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Management in children
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The combination of NIPPV with cough assistance using mechanical insuationexsuation to control bronchial secretions can help to reduce the frequency of
chest infections and their complications. The use of cough assistance and NIPPV has
decreased the need for tracheostomy ventilation in neuromuscular disease.
Current indications for tracheostomy ventilation include:
• Severe bulbar weakness leading to aspiration
• Upper airway problems
• Near 24-h ventilator dependency
• Failure to control ventilation using the noninvasive mode
• Intractable interface problems
• Patient/family choice
Treatment options
Hypoventilation is always most severe during sleep, which is either due to central
or peripheral causes. Ventilatory support aims to provide optimal gas exchange, i.e.
normal P
and grow up with their families. Positive pressure ventilation via tracheostomy, bilevel
aO
2
and P
. Long-term ventilatory support at home allows children to live
aCO
2
positive pressure ventilation via nasal or facial mask (NIPPV) and respiratory pacing
are the main options for patients with sleep hypoventilation syndromes.
Positive pressure ventilation via tracheostomy
Commercially available positive pressure ventilators for long-term home use are
equipped with a battery and are easily portable. However, not all positive pressure
ventilators are suitable for infants and young children who require delivery of small
tidal volumes. Pressure-controlled ventilation is recommended for children with
CCHS; alternatively, use of a pressure-controlled mode with minimal guaranteed tidal
volume or V’E is suggested. Particular attention should be paid to the selection of
a ventilator circuit that is appropriate for the child’s body size and is able to deliver
heated and humidified air to the patient.
Management of tracheostomy should be completed at least annually at a centre with
extensive experience in the care of technology-dependent children. A tracheostomy
cannula smaller than the airway diameter should be selected to avoid traumatic
lesions of the airway wall; it should also be large enough to ensure adequate alveolar
ventilation during sleep.
NIPPV
NIPPV is delivered via a nasal mask or face mask using bilevel positive pressure
ventilators. These ventilators use a blower to produce a variable continuous flow
and can compensate for leaks occurring around the mask or where there is infectionrelated reduced lung compliance. The usual starting pressures are 8 cmH2O for
IPAP and 4 cmH2O for EPAP. Aer titration, they usually range 10–14 cmH2O for IPAP
and 4–6 cmH2O for EPAP. A timed mode ensures a minimal number of breaths is
delivered to a child who is unable or too weak to trigger the ventilator and to patients
with CCHS. Children with an underlying neuromuscular condition should always be
ventilated with a volume-guarantee mode. Some ventilators are now equipped with
a volume preset-pressure mode; for example, average volume-assured pressure
support (AVAPS), which allows delivery of a preset tidal volume by adjusting the
inspiratory pressure support within a set range. Ventilator settings should be checked
regularly with overnight monitoring, including oxygen saturation (S
dioxide tension (P
patient–ventilator synchrony.
), as well as review of ventilator downloads to assess optimal
CO
2
) and carbon
pO
2
404
ERS Handbook: Respiratory Sleep Medicine
Management in children
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Mask ventilation has been associated with midface hypoplasia when used in infants
or young children. The risk of midface hypoplasia induced by NIPPV application can
be reduced by rotation of interfaces. Close follow-up by a multidisciplinary team,
including a paediatric orthodontist or maxillofacial surgeon, is highly recommended.
Respiratory pacing
Respiratory pacing generates breathing by electrically stimulating the patient’s
diaphragm. A battery-operated external device produces trains of electrical pulses that
are transformed into radiofrequency signals and transmitted to internally implanted
receivers. The latter are connected to electrodes either inserted around the phrenic
nerves (phrenic nerve pacing) or directly implanted into the diaphragm muscle (direct
diaphragm pacing). Phrenic nerve pacing requires a normally functioning phrenic
nerve–diaphragm axis. This technique has been shown to provide ecient ventilatory
support. In contrast, direct diaphragm pacing is a newer technique that needs to be
more extensively assessed in adults and is still scarcely used in children. The surgical
procedure and initiation of the pacing system should be completed by a multidisciplinary
paediatric team with extensive expertise in respiratory pacing in children.
Further reading
• Fauroux B, et al. (2022). ERS statement on pediatric long-term noninvasive respiratory
support. Eur Respir J; 59: 2101404.
• Hull J, et al. (2012). British Thoracic Society guideline for respiratory management of children
with neuromuscular weakness. Thorax; 67: Suppl. 1, i1–i40.
• Kaditis AG, et al. (2016). Obstructive sleep disordered breathing in 2- to 18-year-old children:
diagnosis and management. Eur Respir J; 47: 69–94.
• Kaditis AG, et al. (2017). ERS statement on obstructive sleep disordered breathing in 1- to
23-month-old children. Eur Respir J; 50: 1700985.
• Kaditis AG, et al. (2022). Sleep studies for clinical indications during the first year of life: infants
are not small children. Children; 9: 523.
• Logjes RJH, et al. (2021). Objective measurements for upper airway obstruction in infants
with Robin sequence: what are we measuring? A systematic review. J Clin Sleep Med; 17:
1717–1729.
• Marcus CL, et al. (2013). A randomized trial of adenotonsillectomy for childhood sleep apnea.
