APPROACH TO CYANOSIS

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

Mechanism
caused by absolute increase in reduced Hb,higher the Hb greater tendancy towards cyanosis
25 20

20 15

15

TOTAL Hb 10 5 5 5 5 RED-Hb

0 NORMAL POLYCYTHEMIA ANEMIA

 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)

central

peripheral

CAUSE

ARTERIAL BLOOD DESATURATION OR ABNORMAL Hb

Seen in R-L shunt, impaired pulmonary function, abnormal Hb

CUTANEOUS VASOCONSTRICTION DUE TO LOWCO

exposure to cold air or water and abnormally greater extraction ofO2 from normally saturated blood

CONDITIONS

SITES

conjunctiva,palate,tongue,i limited to ears,nose,cheeks nner side of lips& cheeks outer side of lips hands feet&digits certainly central if associated with clubbing and polycythemia, probably central if it deepens on effort clubbing is absent

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

Diagnostic ladder
Clinical evaluation

History

Physical examination

Simple lab investigation PO, CXray & ECG

Hb conc &peripheral blood filim

Echo

Invasive cardiac evaluation

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

CLUES BASED ON ONSET

1st week D TGA Pulmonary Atresia > 1 week TOF TGA Admixture lesions

Tricuspid atresia
Ebstein Critical PS

TAPVC
SV DORV Truncus

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

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

CCHD in Newborns:
Clues based on presentation

Cyanosis No Resp Distress

Cyanosis + Resp Distress

Shock Differential cyanosis

TGA DDPC

TAPVC obstructed

DDSC

CCHD in Newborns:
Clues Based on S2 split
S2

single fixed

normal

DDPC TGA DDSC

TAPVC

Excludes Cardiac cause

CCHD in newborns:
Clues based on Murmurs

Murmurs have poor sensitivity( < 50%)

No Murmur ESM TAPVC with obstruction DDPC( closed PDA) TGA, DDPC, DDSC

Continuous murmurs DDPC with collaterals (are) Diastolic murmur (to & fro) TOF- absent pulmonary valve

cardiac HEART RATE RESPIRATORY RATE GRUNT MURMUR CHF ABNORMAL PULSE RESPONSE TO O2 FASTER FAST ABSENT PROMINENT PRESENT YES NOT MUCH

respiratory FAST FASTER PRESENT CAN BE PRESENT LESSLIKELY NO GOOD

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

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

HYPEROXIA TEST
GIVE 100% O2 ASSES PO2 PO2>200 NO CCHD PO2<150 LIKELY CCHD

PASS

FAIL
150-200 ?CCHD WITH PBF OR PPHN

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

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

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

 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

 Always given as continous IV infusion. Start at 0.05-0.1g/kg/min, can be reduced to 0.005 - 0.01g/kg/min once duct is opened( ^ SaO2) Available as 500 g vial Trade name: Alpostin/Prostin Cost: Rs 5000/- per ampoule One vial will last 2-3 days for a 3Kg baby

 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

 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

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

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

 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

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

CCHD

With PS

With out PS
With VSD

NO VSD

PA pressu re NL

PA pressure

elevated

PBF

PBF

PULM VENOUS OBSTRN

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

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,Ebsteins

PS with VSD (Fallots 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

D/D of fallots physiology Fallotstetrology(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

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

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

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

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

EISENMENGER SYNDROME-DIFFERENTIATION
ASD CYANOSIS CARDIOMEGALY PARASTERNAL IMPULSE 2nd SOUND UNIFORM PRESENT HEAVING WIDE FIXED SPLIT VSD UNIFORM ABSENT MILD SINGLE PDA DIFFERENTIAL ABSENT MILD NORMALLY SPLIT

TR ASC AORTA IN C Xray

COMMON NORMAL

RARE NORMAL

RARE LARGE

PAH-DIFFERENTIATION
HYPERKINETIC HEART SIZE LARGE OBSTRUCTIVE NORMAL(except ASD)

PARASTERNAL IMPULSE
CLICK OF PAH 2nd SOUND(P2accentuated in both) Shunt murmur Flow murmur

HYPERKINETIC
ABSENT ASD-WIDE&FIXED,VSDWIDE&VARIABLE,PDAPARADOXICALLY SPLIT LOUD PRESENT

FORCIBLE &HEAVING-ASD, MILD IN VSD&PDA

PRESENT ASD-WIDEFIXED VSD-SINGLE PDA-NORMAL SHORT OR ABSENT ABSENT

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 PVHcausing GROUND GLASS appearance 2d echo- mitralatresia,aortic atresia,pulmonary venous obstruction, hypoplastic LV

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

Cyanosis +diagnos tic approac h

Booming p2

Cardiomegaly

Large&r elativel y silent heart Huge RA oligemi c lung fields Giant p wave

Dex troc ardi a

Dext rocar dia

Levoc ardia With viscer al situsin versus

Syndrome of Levocardi a with Visceral situs inversus

Prominent pulmonary conus Poor femoral pulse& Large brachiopoplipteal Systolic pressure gradient Peripheral vascular pruning

Prescence of Howelljolly bodies

Asplenia syndrome

com plex

Coarctation syndrome

Ebsteins anomaly Eisenmein ger syndrome

Abnormal rhythm& CHD

L- TGA - Heart Block ; SVT

Ebstein - SVT

Cyanosis&RVH

NL axis/RAD

Plethora

Oligemia

TGA TAPVC DORV

TOF PA PS+ASD&/OR VSD PS+TGA/DORV/ DOLV