Table 2.
Characteristics and outcomes of 60 studies included in the systematic review on infants using long-term NIV.
| First author, year, country | Study design | Study duration | Total n(M/F) | Infants on NIV | Age [mean ± SD or median (range) unless otherwise stated] | Interventions | Infant NIV outcomes |
|
|---|---|---|---|---|---|---|---|---|
| Primary | Secondary | |||||||
| Articles on airway disorders: obstructive sleep apnea | ||||||||
| Downey (20), 2000, USA | Quantitative: observational (cohort) | 7 years | 18 (n/a) | n = 10a | Overall: <2 years | CPAP (n = 14) IMV (n = 4) |
• Changes in respiratory parameters • Discontinuation of NIV |
• Number of subjects on NIV |
| Guilleminault (21), 1995, USA | Quantitative: observational (cohort) | n/a | 74 (35/39) | n = 74 | 24 ± 9 weeks | CPAP (n = 74) | • Discontinuation of NIV | • Number of subjects on NIV |
| Harrington (22), 2003, Australia, Finland | Quantitative: observational (case–control) | n/a | 18 (11/7) | n = 6 | 13 ± 4 weeks | CPAP (n = 6) | • Changes in respiratory parameters | • Number of subjects on NIV |
| Leonardis (23), 2013, USA | Quantitative: observational (cross-sectional) | 4 years | 126 (86/40) | n = 18 | NIV group: 16 months | None (n = 33) NIV (n = 18) IMV (n = 7)b |
• Changes in respiratory parameters | • Number of subjects on NIV |
| Liu (24), 2012, China | Quantitative: observational (case series) | n/a | 3 (2/1) | n = 2 | Overall: 1 month to 5 years Infants: 1–7 months |
CPAP (n = 2) BPAP (n = 2) |
• Changes in respiratory parameters | • Number of subjects on NIV • Benefit of NIV (growth parameters) |
| Marcus (25), 1995, USA | Quantitative: observational (cross-sectional) | n/a | 94 (60/34) | n = 3c | Overall: <1–19 years Infants: <1 year (n = 3) |
CPAP (n = 94) | • Number of subjects on NIV* | |
| Massa (26), 2002, UK | Quantitative: observational (cohort) | 5 years | 66 (39/27) | n = 9c | Overall: 5.9 ± 5.1 years Infants: <1 year (n = 18) |
CPAP (n = 66) | • Number of subjects on NIV* | |
| McNamara (27), 1995, Australia | Quantitative: control before–after | 0.5 years | 5 (2/3) | n = 5 | 8–12 weeks | CPAP (n = 5) | • Changes in respiratory parameters • Discontinuation of NIV • Survival/mortality |
• Number of subjects on NIV |
| McNamara (28), 1999, Australia | Quantitative: observational (case–control) | n/a | 24 (13/11) | n = 8 | CPAP group: 10.8 ± 1.3 weeks | CPAP (n = 8) | • Changes in respiratory parameters • Discontinuation of NIV |
• Number of subjects on NIV |
| McNamara (29), 1999, Australia | Quantitative: observational (cohort) | n/a | 24 (15/9) | n = 24 | 1–51 weeks | CPAP (n = 24) | • Changes in respiratory parameters | • Number of subjects on NIV |
| Robison (10), 2013, USA | Quantitative: observational (cross-sectional) | 4 years | 295 (196/99) | n = 18 | CPAP/bi-level group: 15.6 months (3–29 months) | None (n = 76) NIV (n = 18) T&A (n = 116) IMV (n = 6)b |
• Changes in respiratory parameters | • Number of subjects on NIV |
| Rosen (30), 2010, USA | Quantitative: observational (case series) | 5.5 years | 16 (n/a) | n = 6 | Overall: <2 years | CPAP (n = 6) | • Discontinuation of NIV | • Number of subjects on NIV |
| Articles on airway disorders: Pierre Robin sequence | ||||||||