N Engl J Med; 368: 2366–2376.
• Morton SU, et al. (2016). Treatment options for apnea of prematurity. Arch Dis Chil – Fetal and
Neonatal Edition; 101: F352–F356.
• Simonds AK, et al., eds (2012). ERS Handbook Respiratory Sleep Medicine. Sheeld, European
Respiratory Society.
• Trang H, et al. (2020). Guidelines for diagnosis and management of congenital central
hypoventilation syndrome. Orphanet J Rare Dis; 15: 252.
• Villa MP, et al. (2011). Ecacy of rapid maxillary expansion in children with obstructive sleep
apnea syndrome: 36 months of follow-up. Sleep Breath; 15: 179–184.
• Villa MP, et al. (2012). Mandibular advancement devices are an alternative and valid treatment
for pediatric obstructive sleep apnea syndrome. Sleep Breath;16: 971–976.
Acknowledgement
This is an update of the ERS Handbook of Respiratory Sleep Medicine first edition chapter
‘Management of sleep disordered breathing in children’, by Athanasios Kaditis, Maria Pia Villa,
Anita K. Simonds and Ha Trang.
405ERS Handbook: Respiratory Sleep Medicine
Index
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A
abdominal movements, measurement 120
accelerometers, PSG comparison 349f, 350t
accidents, OSA and
motor vehicle see motor vehicle accidents (MVAs)
occupational 80, 336, 340
acetazolamide 50, 162
in high-altitude periodic breathing 226
in idiopathic CSA 226
in non-hypercapnic CSA 59, 61, 216–217
achondroplasia 102, 377, 397, 399, 400
acid–base buering, chronic hypercapnic ventilatory
failure 70, 71f
acid maltase deficiency myopathy 267
acidosis
cerebral 61
respiratory 256, 257
acromegaly 102, 250–252
clinical features 250–251, 251f
CSA in 39, 251
OSA and 249f, 250
pathophysiology 250–251
treatment 252
prevalence 250
screening 252
treatment 252
ACROSCORE 252
actigraph, description 388
actigraphy
in children 388
in circadian rhythm disorders 334
devices 153
in insomnia 317
in non-respiratory sleep disorders 312
in restless legs syndrome 311
active sleep (AS) in newborns 370, 390, 391
adaptive servo ventilation (ASV) 199, 205–208, 206f
ADVENT-HF trial 208
in CPAP-emergent CSA 225–226
in CSA in HF 205–208
ecacy 207
impact on prognosis 207–208
as second-line therapy 206–207, 208
in CSA in opioid-users 210, 212–213
devices, measurements by 205–206, 206f, 212
first generation device 206, 207
in idiopathic CSA 226
in mixed OSA/CSA in opioid users 212–213
principle and aims 205–206
SERVE-HF trial see SERVE-HF trial
adenoidectomy 399–400
"adenoid facies" 386, 387
adenoids 377, 387
adenosine dysregulation, in restless legs syndrome
322
adenotonsillar hypertrophy 396, 397
adenotonsillectomy 383, 394, 396, 397, 399
benefits in children 400
ecacy for OSA in children 399–400
adherence to treatment
to CPAP in OSA see CPAP, in OSA
definition (WHO) 181
to NIV 282, 291
adjusted apnoea–hypopnoea index (adjusted AHI)
26, 26t, 34
adolescence/adolescents
breathing scoring 391
cystic fibrosis 383
delayed sleep–wake phase syndrome 333
irregular sleep–wake rhythm disorder 334
normative sleep data 392
OSAS 377
advance care planning 306–307
advance directives 306–307
advanced sleep–wake phase syndrome (ASPS) 333
ADVENT-HF trial 208
aerophagy 179
age
as CPAP adherence determinant in OSA 182
NREM–REM sleep cycling 5, 6
sleep stages, normative data 5, 123t, 128
ageing
CPAP side-eects in OSA 174
OSA prevalence 39, 46, 76
elderly phenotype 78f, 79
sleep and 5–6, 123t
see also elderly people
AHI see apnoea–hypopnoea index (AHI)
"air capnometry" 137–138
see also capnometry
airflow
ASV device action 205–206
clinical assessment of children 387
measurement, PSG 120
pressure relationship 120
spontaneous breathing 284f
turbulence at nostrils 120
in type IV sleep studies 131, 153
air leakage
CPAP in OSA 174, 175f, 176, 178
NIV 283, 288, 289–290
air stacking technique 297f, 298, 299f
airway resistance see upper airway
alcohol
abuse, insomnia disorder increasing risk 316
reduction in OSA treatment 157
alkalosis
cerebral 61
hypocapnic 63
allergic inflammation 230
allergic rhinitis 231, 382, 383
406
alveolar–arterial oxygen tension gradient (PAO2)
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65, 66
alveolar gas equation 66
alveolar hypoventilation see hypoventilation
alveolar oxygen tension (PAO2), calculation 66
alveolar ventilation see ventilation (alveolar
ventilation); ventilatory drive
Alzheimer disease 40
American Academy of Sleep Medicine (AASM) 117
