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Pediatric Pulmonology 51:189–195 (2016)

A Comparison of Invasive and Noninvasive Ventilation in

Children Less Than 1 Year of Age: A Long-Term
Follow-Up Study
Tamizan Kherani, MD, Aarti Sayal,1 Suhail Al-Saleh, MBBS, MSc,1,2 Priya Sayal, BSc,

and Reshma Amin, MD, MSc1,2*

Summary. Background: We report on the long-term survival of children initiated on invasive and
noninvasive positive pressure ventilation (NiPPV) before the age of 1 to assess the safety and
efficacy of long-term ventilation at home. Methods: A chart review was performed of children
initiated on long-term home mechanical ventilation (LTHV) before the age of 1 year, at The Hospital
for Sick Children (SickKids), Canada, between January 1991 and April 2014. Results: We report
on 51 children. Twenty-five children (49%) received NiPPV and 26 (51%) received invasive
mechanical ventilation via tracheostomy (IMV). There was one NiPPV initiation between 1991 and
2001, the rest were in subsequent years. Most children had a “musculoskeletal disorder” in the
NiPPV cohort, n ¼ 14 (56%) and a “central nervous system” disorder in the IMV cohort, n ¼ 13
(50%). The pCO2 improved with the initiation of NiPPV, P ¼ <0.0001. Of the 25 subjects initiated
on NiPPV, eight (32%) are currently being followed as compared to 22 (84%) in the IMV cohort.
Seven (28%) of the NiPPV group were weaned off ventilation as compared to three (11.5%) in the
IMV cohort. There were two NiPPV treatment failures. There were more deaths in the NiPPV
cohort: eight (32%) versus two (7.6%) in the IMV cohort. Four of the deaths in the NiPPV cohort
were in children in whom a palliative approach was taken. None were due to NiPPV technical
failure. Conclusions: Based on this long-term follow-up study, NiPPV use in infants appears to be a
viable long-term ventilation strategy. Pediatr Pulmonol. 2016;51:189–195.
ß 2015 Wiley Periodicals, Inc.

Key words: ventilation; pediatric; non-invasive; neuromuscular.

INTRODUCTION particular importance as the majority of ventilators used

in children less than 1 year of age for noninvasive
There have been a number of regional and national
ventilation at home are not approved for use below a
surveys from around the world demonstrating exponential
weight of 30 kg. Therefore, an understanding of the
growth in the pediatric long-term home mechanical
clinical outcomes of “off label noninvasive ventilation” in
ventilation (LTVH) population in the past few decade.1–18
young children is essential to facilitate clinical decision
The significant increase is due to advances in medical care
and assistive technologies partnered with an increasing
number of decisions being made to institute long-term 1
Divisionof Respiratory Medicine, The Hospital for Sick Children, Toronto,
ventilation in children who would have been previously Canada.
palliated.19,20 Additionally, there has been a deeper 2
Department of Pediatrics, University of Toronto, Toronto, Canada.
understanding of the family centered benefits for
transitioning out of acute care facilities. Conflict of interest: None.
Emerging in the last few decades in the LTHV
population is the increasing use of noninvasive positive Funding source: None reported.
pressure ventilation to manage more patients as opposed 
Correspondence to: Reshma Amin, MD, 4539, Hill Wing, Division of
to reliance on invasive mechanical ventilation via Respiratory Medicine, The Hospital for Sick Children, 555 University
tracheostomy alone. In many programs worldwide, the Avenue, Toronto, ON M5G 1X8, Canada.
majority of children are being managed noninvasively as E-mail: reshma.amin@sickkids.ca
compared to invasively.1,5,6,12,14,15,18 Although the pedi-
atric noninvasive positive pressure ventilation (NiPPV) Received 8 October 2014; Accepted 6 May 2015.
population has been described in the international DOI 10.1002/ppul.23229
literature, there is a paucity of literature reporting on Published online 16 June 2015 in Wiley Online Library
the safety and effectiveness of NiPPV in infants. This is of (wileyonlinelibrary.com).

ß 2015 Wiley Periodicals, Inc.

