1. APPROACH TO CYANOSIS

2. Definition
Bluish discolouration of skin or mucous membrane caused by excess amounts of reduced hemoglobin or abnormal hemoglobin
4gm of reduced Hb in capillaries required for cyanosis to be apparent

3. Mechanism caused by absolute increase in reduced Hb,higher the Hb – greater tendancy towards cyanosis

4. In severe anemia , greater systemic arterial desaturation required for cyanosis to be evident
In polycythemia even lesser systemic arterial oxygen saturation may result in clinical cyanosis
If fetal Hb is high, tissue hypoxia may occur even if cyanosis is mild( arterial PaO2 low)

6. central
peripheral
CAUSE
ARTERIAL BLOOD DESATURATION OR ABNORMAL Hb
CUTANEOUS VASOCONSTRICTION DUE TO LOWCO
CONDITIONS
Seen in R-L shunt, impaired pulmonary function, abnormal Hb
exposure to cold air or water and abnormally greater extraction ofO2 from normally saturated blood
SITES
conjunctiva,palate,tongue,inner side of lips& cheeks
limited to ears,nose,cheeks outer side of lips hands feet&digits
certainly central if associated with clubbing and polycythemia,
clubbing is absent
probably central if it deepens on effort

7. DUCT-INDEPENDENT MIXING LESIONS TGA, TAPVC,TA DUCT-DEPENDANT PBF
CARDIOVASCULAR
DUCT-INDEPENDENT MIXING LESIONS
TGA, TAPVC,TA
DUCT-DEPENDANT PBF
TOF,EBSTEINS,TricusidAtre
DUCT-Dependant SBF
HLHS,IAA,CoA,Critical AS
L-RSHUNT &PUL EDEMA
SV States
PRIMARY LUNG DISEASE
AIRWAY OBSTRUCTION
EXTRINSIC LUNG COMPRESSION
PULMONARY AV MALFORMATION
PPHN
CNS DYSFUNCTION
HEMATOLOGIC
MISCELLANEOUS
HYPOGLYCEMIA,
METABOLICACIDOSIS
SEPSIS,
HYPOTHERMIA,
SHOCK

8. Diagnostic ladder Echo Simple lab investigation Clinical evaluation
History
Physical examination
Simple lab investigation
PO, CXray & ECG
Hb conc &peripheral blood filim
Echo
Invasive cardiac evaluation

9. DIABETES- TTN,RDS,HYPOGLYCEMIA ASTHMA -TTN POLYHYDRAMNIOS - TEF
MATERNAL HISTORY
DIABETES- TTN,RDS,HYPOGLYCEMIA
ASTHMA -TTN
POLYHYDRAMNIOS - TEF
PIH – IUGR,POLYCYTHEMIA,HYPOGLYCEMIA
LABOUR& DELIVERY
PROM –SEPSIS,PNEUMONIA
CHORIOAMNIONITIS- SEPSIS
C-SECTION- TTN,RDS,PPHN
NEWBORN
ONSET AT BIRTH- TTN,RDS,MAS,CDH,
ONSET –HRS AFTER BIRTH- CCHD,aspiration,TEF

10. CLUES BASED ON ONSET 1st week > 1 week D TGA TOF TGA
Pulmonary Atresia Admixture lesions
Tricuspid atresia TAPVC
Ebstein SV
Critical PS DORV
Truncus

11. Examined in neutral thermal enviornment Away from blue phototherapy lights Asses capillary refill time- <2 sec Barrel shaped chest –post term-MAS Bell shaped thorax – neurologic abnormalities Scaphoid abdomen-CDH Look for nasal flaring,grunting & retractions

12. Palpate brachial & femoral pulses BP- all four extrimities SBP-gradient quite specific for arch abnormality Not sensitive S2- split in 80% by 48 hours Differential cyanosis

13. CCHD in Newborns: Clues based on presentation
Cyanosis
No Resp Distress
Cyanosis +
Resp Distress
Shock
Differential
cyanosis
TGA
DDPC
TAPVC
obstructed
DDSC

14. CCHD in Newborns: Clues Based on S2 split
single
normal
fixed
DDPC
TGA
DDSC
Excludes
Cardiac
cause
TAPVC

15. CCHD in newborns: Clues based on Murmurs
Murmurs have poor sensitivity( < 50%)
No Murmur
TAPVC with obstruction
DDPC( closed PDA)
ESM
TGA, DDPC, DDSC
Continuous murmurs (are)
DDPC with collaterals
Diastolic murmur
(to & fro)
TOF- absent pulmonary
valve

16. cardiac
respiratory
HEART RATE
FASTER
FAST
RESPIRATORY RATE
GRUNT
ABSENT
PRESENT
MURMUR
PROMINENT
CAN BE PRESENT
CHF
LESSLIKELY
ABNORMAL PULSE
YES NO
RESPONSE TO O2
NOT MUCH
GOOD

17. Pulse oximetry
Standard of care for all infants with respiratory distress & cyanosis Accurate& reliable method of monitoring o2 saturation in infants noninvasively Pulse oximeter probes on R hand & lower extremity Aim for 02 saturation of 90-95% by pulse oximetry PPHN- suspected –aim for higher o2 saturation