| Amaddeo (31), 2016, France | Quantitative: observational (cohort) | 1 year | 44 (n/a) | n = 9 | Infants: 0–2 months | CPAP (n = 9) | • Changes in respiratory parameters • Discontinuation of NIV • Hospitalizations |
• Number of subjects on NIVAdherence to NIV |
| Cheng (32), 2011, Australia | Quantitative: observational (case series) | 5 years | 6 (n/a) | n = 6 | 26 days to 11 months | CPAP (n = 6) | • Number of subjects on NIV* | |
| Daniel (33), 2013, Australia | Quantitative: observational (cross-sectional) | 12 years | 39 (16/23) | n = 18 | n/a | CPAP (n = 18) | • Number of subjects on NIV* | |
| Goudy (34), 2017, USA | Quantitative: observational (cohort) | 9 years | 38 (18/20) | n = 9 | n/a (neonates) | NIV (n = 9) NPA (n = 14) IMV (n = 8) MDO (n = 5) |
• Number of subjects on NIV • NIV success/failure |
|
| Kam (35), 2015, Canada | Quantitative: observational (cohort) | 11 years | 139 (72/67) | n = 20d | 23 months (5 days to 8 years) | None (n = 61) CPAP (n = 20) IMV (n = 19)b |
• Hospitalizations | • Number of subjects on NIV |
| Leboulanger (36), 2010, France | Quantitative: observational (case series) | 10 years | 7 (3/4) | n = 7 | 1–10 months | CPAP (n = 5) BPAP (n = 2) |
• Changes in respiratory parameters • Discontinuation of NIV |
• Number of subjects on NIV • Adherence to NIV |
| Müller-Hagedorn (37), 2017, Germany | Quantitative: observational (cohort) | 7 years | 68 (n/a) | n = 5 | n/a | CPAP (n = 5) | • Number of subjects on NIV* | |
| Articles on upper airway disorders: Laryngo-tracheomalacia | ||||||||
| Essouri (38), 2005, France | Quantitative: control before–after | n/a | 10 (5/5) | n = 10 | 9.5 months (3–18 months) | None (n = 10) CPAP (n = 10) BPAP (n = 10) |
• Changes in respiratory parameters | • Number of subjects on NIV |
| Fauroux (39), 2001, France, UK | Quantitative: control before–after | n/a | 12 (10/2) | n = 5 | Overall: 32.9 ± 25.8 months Infants: 8–19 months |
None (n = 12) BPAP (n = 12) |
• Changes in respiratory parameters • Discontinuation of NIV |
• Number of subjects on NIV • Adherence to NIV • Benefit of NIV (growth parameters) |
| Shatz (40), 2004, Israel | Quantitative: observational (cohort) | 3 years | 50 (36/14) | n = 50 | 6.5 ± 3.5 months (1–18 months) | CPAP (n = 5) BPAP (n = 9) |
• Discontinuation of NIV | • Number of subjects on NIV • Improvement in underlying disease |
| Zwacka (41), 1997, Germany | Quantitative: observational (case series) | n/a | 10 (5/5) | n = 10 | 3 weeks to 5 months | CPAP (n = 7) | • Changes in respiratory parameters | • Number of subjects on NIV • Benefit of NIV (growth parameters) |
| Articles on airway disorders: breath holding spells | ||||||||
| Guilleminault (42), 2007, USA, Taiwan | Quantitative: observational (case–control) | 2.5 years | 19 (11/8) | n = 14 | 31 ± 3 weeks | CPAP (n = 14) | • Changes in respiratory parameters | • Number of subjects on NIV • NIV success/failure |
| Articles on neuromuscular disease: spinal muscular atrophy type 1 | ||||||||
| Bach (43), 2000, USA | Quantitative: observational (case series) | n/a | 11 (6/5) | n = 8 | 3–28 months | BPAP (n = 11) | • Hospitalizations • Survival/Mortality |
• Number of subjects on NIV • Benefit of NIV (extubation) • Benefit of NIV (growth parameters) |
| Bach (44), 2002, USA | Quantitative: observational (cohort) | 5 years | 56 (n/a) | n = 33 | Overall for patient groups: NIV: 11.2 ± 5.7 months IMV: 10.8 ± 5.0months supportive: 6.0 ± 1.3 months |