CPAP in OSA with excessive sleepiness 165, 193
CPAP or APAP in OSA, guideline 172
diagnostic algorithms for OSA 147
economic burden of OSA 339
EDS assessment 141
hypopnoea definition 15
PSG for OSA diagnosis 149
PSG in children 378
sensors and associated monitoring 120, 121
sleep EEG
in children/infants 390
electrode placement 118, 119, 119f
sleep scoring 2, 118, 133
American College of Chest Physicians 117
American Thoracic Society 117, 275
amyotrophic lateral sclerosis see motor neurone
disease (MND/ALS)
anaesthesia 279
in pregnancy with hypoventilation 280–281
angiotensin-converting enzyme inhibitors 204
ankylosing spondylitis 236
antiarrhythmic drugs, SERVE-HF trial mortality 207
anticholinergic agents, in COPD 235
antidiabetic drugs 245–246
antihypertensive drug therapy 195
anti-Iglon5 disease 331
anti-inflammatory nasal topical steroids, with CPAP
therapy in OSA 177, 183
antimuscarinic medications 49, 162
anti-thyroid antibodies 248
anxiety/anxiety symptoms
in advanced COPD 307
CPAP adherence in OSA and 182
insomnia disorder relationship 316, 317, 318
in OSA 80
aortic bodies 10
Apert syndrome 401
apnoea, sleep 15, 32, 348
bradycardia and 373, 374f
brief, in infants 373
causes in infants/children 373
central see central apnoea; central sleep apnoea
(CSA)
diagnosis in infants/children 373
duration, OSA severity 23–24, 24t
hypoxia-reoxygenation cycle in CSA 85–86, 85f
mixed 16f, 373
in infants/children 391, 392
obstructive 15, 16f
in infants/children 391, 392
oximetry trace 134f
oxygen desaturation 113
physiological, in infants/children 373, 374f
portable monitoring device underestimation 194
presentations 16f
prevalence 348
severe, in obesity hypoventilation syndrome 256
severity, AHI classification 23, 33, 125, 127, 166
see also apnoea–hypopnoea index (AHI)
in sleep history 91
as symptom, causes 373
threshold 59, 60f
non-hypercapnic CSA pathophysiology 59
total time 24t
witnessed, in idiopathic pulmonary fibrosis and
OSA 236
apnoea event duration (ApDur) 24t
apnoea–hypopnoea desaturation index (AHDI) 24t
apnoea–hypopnoea index (AHI) 23, 32, 33–34, 36,
112
adjusted 26, 26t, 34
alternative parameters to 23–24, 24t, 32, 36,
112, 113, 166
analysis, artificial intelligence 365
CPAP in OSA reducing 165, 172t
CPAP tracking systems and 188–189, 365
definition 23, 32, 33, 125
driving risk and regulations 33, 115
in heart failure 59
increase 33, 36
persistent aer PAP 188
in type 2 diabetes mellitus with OSA 244
increasing arousal threshold, eect 53
level, driving regulations 115
limitations/disadvantages 23, 32, 33–34, 36, 166
limited channel/type III devices/testing 130, 131,
134
vs PSG, results 134
low 23, 33, 113
mandibular advancement devices reducing 158
men vs women 78
in OSA
in children 378, 379, 393, 398, 400
CPAP reducing 165
epidemiology based on 38
high-risk patients 112, 113, 113f
improvement threshold with PAP therapy 194
PAP therapy ecacy 193, 194, 196
OSA diagnosis 32, 112, 368
in children 393
OSA grades/severity 23, 32, 33, 113, 127, 166,
194
alternative parameters 23–24, 24t, 32, 36,
112, 113, 166
comorbidity risk assessment 112, 113
CPAP therapy indications 166
event number per hour 33, 113
mild OSA 33, 113, 127, 194
moderate OSA 33
overdiagnosis of OSA 34, 368
poor correlation with disease markers 33–34,
112
severe OSA 33, 113, 127
see also OSA/OSA syndrome (OSAS), severity
evaluation
overestimation by tracking devices 189
oxygen desaturation relationship 113
portable monitoring device limitation 193–194
as primary predictor for OSA 33, 34, 36
REM sleep, in women 78
residual (rAHI), telemonitoring of medical events
destabilising 360, 361f
SDB severity based on see grading of SDB
sensitivity and specificity 34
sleep apnoea severity based on 23, 33, 125, 127,
166
sleep eciency impacting 127
smoking relationship 156
weight gain relationship 38–39
407
weight loss decreasing 156
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apnoea–hypopnoea syndrome, sleep 129
apnoea–hypopnoea time (AHT) 24t
apnoea of prematurity 20
management 396–397
apparent life-threatening event (ALTE) (BRUEs) 370,
373–374, 386, 387
application programming interfaces (APIs) 364, 365f
arm movements, EMG 120
arousal(s) 61
AASM definition, EEG frequency 121
in CSA and CSR, eect on HF 85–86, 85f
disruption of breathing stability 45
EEG, scoring 121
children 390
measurement, SDB severity 26–27
arousal intensity 26
arousal morphology 26