190 Kherani et al.

making with families. To our knowledge, Markstrom’s and after the initiation of ventilation; and (14) changes in
case series is the only report to date which has described respiratory technology.
the outcomes of an infant cohort.21 NiPPV was shown to Descriptive statistics were used to summarize the entire
effectively reduce the carbon dioxide levels in all study population, as well as ventilation subgroups (i.e.,
infants.21 IMV or NiPPV). Results are presented as frequencies, or
Our aim was to report on the long-term survival of medians and interquartile ranges as appropriate. The
children initiated on NiPPV before the age of one to assess Mann–Whitney U-test was used to compare non-
the safety and efficacy of NiPPV as compared to a parametric continuous variables. The chi-square test
comparator cohort of invasively ventilated children, the was used to compare categorical variables. Paired t-tests
current gold standard for children less than 1 year of age. were used as appropriate. A P-value <0.05 was
considered significant. Graph Pad Prism 5.0 (GraphPad
METHODS Software, Inc., La Jolla, CA) was used for all statistical
A prospective clinical database was used for a
The Research Ethics Board (REB) at SickKids,
retrospective chart review of pediatric patients who
Toronto, Canada reviewed and approved the study
were initiated on long-term home mechanical ventilation
protocol (REB 1000020884). As this was a retrospective
(LTMV) and followed in the Home Ventilation Program at
study without any identifying data, the REB deemed
The Hospital for Sick Children (SickKids), Canada
informed consent unnecessary.
between January 1991 and April 2014. For this study,
only patients initiated on LTMV, who were less than
1 year of age, were included. LTMV was defined as the
daily use of invasive mechanical ventilation (IMV) or Fifty-one children initiated on LTHVat less than 1 year
NiPPV for at least 3 months, in the patient’s home or in a of age were included in this review. Twenty-five children
long-term residential facility. Children using NiPPV were (49%) received NiPPV and 26 (51%) receive IMV. The
initially identified and then a comparator cohort of IMV demographic information for this cohort is shown in
children was identified. Children using continuous Table 1. There was no difference (P ¼ 0.1) in the median
positive airway pressure (CPAP) were excluded. Ventila- and interquartile range (IQR) for age at the time of
tion was initiated in either the sleep laboratory or the initiation between the NiPPV group, 0.6 (IQR 0.4–0.7)
intensive care unit (ICU). years versus the IMV group, 0.4 (IQR 0.1–0.7) years.
Variables chosen for examination were as follows: (1) There were no gender differences between both groups
gender; (2) age of patients currently being followed for (P ¼ 1.0). The median duration of ventilation was
ventilation; (3) age at initiation of ventilation; (4) date of different between ventilatory subgroups, 4.2 (IQR 1.6–
initiation; (5) type of hospital ward for the initiation of 9.9) years versus 1.0 (IQR 0.4–3.5) years, P ¼ 0.001, IMV
ventilation; (6) type of ventilation; (7) duration of ventilation and NiPPV, respectively. The majority of the patients
use per 24-hr day; (8) duration of ventilation usage (years); lived at home. There were no significant differences in the
(9) residence at home versus a long-term care facility; (10) use of additional technologies at home between the two
reason for ventilation (adapted from Wallis et al.5); (11) other ventilatory subgroups.
technology used in the home (e.g., supplemental oxygen, The majority of the invasively ventilated patients were
enterostomy feeding tube, ventriculoperitoneal shunt); (12) initiated in the ICU (88.5%). The location of NiPPV
current disposition of the patient; (13) CO2 (mmHg) before initiation was as follows: 72% (n ¼ 18) in the ICU, 21%