NIV (n = 33) IMV (n = 16) None (n = 7) |
• Hospitalizations • Survival/mortality |
• Number of subjects on NIV |
| Bach (45), 2003, USA | Quantitative: observational (case series) | n/a | 3 (2/1) | n = 3 | 4–11 months | NIV (n = 3) | • Number of subjects on NIV • Benefit of NIV (growth parameters) |
|
| Bach (46), 2007, USA | Quantitative: observational (cohort) | 13 | 92 (n/a) | n = 92d | Therapy group: none: 6.6 ± 4.1 months bi-level: 10.6 ± 5.7 months IMV: 14.8 ± 15.2 months |
None (n = 18) BPAP (n = 47) IMV (n = 27) |
• Hospitalizations • Survival/mortality |
• Number of subjects on NIV |
| Barnerias (47), 2014, France | Quantitative: observational (cross-sectional) | 20 years | 222 (n/a) | n = 8 | Overall: 3 months (0.5–8 months) | NIV (n = 8) | • Number of subjects on NIV* | |
| Birnkrant (48), 1998, USA | Quantitative: observational (case series) | 2 years | 4 (3/1) | n = 3 | 4–9 months | BPAP (n = 4) | • Survival/mortality | • Number of subjects on NIV • Benefit of NIV (extubation) |
| Chatwin (49), 2011, UK | Quantitative: observational (cohort) | 19 years | 13 (8/5) | n = 13 | 4–24 months | BPAP (n = 13) | • Survival/mortality | • Number of subjects on NIV • Benefit of NIV (growth parameters) |
| Ednick (50), 2008, USA | Quantitative: observational (cohort) | 3.5 years | 7 (1/6) | n = 7 | 8.3 ± 3.7 months | BPAP (n = 7) | • Number of subjects on NIV • Benefit of NIV (extubation) |
|
| Gregoretti (51), 2013, Italy | Quantitative: observational (case series) | 18 years | 194 (103/91) | n = 31 | NIV group: 12.6 ± 14.4 months (0–42 months) IMV group: 6.9 ± 4.3 months |
None (n = 121) NIV (n = 31) IMV (n = 42) |
• Hospitalizations • Survival/mortality |
• Number of subjects on NIV |
| Ioos (52), 2004, France | Quantitative: observational (cohort) | n/a | 180 (n/a) | n = 33 | 19 ± 17 months | n/a | • Number of subjects on NIV* | |
| Lemoine (53), 2012, USA | Quantitative: observational (cohort) | 7 years | 49 (31/18) | n = 49 | Groups: NIV: 136 days (34–196 days) Supportive care: 69 days (38–145 days) |
None (n = 23) BPAP (n = 26) |
• Hospitalizations • Survival/mortality |
• Number of subjects on NIV |
| Ottonello (54), 2011, Italy | Quantitative: observational (cohort) | 4 years | 16 (n/a) | n = 14e | Overall: <3 years Infants: 10.4 ± 6.2 months |
NIV (n = 16) | • Hospitalizations • Survival/mortality |
• Number of subjects on NIV • Benefit of NIV |
| Petrone (55), 2007, Italy | Quantitative: control before–after | n/a | 9 (7/2) | n = 9d | 7 months (2–33 months) | BPAP (n = 9) | • Changes in respiratory parameters | • Number of subjects on NIV |
| Vasconcelos (56), 2005, Portugal | Quantitative: observational (cohort) | 11 years | 22 (16/6) | n = 7d | Overall: 5.5 years (6 months to 26 years) SMA type 1 group: 13 months (3 months to 3 years) |
None (n = 5) BPAP (n = 17) |
• Hospitalizations • Survival/mortality |
• Number of subjects on NIV • Benefit of NIV (growth parameters) |
| Articles on neuromuscular disease: achondroplasia | ||||||||
| Afsharpaiman (57), 2011, Iran, Australia | Quantitative: observational (cohort) | 15 years | 46 (22/24) | n = 7 | Overall: 3.9 years Infants: <2 years (n = 7) |
CPAP (n = 9) AT (n = 13) |
• Number of subjects on NIV* | |
| Articles on neuromuscular disease: multiple (spinal muscular atrophy type 1 and congenital myopathy) | ||||||||