18. HYPEROXIA TEST ADMINISTER 100% O2 FOR 15 MINUTES
ASSES O2 OF UPPER LIMB LOWER LIMB
ABG YES
TCMO YES
PO NO

19. HYPEROXIA TEST
GIVE 100% O2 ASSES PO2 PO2>200 PO2<150 NO CCHD LIKELY CCHD PASS FAIL ?CCHD WITH PBF OR PPHN

20. CARDIOMEGALY NO CARDIOMEGALY PULMONARY VASCULARITY EBSTEINS
PAO2 <50MM
C X RAY
CARDIOMEGALY NO CARDIOMEGALY
PULMONARY VASCULARITY
EBSTEINS
PBF PUL EDEMA PBF
TGA + IVS TAPVR With OBSTRN TA with PA orPS
PA WITH IVS
CRITICAL PS
TOF & TOF + PA

21. Those with decreased PBF &normal or slightly increased heart size differentiated by there QRS axis on ECG & MURMUR + or – TA with PS or PA – SUPERIOR QRS AXIS ( 0 to-90) Critical PS & PA with IVS- 0 to 90 degree Differentiated by loud pul ejection murmur TOF & TOF with PA - QRS 90 to 180 Pulmonary continuous murmur Stenosis murmur

22. Prostaglandin (PGE1) Infusion
Neonates –fail hyperoxia test High Signs& symptoms of CHD or likelihood Present in shock within 1st 3 wk of life of CHD PGE1 administration –open ductus arteriosus Depending on lesion - PBF or SBF or improves Intercirculatory mixing- improves hypoxemia &metabolic acidosis Neonate with shock or CHF in 1st few weeks of life - ductdependant SBF untilproven otherwise

23. mild/no cyanosis + abnormal pulses
PG sensitive lesions:
Cyanosis + murmur or
mild/no cyanosis + abnormal pulses
Can be withheld in a relatively stable child
( SaO2 > 70%; no acidosis)
Target SaO2 >80%; pO2 around 45-50, normal pH
Once diagnosis is confirmed, it is ideal to start PGE1 before transport.
WORSENING AFTER PGE1 – obstruction to blood flow out of pulmonary veins

24. Always given as continous IV infusion.
Start at μg/kg/min, can be reduced to µg/kg/min once duct is opened( ^ SaO2)
Available as 500 μg vial
Trade name: Alpostin/Prostin
Cost: Rs 5000/- per ampoule
One vial will last 2-3 days for a 3Kg baby

25. Efficacy  with  age, less effective after 2 weeks of life, not effective after 4 weeks
Adverse reactions more common in premature& LBW infants
Apnea –typically in 1st few hrs ,tachycardia, bradycardia, fever, NEC, seizures, thrombocytopenia,
Continous cardiorespiratory monitoring

26. PGE1 – peripheral vasodilation – hypotension& cutaneous flushing
Separate IV line should be secured
Hypotension treated by
10-20ml/kg bolus of NS,RL,5%albumin
Remeasure ABG,reasses capillary refill& vitalsigns within 15 to 30 min of starting PGE1
infusion

27. Principles of managment
Intial stabilisation – airway management reliable venous access – umblical vein Arterial line to monitor BP,acid-base,o2 Volume resucitation,inotropic support & correction of metabolic acidosis Blood glucose & sepsis workup-cyanosis+circ collpse

28. Non invasive delineation of anatomic defect- ECHO Evaluation & treatment of additional organ system-pulmonary,renal,hepatic&CNS Evaluation of additional congenital defects Genetic evaluation – if indicated Cardiac Cathetrisation Surgical managment

29. CCHD is an important differential diagnosis in neonate presenting with cyanosis after birth.
Clinical evaluation with CXR and Hyperoxia test excludes CHD in most cases.
Echocardiography recommended in all doubtful cases.
Prior stabilization and a monitored transport to tertiary center ensures a optimal pre-operative state.
Early intervention with very encouraging results is realistic for most forms of critical CHD in newborns

31. r
CCHD derived from heterogeneous group of conditions May have pulmonic stenosis ,PAHor NL pulm pressure with out PS PBF may be NL,INCREASED or DECREASED Decreased PBF may be due to Pulmonic stenosis or PAH Anomalies where free mixing of systemic and venous blood occurs severity of cyanosis determined by pulmonary blood flow Thymus regresses very fast in cyanotic patients

32. Without PS NO VSD With VSD PULM VENOUS OBSTRN PBF PA pressure
CCHD
With PS
NO VSD
With VSD
Without PS
PA pressure NL
PA pressure
elevated
PBF
PULM VENOUS OBSTRN

33. This classification result in six sub group of cyanotic patients PS without VSD PS + large VSD Increased PBF with or without PAH(transposition physiology PAH withdecreased PBF(Eisenmengerphisiology) PAHdue to pul venous obstruction NL or MILD elevated pul pressure without PS

