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