arousal threshold 27
corticoperipheral neuromuscular disconnection
27
sleep depth 27
timing of arousal 26–27
multiple, insomnia with 310
in non-hypercapnic CSA 61
in NREM sleep 121
number, age aecting 5, 6
number and causes, in sleep history 91
OSA worsened by 45
in REM sleep 121
respiratory-induced 26–27
sympathetic activation induced by 85–86, 85f
threshold see respiratory arousal threshold
transient, cyclic alternating pattern (CAP) 26
arousal-AUC metric 26
arousal disorders 328
see also parasomnia, NREM
arrhythmias
in CSA and CSR 85
OSA association 81
arterial blood gas analysis 66, 136
NIV monitoring 290
obesity hypoventilation syndrome 257
arterial chemoreceptors 10
arterial hypertension see hypertension, systemic
artificial intelligence 118, 148, 345, 352, 364–366
algorithms 364
CPAP masks, optimal use 365–366
CPAP telemonitoring in OSA 365
alerts generated 360–361, 362
decision-making and action proposals 365
need for increased use 366
screening tests 356
asphyxia 372
causes 372
in infancy 372
asthma, SDB in 230–232
in children 382–383
clinical presentation 231–232
exacerbations, OSA increasing 231
OSA interactions 230, 231, 231f, 382, 383
prevalence 231
risk factors 230, 231f
treatment and impact on sleep quality 232, 382,
383
ASV see adaptive servo ventilation (ASV)
atherosclerosis
in OSA 81
see also coronary artery disease
408
atomoxetine 162
atonia, REM sleep 2, 5, 255
persisting in wakefulness 329–330
atrial fibrillation (AF) 39, 87, 103
CSA impact 88
new-onset, PAP therapy in OSA 196
OSA association 81
PAP therapy ecacy 196
attention deficit hyperactivity disorder (ADHD) 313,
379, 387
audio recordings, sleep-related symptoms 91
auto-adjusting PAP (APAP) 169, 171, 183
AHI, tracking systems and 189
for air-leakage in CPAP 176
in CSA in chronic heart failure 204, 205
ecacy, in OSA 170, 171
fixed-level CPAP vs 171–172, 172t
expiratory pressure relief with 172
limitations 170
long-term treatment in OSA 171–172
new ASV devices similar to 212
optimal pressure/titration 171
pressure algorithms 183
pressure profile 170f, 171
titration in interstitial lung diseases and OSA 240
trial in OSA 171
autonomic activation, arousal intensity as marker 26
autonomic parameters, SDB severity 27–29
cardiopulmonary coupling 29
heart rate response 29
heart rate variability 28–29
peripheral arterial tonometry 28
pulse arrival time 27–28
pulse transit time 27–28
pulse wave analysis 28
respiratory eort burden 29
autonomic regulation, cardiovascular, sleep and
11–12
AVAPS-AE 283, 288, 404
average volume-assured pressure support (AVAPS)
404
awakenings
early morning 310
in night see arousal(s)
B
bariatric surgery 156, 177, 261
baroreflex control, in NREM sleep 11
barotrauma, CPAP causing 177
Baveno classification 23, 166
bedroom–sleep association 318
behaviour, OSAS in children eect on 379
behavioural disorders, OSAS in children 379
behavioural interventions
insomnia disorder 318
narcolepsy 327
benzodiazepine(s) 319, 329
benzodiazepine receptor agonists 319
bereavement care 308
Berlin Questionnaire 97, 97t
beta-agonists 235
beta-blockers 204
bias flow 169
bicarbonate
elevated 17
acute-on-chronic ventilatory failure 302
neuromuscular conditions in children 380
obesity hypoventilation syndrome 257, 275
retention, hypercapnic ventilatory failure 70, 71
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big data 364–366, 369
bilevel positive airway pressure (BPAP) 169
in children with neuromuscular disorders 404
in CSA in chronic heart failure 204, 205
in CSA in opioid-users 212, 213
fixed CPAP vs in OSA 172, 172t
principle 170–171
spontaneous/timed mode (BPAP-ST) 213
biomarkers 30
high-risk OSA patient identification 114
OSA and SDB severity 30, 32
BLAST-OSA trial 161
blindness, non-24-hr sleep–wake rhythm disorder
334
blood pressure (BP)
24-hour measurement 195
ambulatory 195, 195t
circadian clock 13
diastolic 195t
APAP vs fixed CPAP in OSA 171
dipping phenomenon 11, 13, 80, 114
loss in OSA 80, 114
elevated see hypertension, systemic
MADs eect on 159
measurement 103
automatic remote readout 368
NREM and REM sleep 2, 11, 12, 13
oce/home levels 195, 195t
orexin peptide action, in sleep 12
in OSA 80–81, 195
CPAP reducing BP 165
high-risk patient identification 114
PAP therapy ecacy evaluation 195, 195t
systolic 195t
"blunted respiratory drive", obesity 267
body "clock" 2
see also circadian clock(s); circadian rhythm