34. (cyanosis due to R toL shunt at atrial level& cardiomegaly)
PS without VSD
(cyanosis due to R toL shunt at atrial level& cardiomegaly)
Triad-cyanosis,cardiomegaly,ischemic lung fields on Cxray
Dominant a in JVP
Cardiomegaly
Parasternalimpulse
Widely split 2nd HS p2 late &soft
3rd &4th HS
Pulm ejection systolic murmur,TR murmur
Severe or critical pure PS with failing RV,Ebstein’s

35. Mild parasternal impulse Systolic thrill uncommon
PS with VSD (Fallot’s physiology)
Prominent a wave in jvp
NL heart size
Mild parasternal impulse
Systolic thrill uncommon
Single 2nd sound(widely split with inaudible pulmonic sound)
ESM
Clear diastolic period
Ischemic lungs in xray without cardiomegaly

36. D/D of fallots physiology Fallot’stetrology(commonest >2yrs ) TGA TRICUSPID ATRESIA SV DORV correctedTGA AVCD If no rvh on ecg;posssibilities-rv hypoplastic &small,rv absent,PA not connected to RV Tricuspid atresia,hypoplasticRV with or without straddling TV, & single ventricle

37. Increased pulmonary blood flow with or without PAH(transposition physiology)
Symptomatic in Neonate
Cyanosis-mild to severe
Failure to thrive& gain weight
CCF
Cardiomegaly –in2-3 wks of life
2nd sound single,s3 gallop,insignificant systolic murmur
Cardiomegaly with Incrs pulm vasculatureon xray& thymicshadow absent

38. Anomalies with cyanosis and increased PBF TGA DORV without PS TA with Incrs PBF (largeVSD+ NO PS) Persistent Truncus SV without PS TAPVC

39. Cyanosis with PAH and Diminished PBF (eisenmenger- defnd as nonreactive PAH resulting in a R to L shunt at atrial,ventricular or great artery level)
Characteristics
H/O frequent chest infections in infancy
Cyanosis present from birth and appear late
Jvp-prominent a wave
No cardiomegaly or thrill(except when shunt at atrial level)
No PSH
Constant EC of PAH

40. 2nd sound is palpable,pulmonary component accentuated
Systolic murmur in pulmonary area is insignificant or absent
Pulmonary and/or TR murmurs may be present

41. EISENMENGER SYNDROME-DIFFERENTIATION
ASD
VSD
PDA
CYANOSIS
UNIFORM
DIFFERENTIAL
CARDIOMEGALY
PRESENT
ABSENT
PARASTERNAL IMPULSE
HEAVING
MILD
2nd SOUND
WIDE FIXED SPLIT
SINGLE
NORMALLY SPLIT TR
COMMON
RARE
ASC AORTA IN C Xray
NORMAL
LARGE

42. PAH-DIFFERENTIATION HYPERKINETIC OBSTRUCTIVE HEART SIZE LARGE
NORMAL(except ASD)
PARASTERNAL IMPULSE
FORCIBLE &HEAVING-ASD, MILD IN VSD&PDA
CLICK OF PAH
ABSENT
PRESENT
2nd SOUND(P2accentuated in both)
ASD-WIDE&FIXED,VSD-WIDE&VARIABLE,PDA-PARADOXICALLY SPLIT
ASD-WIDEFIXED
VSD-SINGLE
PDA-NORMAL
Shunt murmur
LOUD
SHORT OR ABSENT
Flow murmur

43. Patients with PAH due to Pulmonary Venous hypertension(HLHS&TAPVC with obstruction) Generally present in neonatal period Severe cyanosis,CHF,S3 gallop,no cardiomegaly,absence of significant murmurs, Cxray –NL sized heart with severe PVH- causing GROUND GLASS appearance 2d echo- mitralatresia,aortic atresia,pulmonary venous obstruction, hypoplastic LV

44. Cyanosis without PS & PA pressure normal Heterogeneous group of anomalies TAPVC- features of 2 ASD but with cyanosis,figure of 8 Single atrium-mostly associated with polysplenia SVC entering LA Pulmonary AV fistula

45. Cyanosis+diagnostic approach Dextrocardia
Levocardia
With visceral
situsinversus
complex
Poor femoral pulse&
Large brachio-poplipteal
Systolic pressure gradient
Booming p2
Cardiomegaly
Prominent pulmonary conus
Peripheral vascular
pruning
Large&relatively silent heart
Huge RA oligemic lung fields
Giant p wave
Prescence of Howell-jolly bodies
Syndrome of
Levocardia with
Visceral situs
inversus
Coarctation
syndrome
Eisenmeinger syndrome
Ebstein’s anomaly
Asplenia

46. Abnormal rhythm& CHD
L- TGA Heart Block ; SVT
Ebstein - SVT

47. TGA TAPVC DORV Cyanosis&RVH Plethora Oligemia TOF PA PS+ASD&/OR VSD
NL axis/RAD
Plethora
Oligemia
TGA
TAPVC DORV
TOF PA PS+ASD&/OR VSD
PS+TGA/DORV/DOLV