| Han (58), 2015, Korea | Quantitative: observational (cohort) | 13.4 years | 57 (n/a) | n/a | Overall: 7.7 months (2–158 months) Infants with SMA type 1: 6.6 months (2–26) CM: 7.8 months (3–121) |
NIV (n = 8) IMV (n = 46) |
• Survival/mortality | • Number of subjects on NIV • NIV success/failure |
| Articles on neuromuscular disease: myotonic dystrophy | ||||||||
| Wood (59), 2017, UK, Germany | Quantitative: observational (cross-sectional) | 4 years | 610 (272/338) | n = 2 | 41.1 years (8 months to 78 years) | NIV (n = 35) | • Number of subjects on NIV* | |
| Articles on central nervous system disease: congenital hypoventilation syndrome | ||||||||
| Garcia Teresa (60), 2017, Spain | Quantitative: observational (cross-sectional) | 3.75 years | 38 (17/21) | n = 8d | 11.35 (5 months to 28.6 years) | NIV (n = 8) | • Hospitalizations • Survival/mortality |
• Number of subjects on NIV • NIV failure/success |
| Hartmann (61), 1994, UK | Quantitative: observational (case series) | n/a | 9 (3/6) | n = 6 | 22 days to 52 months | VNEP (n = 9)f CPAP (n = 3)g |
• Discontinuation of NIV | • Number of subjects on NIV • Benefit of NIV (growth parameters) • NIV success/failure Quality of life |
| Khayat (62), 2017, Canada, USA | Quanitative: observational (control before–after) | 2.7 years | 8 (4/4) | n = 2 | Overall: 10.0 years (8.4–11.6 years) Infants: 1.1 years |
BPAP (n = 8)h | • Number of subjects on NIV • NIV modality |
|
| Noyes (63), 1999, UK, Germany | Qualitative: content analysis | n/a | 7 (3/4) | n = 5 | 66 days to 59 months | VNEP (n = 5) CPAP (n = 1)g IMV (n = 2) |
• Discontinuation of NIV | • Number of subjects on NIV • Benefit of NIV (growth parameters) • Quality of life |
| Ramesh (64), 2008, UK | Quantitative: observational (cross-sectional) | n/a | 15 (5/10) | n = 7 | Early start: 8 weeks (5–26 weeks) Late start: 8 years (1.5–11 years) |
NIV (n = 15) | • Number of subjects on NIV • Benefit of NIV (extubation) • Mask complications |
|
| Tibballs (65), 2003, Australia | Quantitative: observational (case series) | n/a | 4 (2/2) | n = 2 | 6 weeks to 9 years | BPAP (n = 4) | • Changes in respiratory parameters | • Number of subjects on NIV • Benefit of NIV (extubation) • Mask complications |
| Articles on cardio-respiratory disease: congenital heart disease | ||||||||
| Bunn (66), 2004, UK | Quantitative: observational (case series) | n/a | 4 (0/4) | n = 3 | 5–34 months | NIV (n = 4) | • Changes in respiratory parameters • Discontinuation of NIV |
• Number of subjects on NIV |
| Articles on multiple underlying disease conditions | ||||||||
| Adeleye (67), 2016, Canada | Quantitative: observational (cohort) | 5 years | 92 (54/38) | n = 49 | 208.5 ± 101.2 days | NIV (n = 49) | • Number of subjects on NIV • Adherence to NIV |
|
| Amaddeo (3), 2016, France | Quantitative: observational (cohort) | 1 year | 76 (39/37) | n/a | Overall for patient groups: acute: 0.3 year (0.1–13.5) Sub-acute: 0.6 year (0.2–18.2) Chronic: 1.6 years (0.1–19.5) |
CPAP (n = 64) BPAP (n = 12) |
• Number of subjects on NIV • Predictors of NIV requirement |
|
| Bertrand (68), 2006, Chile | Quantitative: observational (cohort) | 10.5 years | 35 (18/17) | n = 9d | 12 months (5 months to 14 years) | CPAP (n = 1) BPAP (n = 8) IMV (n = 26) |
• Hospitalizations • Discontinuation of NIV • Survival/Mortality |