body mass index (BMI) 102
obesity hypoventilation syndrome 254, 256, 256t
as OSA risk factor 38, 76
surgery and weight loss 156
body movement, EEG in children 390
body position
monitoring, PSG 120
respiratory events in OSA and 159–161
Bötzinger expiratory complex neurons 7
bradycardia 127
apnoea with 373, 374f
brain
iron deficiency 322
ischaemic injury in children with OSAS 379
brain arousal systems, breathing control 8–9, 8f
brain natriuretic peptide (BNP) 85, 87
brainstem centres 1, 49, 351, 371
central chemoreceptors 7, 9, 10, 60–61, 371
neuromuscular diseases involving 263, 264–265,
265t
plasticity 372
REM sleep behaviour disorder 329
respiration control 7, 8f, 49, 58f, 351
brainstem conditions
CSA due to medical disorder without CSR 19
hypercapnic CSA 16
hypoventilation/respiratory failure 70, 264, 265t,
273, 274–275
injury 264
brainstem respiratory network 7–8, 8f, 49
breath-holding 380
breathing 49–50
automatic rhythm 8–9, 8f
control
congenital failure see congenital central
hypoventilation syndrome (CCHS)
development in infants/children 7, 371–373
dysfunction, infants/children 373
during sleep/wakefulness 4f, 7–9, 8f, 57, 57f
depth, fall at sleep onset 372
development in first years of life 7, 370–375
foetal, and neonatal 7, 370–371
goals 371
irregularities, aetiology in infants 373
neural transmission pathway 7–8, 71–72, 72f
neurobiology and physiology 7–11, 8f
normal 263
periodic see periodic breathing (PB)
rate, fall at sleep onset 372
scoring in infants/children 391–392
shallow, in hypoventilation 275
in sleep, in infants/children 372
stability, arousals disrupting 45
unstable
apnoea threshold, non-hypercapnic CSA 59, 60f
arousals in non-hypercapnic CSA 61
CSR 18, 19
loop gain and 49, 50, 52, 58–59
parameters 199t
breathlessness 269, 305
nocturnal 91
noninvasive tests 270–271, 270t
palliation 306
brief resolved unexplained events (BRUEs) in infants
370, 373–374, 386, 387
bronchiectasis 279, 303
bronchopulmonary dysplasia (BPD) 384
Brouillette questionnaire 378
BRUEs (brief resolved unexplained events) 370,
373–374, 386, 387
bruxism 120
bulbar innervated muscular dysfunction 265, 298,
307
BURR (backup respiratory rate) 285t, 286t, 287
buspirone 59
CSA treatment 217, 219
C
Caesarean section 281
cancer, OSA association 82, 109
CANPAP trial 86–87, 205
capnography 137
future developments 140
nocturnal 136–140
phases 137f
capnometry 136, 137–138
future developments 140
mainstream 137
microstream 138
sidestream 137–138
carbon dioxide (CO2)
chemoreception 8f, 9
elevated see hypercapnia
end-tidal (P
measurement, capnometry 136, 137
reduction in NREM sleep (CSA) 62
sleep hypoventilation detection 136
levels, blood 9, 138
measurement methods 257
ETCO
)
2
409
carbon dioxide (cont.)
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monitoring
in infants/children 371
during sleep, transcutaneous 121
in non-hypercapnic CSA 57, 61
in NREM sleep, ventilation and 57
P
57, 59
aCO
2
increased see hypercapnia
obesity hypoventilation syndrome 254–255,
256t
in pregnancy 280
P
rise, hypercapnic respiratory failure 65
relationship/dierences 138
tcCO
2
sleep-induced hypoventilation 17, 136
V'E determined by 59, 60
on waking 17
rebreathing in COPD, ventilatory response 72
reserve 59, 60f
respiratory responses, brain regions 8f
sensitivity, in infants/children 371
set-point, wakefulness vs sleep 61
tension (P
continuous monitoring at night 138
)
CO
2
monitoring in oxygen therapy 302
monitoring methods 137, 138
nocturnal hypoventilation 273
rise, uncontrolled oxygen 302
in skin vs blood 138
see also capnography; capnometry
transcutaneous (P
bias, sources 136, 138
) 121, 136, 138, 140
tcCO
2
measurement 121, 136, 138–139, 276
measurement, technical issues 138, 139
monitoring in oxygen therapy 303
NIV monitoring 290
nocturnal hypoventilation definition 274t
P
relationship 138
aCO
2
sensor dri 138
sleep hypoventilation detection 136, 138, 139,
139f, 276
V'E change and loop gain 58, 58f
ventilatory (chemical) drive and 49
carbonic anhydrase inhibitors 50, 162, 216
see also acetazolamide
cardiac insuciency, surgery risk 279
cardiac output, in pregnancy 280
cardiogenic pulse artefact 189
cardioprotective eects, of OSA 81
cardiopulmonary coupling (CPC) 29
cardiorespiratory monitors 129–131
cardiotropic drugs 204
cardiovascular disease (CVD)
development/change, PAP treatment monitoring
188
mortality
increase with increasing hypoxic burden 25