TABLE 1— Demographic Characteristics of the Study Population

Characteristic NiPPV n ¼ 25 IMV n ¼ 26 P-value

Male, n (%) 15 (60%) 15 (58%) 1.0
Age of active patients, median (IQR) years1 6.2 (1.7–9.0) 4.9 (2.7–10.8) 0.7
Age of initiation of ventilation, median (SD), years 0.6 (0.4–0.7) 0.4 (0.1–0.7) 0.1
Years on ventilation, median (IQR) years 1.0 (0.4–3.5) 4.2 (1.6–9.9) 0.001
Length of time spent using mechanical ventilation nocturnal only, n (%) 25 (100%) 17 (65%) 0.002
Resides at home, n(%) 24 (96%) 23 (88%) 0.6
Supplemental oxygen 7 (28%) 4 (15%) 0.3
Enterostomy feeding tube 19 (76%) 18 (69%) 0.8
VP shunt 0 (0%) 1 (4%) 1.0
Date provided for active patients based on NiPPV (n ¼ 8), IMV (n ¼ 22).
Bold values are statistically significant with P-values < 0.05

Pediatric Pulmonology
Noninvasive Ventilation in Infants 191

(n ¼ 5) on the hospital ward, and 13% (n ¼ 2) in the sleep

96 Number of Ventilation Initiations

laboratory. For the IMV cohort, two children were NiPPV
initiated at other acute care hospitals and the remainder IMV
were initiated in the ICU of our institution. There were 6
over nine times (n ¼ 5 vs. 46) more total ventilatory
initiations between 2002 and 2013, as compared to 4
between 1991 and 2002 (see Fig. 1). For NiPPV subgroup,
there was one NiPPV initiation between 1991 and 2001, as 2
compared to 24 initiations between 2002 and 2013.
The pre- and post-NIV blood gases are shown in 0
Table 2. The majority of these blood gases were obtained

98 7

00 9

02 1

04 3

06 5

08 7

10 9

12 1
19 199

20 199

20 200

20 200

20 200

20 200

20 200

20 201
as capillary blood gases and were taken between 8 am and


4 pm. The median (IQR) pCO2 immediately prior to
NiPPV initiation was 58.0 (50.0–82.3) mmHg. The
Fig. 1. The number of children initiated on noninvasive and
median (IQR) pCO2 after NiPPV initiation with CO2 invasive long-term mechanical ventilation at home from 1996 to
normalization between 35 mmHg to 45 mmHg was 43.0 2013.
(IQR 39.8–45.0) mmHg. The pCO2 significantly im-
proved with the initiation of NiPPV, P ¼ <0.0001 (see
Fig. 2). The median (IQR) length of time between the two
pCO2 measurements was 0.30 (IQR 0.05–0.62) months. category between the two ventilatory subgroups,
Table 3 lists the indications for long-term ventilation by P ¼ 0.002. The reason for ventilation was “musculoskel-
major diagnostic category. There were significant differ- etal” in origin in the largest proportion of total ventilated
ences in the indication for ventilation by diagnostic group, n ¼ 19 (37.2%). The highest percentage of children

TABLE 2— Pre-NIV and Post-NIV Blood Gases for the Study Cohort

Pre NIV Gases Post NIV Gases

Length of time from NIV

Patient Type of gas Time of day Support prior to NIV Type of gases Time of day initiation to normalization (days)
1 Venous 14:45 None Venous 11:10 38.1
2 Arterial 8:45 IþV1 Arterial 10:45 2.1
3 Arterial 8:50 IþV Arterial 8:55 0.9
4 Capillary 6:30 IþV Venous 19:12 18.0
5 Capillary 7:05 None Capillary 10:05 3.0
6 Venous 12:20 None Arterial 16:20 0.90
7 Capillary 15:40 CPAP Capillary 8:45 0
8 Capillary 13:10 CPAP Venous 12:50 17.1
9 Venous 14:25 CPAP Capillary 15:15 5.1
10 Venous 13:50 None Venous 9:10 18.9
11 Arterial 19:55 CPAP Arterial 23:10 324.0
12 Capillary 11:20 CPAP Capillary 12:27 17.1
13 Venous 16:10 IþV Capillary 23:15 5.1
14 Venous 16:20 CPAP Capillary 6:25 18.9
15 Arterial 4:15 None Arterial 11:50 0
16 Capillary 23:15 CPAP Capillary 10:35 2.01
17 Capillary 6:25 None Venous 14:42 324.0
18 Arterial 5:40 IþV Venous 9:45 12.0
19 Venous 22:48 None Capillary 6:48 2.1
20 Arterial 6:00 CPAP Venous 15:35 1260.9
21 Capillary 5:45 CPAP Capillary 10:15 9.0
22 Capillary 3:18 CPAP Capillary 19:25 12.0
23 Unknown Unknown None Unknown Unknown 02
24 Unknown Unknown CPAP Unknown Unknown 03
25 Unknown Unknown CPAP Unknown Unknown 17.1
Intubated and ventilated.
NiPPV started for tachypnea, not hypercarbia.
NiPPV started during PSG and improved during the overnight study.

