Management of a child with cyanosis

Transcription

1 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

2 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%

3 O'Donnell CP et al. Arch Dis Child Fetal Neonatal 2007;92:F465-7 Fotos: left: Ермоленко Елена Евгеньевна; right: GNU Free Documentation License Management of a child with cyanosis 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!!!

4 Cyanosis or Hypoxia?! Fetal Hb Met-Hb: Klaire Johnson and Brooke Bledsoe as blue people Foto: Eli Blevins

5 Familial congenital Methemoglobinemia Reproduced with permission from Trost, C., The blue people of Troublesome Creek. Science 82, November, pp , Illustration by Walt Spitzmiller....however, they are more blue than sick.

6 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)

7 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

8 Differential diagnosis of cyanosis Organ system Examples Cardiac Vascular Respiratory Gastrointestinal/ metabolic Transposition of the great arteries, univentricular heart, Ebstein anomaly, hypoplastic left heart syndrome, Eisenmenger syndrome Anomalies of the aortic arch, pulmonary sling, pulmonary arterio-venous fistula, persistent pulmonary hypertension of new-born Hyaline membrane disease, meconium aspiration, choanal atresia and other abnormalities, spontaneous pneumothorax, asthma exacerbation Tracheo-esophageal fistula, gastro-esophageal reflux, infants of diabetic mothers, methaemoglobinaemia

9 Differential diagnosis of cyanosis Organ system Examples neurological seizures, cerebral infarction, breath holding spells ALTE (apparent life threatening event) miscellaneous cyanosis as a risk factor new-borns at high altitude sepsis, hypoglycemia of the new-born loss of breath after tonsillectomy HIV (children aged 2-18 month)

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11 Response to oxygen supply: Disease Pathophysiology O2-Saturation Univentricular heart, TGA (transposition of the great arteries) Truncus arteriosus, TAPVC (Total anomalous pulmonary venous connection) PPHN with patent arterial duct and differential cyanosis Pulmonary problem Severe pneumonia central mixing/ parallel circuits Cave: ductual closure increased pulmonary blood flow right to left shunt reverses to left to right shunt benefits from oxygen opening of intrapulmonary right to left shunts

12 nach Lindinger A, Dähnert I, Riede FT. Stellungnahme der Deutschen Gesellschaft für Pädiatrische Kardiologie (DGPK) vom : Pulsoxymetriescreening zur Erfassung von kritischen angeborenen Herzfehlern im Neugeborenenalter. In Vorbereitung des GBA-Beschlusses vom

13 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:

14 Endangered by the diagnostic gap Severe coarctation of the aorta: palpable femoral pulses when duct is still patent

15 Newborn, 2 th day of life 75/42 (63) 74/39 (60) 100% 100% 98% 100% 66/50(57) 67/46(57)

16 Timely diagnosis of coarctation Loud systolic murmur at the time of routine physical examination on day 2 tricuspid insufficiency because of pulmonary hypertension

17 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

18 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 Nov;9(44):1-152, iii-iv. Sensitivity false-positive costs (per newborns) Physical 32% 0,5% Pulse oximetry 68% 1,3 % Echocardiography 69% 5,4%

19 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: 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

20 Thank you very much for your attention!! L.Kändler, Oil on canvas, 30 x 35, 2003