Cyanosis diagnosis and management

1. Evaluation of Cyanotic child &
Management of Cyanotic spell
DR DHANESH KUMAR

2. CYANOSIS
• Definition
• Clinically determined by-
History & Examination
Saturation probe!!
 Types : Central, Peripheral, Abnormal pigments, mixed or
differential
 Cause- Cardiac, pulmonary , hematologic , Infectious or High
altitude ??
The cyanotic neonates: By Suzanne Roberts , D. O ., MPH, and Jahn Avarello , M.D.

3. Clinical Diagnosis
% of arterial blood that is desaturated
Hb concentration!!
If art O2 saturation is 60%
– Cyanosis is detectable if Hb >12.5gm/dl !
– But not if Hb <10gm/dl !
i.e. 4gm/dl is insufficient for detection of cyanosis !
• Cyanosis is recognizable at a higher arterial
oxygen saturation in patients with polycythemia
and at a lower arterial oxygen saturation in
patients with anemia

4. Diagnosis
Hyperoxia Test: Confirm
• Place infant on 100% O2 hood for 10 min.
• PaO2 >100 mmHg = Parenchymal lung disease
• PaO2=50-100 mmHg=Parenchymal lung
disease or cardiovascular disease
• PaO2 <50 mmHg=Fixed R-L shunt
– Cyanotic congenital heart disease of PPHN

5. Unusual causes of cyanosis
Without murmurs!
Surviving to adolescent/ adult life
Left SVC to LA
IVC to LA
Rt.SVC to LA
Pulmonary AV Fistulae(Osler Rendu Weber
syndrome)

7. Approach to a child with cyanotic CHD
• History:
• H/o. cyanotic spell(TOF physiology)
• Cyanosis increased during defecation, feeding
• H/o squatting
• Easy fatigability
• Failure to thrive
• Syncope-TOF
• Hemoptysis: Eisenmenger’s
• Convulsions: Cerebral abscess
• Cough, breathlessness & repeated chest infection due
to increased PBF-TAPVC, TGA, Truncus arteriosus

9. Some points in clinical examination &
History
• Prenatal History
• Consanguinity
• Age of Parents(esp. mother)
Maternal medication during pregnency
• Sod. Valproate -TOF,VSD
• Lithium-ASD, Tricuspid atresia, Ebstein’s
• Marijuana- Ebstein’s
• Clomiphene -TOF
• Sex hormones-VSD,TGA,TOF

10. Approach to a child with cyanotic CHD
Cyanosis at birth
• TGA with intact IVS
• HLH Syndrome
• Hypoplastic RV with
pulmonary atresia
• Truncus arteriosus
• Obstructive TAPVC
• TOF with pulmonary
atresia
Cyanosis in 1st week
• Pulmonary atresia
• Tricuspid atresia
• HLH Syndrome
• Ebstein’s anomalies
Cyanosis after 1 month
• TOF
• TGA
• TAPVC

11. Sex of Patient
Males-more common
• D-TGA
• Hypoplastic left heart
• Single ventricle
Females-more Common
• OS-ASD
• PDA
Equal in both sexes
• Ebstein’s anomaly
• Pulmonary atresia
• Tricuspid atresia
• Truncus atreriosus
• TOF
• Congenital venacaval to
LA communication

12. • Marfan’s and Down syndrome: TOF
• Noonan & Maternal rubella syndrome: TOF
• Cat cry syndrome: Tricuspid Artesia
• Di George syndrome: Truncus arteriosus
(hypoplastic mandible, defective ears and short
philtrum)
• Anomalies of 16-18 chromosomes :DORV
• Eisenmenger’s syndrome: Differential clubbing
only in toes, shortstature, kyphoscoliosis,
arthropathy, dental anomalies, growth retardation

13. Approach to a child with cyanotic CHD
Pulse :
Collapsing:
o Truncus arteriosus
o TOF with collaterals/AR
o AV Malformation
Decreased L carotid and left brachial pulses:
o HLHS

14. Jugular Examination
• Enlarged
– Tricuspid Artesia
– Hypo plastic left heart
– TAPVR
– TGA with ↑PBF
– DORV with ↑PBF
– Truncus arteriosus
• Normal jugulars
– Fallot’s tetrology
– TGA with PS
– DORV with PS

