FRASSANITO [17] 2011 Case report 1 ARDS, sepsis ROJAS-SUAREZ [7] 2011 Case report 1 Non-cardiogenic pulmonary oedema, severe preeclampsia DUAN [8] 2012 Case report 1 Aspiration pneumonia DALAR [18] 2013 Case report 1 Community-acquired pneumonia DRAISCI [19] experienced in its use and patients should be in a protected environment, such as an ICU, in order to monitor changes in the clinical state of the mother and fetus.…”

“…With the assistance of NIV, all four females delivered healthy, full-term babies [2]. Further descriptions of NIV use in neuromuscular disease include two cases of successful pregnancies in females with chronic ventilatory insufficiency due to mitochondrial myopathy [4,5].NIV in pregnancy for indications other than ventilatory failure have also been described. Several studies demonstrating favourable outcomes with NIV use in parturients with hypoxaemic respiratory failure have been performed.…”