Pediatric Pulmonology
192 Kherani et al.

hospital with a respiratory illness per year of ventilation

was not significantly different (P ¼ 0.76) in the NiPPV
PaC02 (mmHg)

and IMV group, with a median (IQR) of 21.7 (1.5–7.6)

80 and 20.1 (7.6–53.6) days, respectively. The outcomes of
the 51 children are summarized in Table 4. Of the 25
60 subjects initiated on NiPPV, eight (32%) are currently
being followed as compared to 21 (81%) in the IMV
40 cohort. Twenty-eight (n ¼ 7) percent of the NiPPV group
was weaned off ventilation as compared to three (12%) in
the IMV cohort. There were two treatment failures in the
NiPPV cohort (8%). One child was not adherent to NiPPV

Po and the other child went to invasive ventilation via

Fig. 2. The PaCO2 (mmHg) pre- and post-NiPPV initiation. Data
are represented as median (interquartile range). There were more deaths in the NiPPV cohort: eight
(32%) versus two (8%) in the IMV cohort (Table 5). The
median age at the time of death was 1.1 years and 3.0 years
were affected by “musculoskeletal disorders” for the for the NiPPV and IMV groups, P ¼ 0.4, respectively. The
NiPPV children, n ¼ 14 (56%) and by “central nervous median duration of ventilation at the time of death was not
system” disorders for the invasively ventilated children, significantly different between the two cohorts, 0.4 years
n ¼ 13 (50%). versus 2.9 years, P ¼ 0.1. In the IMV cohort, one patient
Of the 25 children receiving NiPPV, 12 (48%) patients died from sepsis after developing a pneumonia, which
were previously on CPAP, five (20%) patients were progressed to acute respiratory distress syndrome
intubated and ventilated in the ICU, and eight (32%) (ARDS). The other death was from an unknown cause.
patients were not receiving any form of technology prior In the NiPPV group, four of these patients were receiving
to NiPPV initiation. All NiPPV and IMV patients were palliative care. Patient NiPPV #1 had spinal muscular
sent home as per standard of care, including pulse atrophy (SMA) Type I. The patient developed increasing
oximetry monitoring. The number of days admitted to secretions and recurrent pneumonia and died at home.
Patient NiPPV #2 had SMA Type 1 and was prescribed
NiPPV for respiratory support. However, this was
discontinued prior to the time of death due to increasing
TABLE 3— The Etiology of the Need for Ventilatory Support
According to Type of Mechanical Ventilation agitation and restlessness. Patient NiPPV #5 also had SMA
Type 1. He was found to be unresponsive, during the day at
Cause of ventilation Major subgroup NiPPV IMV home. Withdrawal of care in the ICU occurred 3 days later.
Central nervous system Congenital central 2 7 Patient NiPPV #8 had infantile Pompe’s disease and
(n ¼ 16, 31.4%) hypoventilation associated hypertrophic cardiomyopathy. She was pre-
syndrome scribed NiPPV to support her respiratory status during a
Acquired central 0 4 6-month trial of enzyme replacement therapy. She was
hypoventilation admitted to hospital for respiratory failure and hypoten-
Spinal injury 0 1 sion. After two failed extubations, elective withdrawal of
Other central causes 1 1
Subtotal 3 13
TABLE 4— Patient Outcomes and Disposition for the 48
Neuromuscular Spinal muscular atrophy 3 1
Children Followed Between 1991 to 2013
(n ¼ 19, 37.2%) type 1
Congenital myopathy 2 2
Other myopathy 3 0
ventilation IMV
Congenital dytrophy 2 2 N ¼ 25 N ¼ 26 P-value
Musculoskeletal 4 0
Subtotal 14 5 Currently followed, n(%) 8 (32%) 21 (81%) 0.0002
Respiratory Chronic lung disease 3 3 Improved (no longer requiring 7 (28%) 3 (12%) 0.2
(n ¼ 16, 31.4%) Airway malacia 2 0 ventilation)
Diaphragm paresis 1 0 Mortality 8 (32%) 2 (8%) 0.04
Pulmonary atresia 1 0 Age at time of death, median 1.1 (0.9–1.4) 3.0 0.4
OSA 1 0 (IQR) years (1.0–5.0)
Pulmonary hypoplasia 0 2 Duration of ventilation at time 0.4 (0.2–0.9) 2.9 0.1
Airway stenosis 0 1 of death, median (IQR) (0.8–4.9)
Other 0 2 years
Subtotal 8 8 Treatment failure 2 (8%) 0 0.2