15. Precordium Examination
Normal
• TOF
• DORV
• Pulmonary atresia with
intact ventricular
septum
• Tricuspid atresia
Precordial Bulge
• TAPVC
• TGA
• Hypoplastic Lt. Ventricle
• PS with intact IVS

16. Approach to a child with cyanotic CHD
• Splitting of S1: Epstein's anomaly
• Loud P2:TAPVC,DORV with increased PBF,
Eisenmenger syndrome
• Single S2:TOF, TOF With PS,DORV with PS,
Tricuspid atresia, Truncus arteriosus
• Muffled S2:Ebstein’s anomaly
• Continuous murmur: TOF with collateral,
Truncus arteriosus

17. Chest X-ray
• Heart size
• Cardiac silhouette
• Right aortic arch?
• Midline Liver?

18. Radiology
• Boot shape heart: TOF
• Enlarged heart: TOF with collaterals,
associated anemia or hypertension
• Concavity in pulm. Artery area: PS
• Rt. Sided aortic arch:25% cases of TOF
• Rib notching: TOF with collaterals
• Egg on side appearance: TGA
• Figure of 8 appearance: TAPVC

19. ECG
Cyanotic
Defects
Increased
PBF
LVH or BVH
PDA
SV
TGA+VSD
RVH
TGA
TAPVR
HLHS
Decreased
PBF
BVH
TGA+PS
PDA+Hypoplastic
PA
SV+PS
LVH
Tricuspid atresia
Pulmonary
atresia+Hypoplast
ic RV
RVH
TOF
PVOD
(2ry to L-R Shunt)
Ebestein’S
anomaly
(RBBB)

20. Other non invasive tests
• Echocardiography
• Holter monitoring
Kadle et al. Estimating PG by auscultation :How tecnology (echocardiography)
can improve clinical skills. World J Cardiol 2017

21. Invasive methods
• Cardiac catheterization
• Angiography
• Contrast CT
• 3-D Printing
Temel et al. Prevalence and characteristics of coronary artery anomalies in children with
congenital heart disease diagnosed with coronary angiography. Turk Kardiyol Dern Ars 2017

22. Keys to clinical diagnosis
• Work in order
• Pulses?
• Colour -cyanosis, pallor, polycythemia
• Inspect for chest form and pulsations
• Palpate to determine which ventricle?
• Forget the murmur!!
• Listen first to S1 then S2
• Can you split the S2??
• Then concentrate on components
• Finally the murmurs- systolic, ejection or pan systolic
• Is there a diastolic murmur??

23. Cyanotic Spell
• Progressive increase in
rate & depth of
respiration &
culminates in
paroxysmal
hyperapnea, deepening
of cyanosis, limpness,
syncope & ocasionally
convulsion or death.

24. Cyanotic Spell
Cause:
• TOF
• DORV,VSD,PS
• VSD,PS
• TGA,VSD,PS
• ASD,PS
• Tricuspid Atresia 1b/IIb
Triggering factors
• Spontaneous
• Crying
• Feeding
• Bowel movement
• Sudden wake up

25. Cyanotic Spell
• Medical emergency
• Early hours
• Mechanism: Exact cause-Not Known
• Wood’s Theory: Local intracardiac production of
catecholamine's increased due to stress-which
increase infundibular spasm leading to more R to
L shunt
• Vulnerable resp. centre(Guntheroth & Morgan)
• Paroxysmal attack of arrhythmias: Atrial
Tachycardia increased R-L shunt(Young)

26. Cyanotic Spell
Supportive Treatment:
1.Knee chest position
2.O2 inhalation
3.I.V access
4.Sodium bicarbonate(1-2meq/Kg/dose) for
acidosis

28. • Intubation and ventilation
• Emergency BT Shunt
• If ductal dependent-PGEI/Duct stenting
• If TGA=Baloon atrial septostomy

29. Prevention
• Propronalol (oral) :1-4mg/Kg
• Iron supplement:1mg/kg/day
• Avoid precipitating cause –Vasodilators
• Treat URTI promptly
• Blood letting
• Plan surgical management

30. Polycythemia-Management
• 1.Avoid dehydration
• 2.Iron therapy
• 3.Phlebotomy-if Hct. > 65-70%

31. THANK YOU !