T90 (90% threshold) association 24–25
in obesity hypoventilation syndrome 258
in OSA and metabolic syndrome 244, 245
OSA comorbidity 107–108, 112, 165, 188
OSA consequence 80–81, 80f, 107–108, 195,
196
risk factors 244
see also coronary artery disease; heart failure (HF)
cardiovascular function during sleep 11–13
autonomic regulation in sleep 11–12, 12f
circadian clocks in cardiovascular cells 12–13, 12f
impaired, with circadian clock misalignment 13
REM and NREM sleep 11, 12f, 13
410
cardiovascular system
assessment 103
chronic intermittent hypoxia eect on 86
hypoxic burden eect 25
carers
polygraphy diculties for 356
support for 308
telemonitoring role, CPAP in OSA 369
chemosensitivity, in infants 371
denervation, breathing pattern 215, 216
carotid sinus nerve (CSN) fibres 10
case management, telemedicine 344
cataplexy 92, 312, 325
catecholamines, release, arousals in CSA 86
central apnoea 222
asphyxia due to 372
in bronchopulmonary dysplasia 384
in infants/children 372, 391, 392
see also central sleep apnoea (CSA)
central apnoea index (CAI) 39, 59, 222
central breathing disturbances 55
classification 16, 55–56, 56t
central disorders of hypersomnolence 310t
central hypopnoea, in TECSA 223
central hypoventilation, management in children 403
central respiratory drive disorders 273
see also congenital central hypoventilation
syndrome (CCHS)
central sleep apnoea (CSA) 11, 15, 16f, 84–89
in acromegaly 39, 251
central apnoea index (CAI) 39, 59, 222
characteristics 222
in chronic heart failure see chronic heart failure
(CHF)
clinical aspects 84–85, 148–149, 198
clinical consequences 85–88, 85f
atrial fibrillation 87, 88
chronic kidney disease 88
impact on HF outcomes 85–87, 85f
opioid use 88
pathophysiology 85–87, 85f
stroke 88
clinical features 84–85, 149, 198
CPAP-emergent see treatment-emergent CSA
(TECSA)
CPAP-resistant 61–62, 224
with CSR 18–19, 85–87, 85f
ASV therapy eect, in HF 208
heart failure outcome and 85–87, 85f
in opioid-users 211f
PAP eect on HF 86–87
prevalence 39
risk factors 39
screening, importance 87
cycle length 56
definition/description 15, 18–21, 222
diagnosis 87, 148–149
due to high-altitude periodic breathing 19, 40,
226
pathophysiology 63
prevalence 40
treatment 198, 226
due to hyperventilation see central sleep apnoea
(CSA), non-hypercapnic
due to medical disorder without CSR 16, 19,
39–40
diabetes and acromegaly 39–40, 226–227
end-stage renal disease 40, 227
central sleep apnoea (CSA) (cont.)
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interstitial lung disease 40
neurodegenerative diseases 40
neurological disorders 227
pathophysiology 62
prevalence 39–40
pulmonary hypertension 40
renal failure 227
treatment 226–227
due to medication or substance 16, 19–20, 40
ASV therapy 210, 212–213
CPAP therapy 210–214
pathophysiology 63
prevalence 40
see also opioids, chronic use
epidemiology 39–41
in heart failure see chronic heart failure (CHF);
heart failure (HF)
hypercapnic (CSA with hypoventilation) 16, 56
conditions leading to 56, 57t
pathophysiology 56
hypoventilation syndromes 56, 57t
ICSD-3 subgroups 16, 55–56, 56t
idiopathic 226
non-hypercapnic (CSA due to hyperventilation) 16,
56–57, 56f
acetazolamide therapy 59, 61, 216–217
apnoea threshold 59
arousals 61
ASV therapy see adaptive servo ventilation (ASV)
buspirone therapy 217, 219
in chronic HF see chronic heart failure (CHF)
conditions leading to 16, 57t
cycle length 56
heart failure 57, 60
HVR and HCVR 59–61
loop gain 57–59, 58f, 199, 205
lung mechanics 61, 200
nocturnal low flow O2 therapy 215–216, 216f
PAP therapy 204–205, 206
pathophysiology 55, 56–61
prognostic impact see below
theophylline therapy 217
see also heart failure (HF)
obstructive apnoea vs, tracking systems 189
opioid-induced see opioids, chronic use
OSA coexisting
ASV therapy, in opioid users 212
PAP therapy 204
treatment flowchart 203f
pathophysiology 55–64, 199, 222
periodic breathing subgroup see periodic breathing
(PB)
phenotypes
ASV therapy response in HF 208
PAP therapy response in HF 205
polysomnography (PSG) scoring 125, 126f
prevalence 39–40
primary 20
pathophysiology 63
prevalence 41
primary of infancy 20, 41
primary of prematurity 20, 396–397
prognostic impact 198–201, 199t, 215
heart failure and LVEF severity 199, 202, 204,
215
hypoxia 199t, 200
lung mechanics 200
markers of poor outcome 199t
central sleep apnoea (CSA) (cont.)