Pediatric Pulmonology
Noninvasive Ventilation in Infants 193
TABLE 5— Descriptive Characteristics of the Patients That Died While Prescribed NIV

Palliative Autopsy Monitoring Duration of

Patient Underlying disease (Yes/No) (Yes/No) Cause of terminal event during NIV NIV (years)
IMV #1 Congenital myopathy No No Pneumonia leading to ARDS Oximetry 4.87
and sepsis
IMV #2 Hypoplastic left heart and Yes No No death note Oximetry 0.84
chronic respiratory failure
NiPPV #1 SMA type I Yes No At home; unknown Oximetry 0.99
NiPPV #2 SMA type I Yes No Unknown Oximetry 0.20
NiPPV #3 Congenital myopathy No No At home; unknown Oximetry 0.51
NiPPV #4 Arthrogryposis No No Cardiorespiratory Oximetry 0.25
NiPPV #5 SMA type I Yes No Cardiorespiratory failure Oximetry 0.23
NiPPV #6 Thoracic insufficiency Unkown No Unknown Oximetry 2.51
NiPPV #7 Pulmonary atresia Unknown No Unknown Oximetry 0.44
NiPPV #8 Pompe’s disease Yes No Elective withdrawal of care Oximetry 0.29

care was completed with a one-way extubation. The four safely managed with noninvasive ventilation from
remaining deaths were in patients prescribed NiPPV who infancy.23,24 This is, in part, due to the significant
were not receiving palliative care. Patient NiPPV #3 had improvements in the availability of commercially
congenital myopathy and was prescribed NiPPV for available interfaces for young children, as well as an
hypoventilation. Despite treatment with NiPPV, this emerging option to have custom-made interfaces.25
patient continued experiencing multiple episodes of Furthermore, there have been significant advances in
aspiration pneumonia. Within 6 months of NiPPV the ventilators themselves. There were more IMV
initiation, death occurred at home. The reason for death ventilation initiations over the 2012–2013 period as
is unknown. Patient NiPPV #4 had a diagnosis of compared to any other time periods. Unfortunately, as a
arthrogryposis, scoliosis, pulmonary hypertension, and result of the relatively small patient numbers in our
GERD. She was prescribed NiPPV for nocturnal use cohort, in conjunction with this finding occurring in the
because of hypoventilation. She was found unresponsive last 2 years of the study period, we are unable to comment
during the day at home and received cardiopulmonary on whether this is an isolated finding or an emerging trend.
resuscitation at home for 25 min prior to the arrival of the The main documented complication in the noninva-
Emergency Medical Services. Brain death was confirmed sively ventilated group was midface hypoplasia (n ¼ 3 of
on day 3 of admission and withdrawal of care occurred the 25) and dermatographism (n ¼ 3 of 25), both of which
next day. The circumstances surrounding the deaths of were seen in a small minority of patients. Midface
patient NiPPV # 6 and # 7 are not available. hyopoplasia is an important consideration for pediatri-
cians initiating NiPPV because by the age of 4 years, only
60% of the adult facial structures have developed.26–28
More children were able to be weaned off ventilation in
To the best of our knowledge, we are reporting on the the NiPPV cohort as compared to the IMV comparator
largest cohort of children initiated on NiPPV at less than group. Therefore, NiPPV may be more likely to be
1 year of age. Similar to the Markstrom and Bach studies, prescribed to children with a chance of clinical improve-
we found that NiPPV was effective; there was a ment, an unclear prognosis or as part of a palliative care
significant improvement in PaCO2 levels in all patients approach where symptomatic relief is warranted, such as
after the initiation of NiPPV.21,22 No deaths were SMA type I. On the other hand, IMV was predominantly
attributable to equipment failure in our cohort which is prescribed to children with mostly central nervous system
another significant finding. (CNS) diagnoses and significant neurodisability. These
Our study provides insight into clinical practice patients were perceived as having less chance of disease
patterns over the past two decades. There has been a resolution.
significant increase in the prescription of NiPPV for There were more deaths in the NiPPV cohort. At first
children less than 1 year of age at our center. Twenty-four glance, this would suggest that NiPPV itself may be
of the 25 NiPPV initiations in the last 23 years were within contributing to a higher mortality rate than children
the last 10 years. This is consistent with the emerging initiated on invasive ventilation via tracheostomy.
literature for diagnoses, such as congenital central However, four of the eight deaths occurred in children
hypoventilation syndrome, that these children can be in whom a palliative care approach was taken at the time
Pediatric Pulmonology
194 Kherani et al.

