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German Society of Paediatric Cardiology
Guidelines
Management of a child with cyanosis
L. Kändler (Wittenberg), N. Haas (München),
M. Gorenflo (Heidelberg)
Acknowledged by the executive board of the
German Society of Paediatric Cardiology
on September 6th 2017
Management of a child with cyanosis
Definition: Skin, nail bed (and mucous membranes)
are visible blue
• Greek: kyanos = blue
• Visible: > 3 g/dl desoxygenated hemoglobin in the arterial blood or
> 4-5 g/dl desoxygenated hemoglobin in the skin veins respectively
• Cyanosis = mostly implies hypoxia
• Confirm by arterial blood gas analysis or pulse oximetry
• New born: cyanosis often visible only in O2 -saturation <= 80%
Management of a child with cyanosis
O'Donnell CP et al. Arch Dis Child Fetal Neonatal 2007;92:F465-7
Fotos: left: Ермоленко Елена Евгеньевна; right: GNU Free
Documentation License
Clinical impression
may be misleading! Video clips of 20 neonates:
• starting with arriving on the
resuscitating trolley
• ending when SpO2 was >= 90%
for > 10 sec
• medical (n=13) and nursing staff
(n=14) had to indicate if and
when the infant looked pink
• enormous range !!!
Management of a child with cyanosis
Cyanosis or Hypoxia ?!
Met-Hb: Klaire Johnson and
Brooke Bledsoe as blue people
Foto: Eli Blevins
Fetal Hb
Management of a child with cyanosis
Familial congenital Methemoglobinemia
Reproduced with permission
from Trost, C.,
The blue people of Troublesome Creek.
Science 82,
November, pp. 35-39, 1982. Illustration
by Walt Spitzmiller.
„...however, they are
more blue than sick.“
Management of a child with cyanosis
History
• Fetal echocardiogram, gestational diabetes, polyhydramnion?
• Premature rupture of membranes, nonsteroidal anti-inflammatory drugs,
anaesthesia/analgesia of the mother?
• Cyanosis immediately after birth or free interval
(„late onset cyanosis“ e.g. in Tetralogy of Fallot )?
• Cyanosis during feeding (tracheoesophageal fistula, vascular rings)
• Cyanosis in supine position/ during sleep (reflux: 2-4th month)
Management of a child with cyanosis
Physical examination
• Peripheral - central – ((reverse)) differential cyanosis
• Eupnoea - tachypnea – dyspnea - hypoventilation - apnea
• Auscultation
CAVE: heart murmur may be absent or develop late
• Palpation of peripheral pulses
CAVE: patent arterial duct
• Pulse oximetry at all 4 extremities
Management of a child with cyanosis
Differential diagnosis of cyanosis
Organ system Examples
Cardiac Transposition of the great arteries, univentricular heart,
Ebstein anomaly, hypoplastic left heart syndrome, Eisenmenger
syndrome
Vascular Anomalies of the aortic arch, pulmonary sling, pulmonary
arterio-venous fistula, persistent pulmonary hypertension of
new-born
Respiratory Hyaline membrane disease, meconium aspiration, choanal
atresia and other abnormalities, spontaneous pneumothorax,
asthma exacerbation
Gastrointestinal/
metabolic
Tracheo-esophageal fistula, gastro-esophageal reflux, infants of
diabetic mothers, methaemoglobinaemia
Management of a child with cyanosis
Differential diagnosis of cyanosis
Organ system Examples
neurological seizures, cerebral infarction, breath holding spells
ALTE
(apparent life
threatening event)
cyanosis as a risk factor
miscellaneous new-borns at high altitude
sepsis, hypoglycemia of the new-born
“loss of breath” after tonsillectomy
HIV (children aged 2-18 month)
Management of a child with cyanosis
Management of a child with cyanosis
Response to oxygen supply:
Disease Pathophysiology O2-Saturation
Univentricular heart, TGA central mixing/
(transposition of the great arteries) parallel circuits
Cave: ductual closure
Truncus arteriosus, TAPVC increased pulmonary
(Total anomalous pulmonary blood flow
venous connection)
PPHN with patent arterial duct right to left shunt
and differential cyanosis reverses to left to right shunt
Pulmonary problem benefits from oxygen
Severe pneumonia opening of intrapulmonary
right to left shunts
Management of a child with cyanosis
nach Lindinger A, Dähnert I, Riede FT. Stellungnahme der Deutschen Gesellschaft für Pädiatrische Kardiologie
(DGPK) vom 10.07.2013: Pulsoxymetriescreening zur Erfassung von kritischen angeborenen Herzfehlern im
Neugeborenenalter. In Vorbereitung des GBA-Beschlusses vom 24.11.2016.
Management of a child with cyanosis
The „diagnostic gap“ in critical congenital heart disease
n = 4
Effectiveness of neonatal
pulse oximetry screening
for detection of critical congenital
heart disease in daily
clinical routine—results
from a prospective multicenter
Study
Frank Thomas Riede & Cornelia Wörner &
Ingo Dähnert & Andreas Möckel & Martin Kostelka &
Peter Schneider
Eur J Pediatr (2010) 169:975–981
Management of a child with cyanosis
Severe coarctation of the aorta: palpable femoral pulses when duct
is still patent
Endangered by the “diagnostic gap”
Management of a child with cyanosis
Newborn, 2th day of life
75/42 (63) 74/39 (60)
100% 100%
98% 100%
66/50(57) 67/46(57)
Management of a child with cyanosis
Timely diagnosis of coarctation
• Loud systolic murmur at the time of routine
physical examination on day 2
• tricuspid insufficiency because of
pulmonary hypertension
Management of a child with cyanosis
The „diagnostic gap“ in critical congenital heart disease
CAVE! : * reduced lengths of stay in maternity units
* rooming with the mothers under more „domestic
light conditions“
* postnatal examination routines have altered:
e.g. stop of examining femoral pulses routinely
* training level of the staff
Highest detection rate provided by neonatal physical examination
plus pulse oximetry
Anne de Wahl-Granelli et al. BMJ 2009
Mahle WT et al. Pediatrics 2009
Meberg A et al. J Pediatr 2008
Mellander M et al. Acta Pediatr 2006
Management of a child with cyanosis
Cost benefit analysis of pulse oximetry screening Knowles R1, Griebsch I, Dezateux C, Brown J, Bull C, Wren C. Newborn screening for
congenital heart defects: a systematic review and cost-effectiveness analysis.
Health Technol Assess. 2005 Nov;9(44):1-152, iii-iv.
Sensitivity false-positive costs (per 100.000 newborns)
Physical 32% 0,5% 300.000 £
Pulse oximetry 68% 1,3 % 480.000 £
Echocardiography 69% 5,4% 3.540.000 £
Management of a child with cyanosis
Further reduction of the diagnostic gap ? Oxygen saturation pre- and postductal
= right hand and foot < 95%
+ plus difference between hand und foot > 3%
Sensitivity of pulse oximetry = 98,5%
Anne de Wahl Granelli et al. Acta Paediatr. 2005;94: 1590-1596
Screening for duct-dependent congenital heart disease with pulse oximetry: a critical evaluation of
strategies to maximize sensitivity.
n= 66 new born with critical CHD, n=200 controls
Thank you very much for your attention!!
L.Kändler, Oil on canvas, 30 x 35, 2003