pathophysiological risk factors 199–200, 199t
risk factors/predictors 39, 87, 149
high loop gain, ventilatory overshoot 199–200,
223
screening
in heart failure patients 87
limited sleep tests (type III) 152–154, 153f
severity
in opioid-induced SDB 210
prediction 87
treatment 215–221
acetazolamide 216–217
ASV therapy see adaptive servo ventilation (ASV)
buspirone 217, 219
ERS therapeutic approach 202, 203f, 208
flowchart 203f, 208
heart failure therapy 204
indications for 198
nocturnal low flow oxygen therapy 215–216,
216f
PAP therapy 204–205, 206
phrenic nerve stimulation 218f, 219
theophylline 217
treatment-emergent see treatment-emergent CSA
(TECSA)
centronuclear myopathy 279
cerebral acidosis 61
cerebral alkalosis 61
cerebral blood flow 60
cerebrospinal fluid (CSF), leakage 178
cerebrovascular events
CSA impact 88
in OSA 81
see also stroke
Charcot–Marie–Tooth disease 266
chemical drive 49, 50, 51f
increasing via hypercapnia 49, 50
reduced in sleep 50
chemoreceptors/chemoreception 8, 8f, 9, 10, 58f,
59–60
aortic 8, 10
arterial 10
carbon dioxide level sensitivity 8f, 9, 371
central and peripheral chemoreceptors 8f, 9,
371
compared with O2 9
infants/children 371
carotid see carotid body (CB)
central 7, 8f, 9, 10, 371
role in CSA pathophysiology 60–61
development in newborn infants 371
dysfunction 371, 372
hyperreactivity, in HF 60
in hypoxic response 9, 9f, 10, 371
in infants/children 371, 372, 373
neurological conditions involving CNS/brainstem
264
NREM sleep 9
oxygen level sensitivity 9, 9f
infants/children 371
peripheral 7, 9f, 371
hypoxia response 9, 9f, 10, 371
role in CSA pathophysiology in HF 60, 61
see also carotid body (CB)
plasticity, infants/children 372–373
proton level 8f, 9, 10
"reprogramming", in infants 372
upper airway muscle tone/activity 372
411
upregulation in HF, in CSA 86
https://t.me/medicina_free
chemosensitivity 9
carotid body, increase in newborns 371
to CO2 see under chemoreceptors/chemoreception
CSA developing under OSA treatment 62
HCVR to estimate 59–60
HVR to estimate 9f, 10, 60
to oxygen 9, 9f, 371
chemosensors 8f, 58f
brainstem respiratory network 7, 8f
see also chemoreceptors/chemoreception
chest radiography 271
chest wall compliance
in COPD 68, 68f
in kyphoscoliosis 69
in obesity and OSA 69
chest wall disorders (CWDs) 263, 267, 273
disease types 265t, 267
nocturnal hypoventilation 274
pregnancy feasibility/assessment 280
surgery and see surgery
Cheyne–Stokes respiration (CSR) 18, 55
as compensatory mechanism in HF 86–87
criteria 19
CSA with see central sleep apnoea (CSA)
impact on HF outcomes 85–87, 85f
oximetry trace 135f
in pulmonary hypertension 40
suppression, HF mortality and 86–87
Chiari malformations, type I 227
Childhood Adenotonsillectomy Trial (CHAT) 399–400
children
behavioural insomnia 386
breathing control 7, 371–372
central apnoea 391
clinical assessment 386–389
indications for respiratory investigation 388–
389
PSG see polysomnography (PSG)
comorbid respiratory diseases 382–385
allergy (allergic rhinitis) 382, 383
asthma 382–383
bronchopulmonary dysplasia 384
cystic fibrosis 383–384
interstitial lung disease 384
diagnostic techniques in 389–394
breathing, scoring 391–392
daytime sleepiness assessment 394
EEG, scoring 389–391
home studies 393
oximetry 389, 393–394
oximetry with ECG 394
PSG see polysomnography (PSG)
hypopnoea 391
irregular sleep–wake rhythm disorder 334
mixed apnoea 391
nonrespiratory sleep disturbances (non-SDB) 382,
387
in cystic fibrosis 383
in OSAS 377
normative data, PSG 392
NREM parasomnia 328
obstructive apnoea 391
OSA/OSAS 376–379
adult OSAS vs 376–377
associated complications 379, 402
clinical assessment 377
clinical presentation 377
comorbid diseases and 382, 383
412
children (cont.)