of the initiation of NiPPV and none of the known deaths studies, adherence data for NiPPV are lacking in our
were because of equipment failure. Most of the deaths study. Our study did not report on the healthcare
appeared to be a result of the underlying disease leading to utilization in this cohort that was managed on NiPPV as
progressive respiratory failure. compared to IMV. Future directions will include a
The significant challenges with adherence to therapy provincial review of the healthcare utilization of
for pediatric NiPPV are well established in the litera- invasively and noninvasively ventilated children using
ture29–31 Only one child in our cohort was non-adherent provincial healthcare administration databases to ensure
with therapy as per parental report. This number may in acute care and homecare expenditures are not significant-
fact have been higher but objective assessment of ly greater for children managed noninvasively. Lastly, the
adherence by downloads from the ventilator itself was neurocognitive outcomes of the two cohorts of children
not formally available in the medical records for the were not formally assessed. This would be an important
duration of our retrospective review. Therefore, these data outcome measure for future, prospective, studies of long-
could not be included. Although only one patient was term use of NiPPV and IMV in infants.
completely non-compliant with therapy, we suspect that
the number of children with suboptimal adherence to
therapy in our cohort would have been much higher.
The differences in the caregiver requirements for a NiPPV initiated in children less than 1 year of age is
child at home on long-term NiPPV versus IMV, as well as increasingly being used, appears to be an effective
the associated risks, are other important considerations treatment, and many children seem to be able to be
when deciding on the mode of ventilation for a child. eventually weaned off ventilation. Although, the overall
Children who are invasively ventilated via a tracheosto- mortality rate was higher in the NiPPV cohort as
my require “24/7 eyes on care” of the tracheostomy to compared to children maintained on IMV, this appeared
ensure a stable, patent airway. There are also associated to be related to the underlying medical diagnosis as
risks of weakened cough, bleeding, and increased several children were receiving palliative care. None of
secretions related to a tracheostomy. Additionally, these the known deaths were due to equipment dysfunction.
children have the social stigma associated with a Based on the results of this long-term study, NiPPV use in
tracheostomy. On the other hand, the use of NiPPV for infants appears to be a viable long-term ventilation
more than 12 hr is associated with increased risk of skin strategy. Future directions include the development of a
breakdown from the interface. NiPPV needs to be national registry for ventilated children, and the assess-
discontinued during oral feeds and occasionally during ment of the neurocognitive outcomes and relative
enterostomy feeds to minimize the risk of gastroesopha- healthcare costs of children receiving invasive versus
geal reflux and aspiration. Surprisingly in our cohort, non-invasive long-term ventilation at home.
albeit small, very few of these potential complications
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