• Number of subjects on NIV |
| Chatwin (7), 2015, UK | Quantitative: observational (cohort) | 18 years | 449 (281/168) | n = 59c | Overall: 10 years (3–15 years) Infants: <1 year (n = 59) |
CPAP (n = 57) BPAP (n = 392) |
• Number of subjects on NIV* | |
| Fauroux (69), 2005, France | Quantitative: observational (cross-sectional) | 0.5 year | 40 (22/18) | n = 16 | Overall: 10.0 years (0.6–18 years) Infant: 1.8 years (0.2–15.3 years)i |
NIV (n = 40) | • Number of subjects on NIV • Adherence to NIV • Mask complications |
|
| Kherani (70), 2016, Canada | Quantitative: observational (cohort) | 23 years | 51 (30/21) | n = 25 | NIPPV: 0.6 year (0.4–0.7 year) IMV: 0.4 year (0.1–0.7 year) |
NIV (n = 25) IMV (n = 26) |
• Changes in respiratory parameters • Discontinuation of NIV • Survival/mortality |
• Number of subjects on NIV |
| Koontz (71), 2003, USA | Quantitative: observational (cohort) | n/a | 20 (n/a) | n = 6 | 1–2 years | BPAP (n = 6) | • Number of subjects on NIV • Adherence to NIV |
|
| Machaalani (72), 2016, Australia | Quantitative: observational (cohort) | 2 years | 99 (63/36) | n = 22 | n/a | CPAP (n = 55) BPAP (n = 44) |
• Number of subjects on NIV* | |
| Markstrom (9), 2008, Sweden | Quantitative: observational (cohort) | 7 years | 18 (11/7) | n = 18 | 4 months (1–12 months) | BPAP (n = 18) | • Changes in respiratory parameters • Discontinuation of NIV |
• Number of subjects on NIV |
| Nathan (73), 2017, Malaysia | Quantitative: observational (cohort) | 13 years | 70 (40/30) | n = 51 | Overall: 12 months CPAP: 6 months (3–12 months) BPAP: 12 months (5–33 months) IMV: 30 (12–57 months) |
CPAP (n = 30) BPAP (n = 30) IMV (n = 10) |
• Discontinuation of NIV • Hospitalizations • Survival/mortality |
• Number of subjects on NIV • Predictors of NIV • NIV modality |
| Ramirez (74), 2012, France | Quantitative: observational (case series) | 18 months | 97 (n/a) | n = 18 | Infants: <2 years (n = 18) | CPAP and BPAP (n/a) | • Number of subjects on NIV* | |
| Zhou (75), 2012, China | Quantitative: observational (cohort) | 2 years | 14 (12/2) | n = 6c | Overall: 50 days to 12 years Infants: <1 year (n = 6) |
CPAP (n = 1) BPAP (n = 13) |
• Number of subjects on NIV* | |
Studies have been classified according to the primary disease category and disease condition reported. Studies with multiple disease categories have been included at the end of the table.
AT, adenotonsillectomy; CPAP, continuous positive airway pressure; BPAP, bi-level positive airway pressure; IMV, invasive mechanical ventilation; n/a, data not available/reported; MDO, mandibular distraction osteogenesis; NIV, non-invasive ventilation; NPA, nasopharyngeal airway; SMA, spinal muscular atrophy; VNEP, negative extra-thoracic pressure ventilation.
*Articles reporting only on the number of subjects using NIV were excluded from synthesis.
aFour patients did not tolerate CPAP.
bFull list of non-surgical and surgical interventions are in the full text of article.
cNumber of patients less than 1 year of age.
dDetermined by the mean/median age of the population during NIV initiation.
eDetermined by age at first respiratory decompensation.
fVNEP failed in two patients.
gCPAP used in conjunction with VNEP.
hCompared intelligent volume-assured pressured support BPAP to traditional BPAP.
iOnly includes infants in the obstructive sleep apnea group.