in craniofacial abnormalities 399, 401
definition using PSG 393
diagnosis confirmation 378
epidemiology 376–377
intermediate presentations 376
mild 399
moderate-to-severe 399
pathophysiology 377
PSG or polygraphy 388–389, 393
screening 388
severity assessment by PSG 378, 399
OSAS management 398–403
benefits 399
CPAP and NIPPV 400t, 402
craniofacial procedures 400t, 401
ENT procedures 399–400
indications 398–399
intranasal corticosteroids 400–401, 400t
lifestyle management 402–403
oropharyngeal exercise 402
orthodontic procedures 400t, 401–403
stepwise approach 399, 400t
tonsillectomy and/or adenoidectomy 399–401,
400t
tracheostomy 400t
treatment approach/options 399–403
paediatric rules, age 391
periodic breathing 392
physical examination 387–388
preschool, normative sleep data 392
respiratory disorders in sleep see children, sleep
disordered breathing
respiratory eort-related arousal 391–392
sleep development 7, 370–371
sleep disordered breathing (SDB) 376–381, 382
clinical assessment 377, 387–388
clinical features 377, 387–388, 388t
management 396–405
OSA/OSAS see above
sleep history 387–388
sleep hypoventilation syndromes see below
sleep duration/requirements 5, 370, 383, 392
sleep hypoventilation syndromes 380–381
CCHS see congenital central hypoventilation
syndrome (CCHS)
management 403–405
neuromuscular conditions 380–381, 403–404
NIPPV 403, 404–405
positive pressure ventilation vs tracheostomy
404
sleep-related hypoventilation, CO2 measures 392
sleep stages 389–390
stage N1 389–390
stage N2 and N3 390
stage R (REM) 390
stage W 389
snoring by 376, 377, 378, 383, 387
tracheostomy-delivered ventilation (T-IPPV)
293–294
upper airway resistance syndrome 376, 378
weight gain aer adenotonsillectomy 400
see also infant(s)
chin straps, for masks 175f, 176
choking 92t
cholinergic neurons 3f
motor inhibition of REM sleep 4f
chronic autoimmune thyroiditis 248
chronic heart failure (CHF) 103
CSA in 16, 199, 202–209, 215
https://t.me/medicina_free
ASV therapy 205–208, 206f
ASV therapy impact on prognosis 207–208,
215
ASV vs PAP therapy 207
mortality with ASV 207–208
optimising HF therapy 204
PAP treatment 204–205, 206
personalised therapy approach 208
predicting PAP therapy response 205
prognostic markers 205
reduced survival 202
symptoms 202
treatment flowchart/strategy 202, 203f, 208
see also adaptive servo ventilation (ASV)
see also heart failure (HF)
chronic hypersensitivity pneumonitis 236
chronic intermittent hypoxia (CIH) 230
eect on cardiovascular system 86
chronic kidney disease (CKD)
CSA association/impact 88
OSA comorbidity 109–110
chronic lung disease
course, and advance care planning 306–307
palliative care 306
chronic obstructive pulmonary disease (COPD)
232–235
acute exacerbation, low alveolar ventilation 65
advanced, anxiety/depression 307
airways resistance 67, 68f
awake hypoxaemia, oxygen therapy 235
diaphragmatic muscle strength 69
gas trapping in 68
hypercapnia 72, 73, 233f, 235
hypoventilation 274–275
pathophysiology 67–69, 68f, 233, 233f
management 234–235
daytime oxygen therapy 303
NIV 69, 235, 287
NIV titration 286t, 287
oxygen therapy 234–235, 302, 303
palliative 305, 306
pharmacological agents 235
tracheostomy-delivered ventilation, outcome
294
monitoring during sleep 234
nocturnal hypoxaemia 233, 233f
consequences 234
nocturnal respiratory abnormalities 233, 233f,
234
investigation 234
management 234–235
OAS overlap syndrome 103, 106–107, 232–233,
234, 275
OSA in 106, 232–235
air leakage with CPAP in 174
hypoventilation 275
outcomes 103, 234
pathophysiology 67–69, 232–233, 233f
protective/promoting factors 106
palliative care 305, 306
sleep quality 233–234
surgery in, and risks 279
ventilatory drive 72
ventilatory response to rebreathing CO2 72
work of breathing increased 68, 68f
chronic sleep deprivation 313
circadian clock(s)
in cardiovascular cell types 12–13
master (in suprachiasmatic nucleus) 1–2, 12, 332
peripheral, in cardiovascular cells 12–13, 12f
shi in adolescence 333
shi in older people 333
circadian disorders 2, 332–335, 333t
see also circadian rhythm sleep–wake disorders
(CRSWD)
circadian rhythm 1–2, 332
body functions 332
disturbances see circadian rhythm sleep–wake
disorders (CRSWD)
dopamine levels 322, 323f
duration (>24-hr) 2, 332, 334
entrainment process 2, 332
inadequate light eect, N24SWRD 334
in infants/children 370, 371
in utero 370
light on retina, setting 2, 332
measuring/monitoring 2
digital tools see digital technology
sleep–wake 1–2, 332
see also sleep–wake cycle
circadian rhythm sleep–wake disorders (CRSWD) 93t,
310t, 332–335
advanced sleep-wake phase syndrome (ASPS) 333
causes 332
clinical features 311, 333
definition/features 332
delayed sleep-wake phase syndrome (DSPS) 333
diagnostic approach 311, 312, 332, 333
ICSD-3, types 310–311, 310t, 332, 333t
irregular sleep–wake rhythm disorder (ISWRD)
333–334
jet lag disorder 334
neurodegenerative processes associated 314, 334
non-24-hr sleep–wake rhythm disorder
(N24SWRD) 334
shi work disorder 334
types 310–311, 310t, 333t
circadian signals 1
circulatory delay 50
claustrophobia 179, 239f, 240
clinical assessment methods 90–116
physical examination see clinical examination
questionnaires see questionnaires
sleep history see sleep history
see also specific methods and conditions
clinical examination 101–105
cardiovascular system 103
general inspection 102
head and neck 102, 104, 104f
neurological 103–104
obesity 101–102
in OSA 76–77, 76t
point-of-care ultrasound of OSA 104, 104f
pulmonary system 103
upper airway 102–103
clomipramine 326t
CNS depressants 41
CNS disorders, hypoventilation/respiratory failure 70,
264, 265t, 273, 274–275
cognitive-behavioural therapy (CBT)
for comorbid insomnia and sleep apnoea
(COMISA) 319, 360
for insomnia (CBT-I) 318, 319
for nightmare disorder 330
cognitive impairment, in idiopathic pulmonary
fibrosis and OSA 238, 238t
cognitive outcome
413
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