Basic Approach to cyanosis in infancy

Cardiology

Red Cross Children's Hospital

“You do not really understand something unless you can explain it to your grandmother.”

Dr. Albert Einstein

Most common reason for referral to Most common reason for referral to cardiologist in the 1cardiologist in the 1stst years of life: years of life: Heart MurmurHeart Murmur CyanosisCyanosis Congestive Cardiac FailureCongestive Cardiac Failure ArrythmiasArrythmias Abnormal CXRAbnormal CXR Abnormal ECGAbnormal ECG

CyanosisCyanosis (form the Greek word meaning (form the Greek word meaning dark bluedark blue))

What is cyanosis?What is cyanosis? Blue discolouration of lips / tongue / extremetiesBlue discolouration of lips / tongue / extremeties Oxygenated Hb is bright redOxygenated Hb is bright red Reduced Hb is blue / purpleReduced Hb is blue / purple

Cyanosis is dependent upon the absolute Cyanosis is dependent upon the absolute concentration of reduced Hb.concentration of reduced Hb.

TTccSaOSaO22<85% OR > 3g deoxygenated Hb<85% OR > 3g deoxygenated Hb If present through the entire body= CENTRAL If present through the entire body= CENTRAL

CYANOSISCYANOSIS If present only in the extremeties = If present only in the extremeties =

PERIPHERAL CYANOSISPERIPHERAL CYANOSIS

Lees 1970

Making the diagnosisMaking the diagnosis Clinically:Clinically:

Early detection in Early detection in newborns is essentialnewborns is essential

May be difficult to see in May be difficult to see in dark skinned individuals – dark skinned individuals – tip of tonguetip of tongue

Good lighting essentialGood lighting essential Thermo-neutral Thermo-neutral

environmentenvironment Is the child distressed/any Is the child distressed/any

other signs of cardiac other signs of cardiac abnormalities??abnormalities??

Does the blueness fade on Does the blueness fade on pressure?pressure?

Is the child cold / poorly Is the child cold / poorly perfusedperfused

If in doubt – Saturation with pulse oximeter If in doubt – Saturation with pulse oximeter and/or arterial blood gas:and/or arterial blood gas: Normal TNormal TccSaOSaO22 in newborn in room air is 92% in newborn in room air is 92%

Normal PNormal PaaOO22 in newborn is >60mmHg in newborn is >60mmHg

(>8Kpa)(>8Kpa)

Transcutaneous Saturation Transcutaneous Saturation measurementmeasurement

Uses light absorption at a given wavelength Uses light absorption at a given wavelength measures bound Omeasures bound O2 2

assumes a normal haemoglobin moleculeassumes a normal haemoglobin molecule Sat of 94% - equivalent to Sa0Sat of 94% - equivalent to Sa022 of 90% of 90%

Misses bradyarrythmiasMisses bradyarrythmias Completely inaccurate below 70% (beware of Completely inaccurate below 70% (beware of

nail polish!!!!)nail polish!!!!) Ear best in a shocked patientEar best in a shocked patient

Causes of CyanosisCauses of Cyanosis

In a nutshell…..In a nutshell…..

Respiratory diseaseRespiratory disease Shock Shock metabolic derangementmetabolic derangement

hypoglycaemia, hypothermiahypoglycaemia, hypothermia congenital heart diseasecongenital heart disease methaemoglobinaemiamethaemoglobinaemia persistent foetal circulationpersistent foetal circulation

STEPS IN MANAGEMENT OF STEPS IN MANAGEMENT OF CYANOSED NEWBORNSCYANOSED NEWBORNS

Steps in the management of Steps in the management of cyanotic newborns:cyanotic newborns:

1.1. CHEST XRAY:CHEST XRAY: May reveal pulmonary causeMay reveal pulmonary cause May hint to the presence or absence of cardiac lesionMay hint to the presence or absence of cardiac lesion

2.2. ARTERIAL BLOOD GAS IN ROOM AIR:ARTERIAL BLOOD GAS IN ROOM AIR: Confirm or reject cyanosisConfirm or reject cyanosis Elevated pCOElevated pCO22 suggests pulmonary or CNS cause suggests pulmonary or CNS cause LOW pH in severe shock, sepsis, severe hypoxaemiaLOW pH in severe shock, sepsis, severe hypoxaemia

3.3. HYPEROXIA TEST:HYPEROXIA TEST:4.4. ECG:ECG:

If cardiac disease suspected – will give clue to diagnosisIf cardiac disease suspected – will give clue to diagnosis5.5. PROSTAGLANDIN E1:PROSTAGLANDIN E1:

If heart lesion suspected that is ductus dependent (eg pulmonary If heart lesion suspected that is ductus dependent (eg pulmonary atresia,Tetralogy of Fallot, TGA, Coarctation of the aorta)atresia,Tetralogy of Fallot, TGA, Coarctation of the aorta)

Assessment of cyanosisAssessment of cyanosis

CXR

Pulmonary/Cardiac

ABGHyperoxia test

Cardiac vs Pulmonary cause

SepsisHypoglycaemiaPolycythaemia

Cardiac CausePPHN

Septic ScreenBlood glucose

ECGEchocardiogram

Asphyxia

CNS(hypoventilation)

Hb abnormalities

Metabolic causes

The Chest X RayThe Chest X Ray

““classical” patterns - rareclassical” patterns - rare better use is to judge pulmonary flowbetter use is to judge pulmonary flow oligaemia - dark lung fields - tetralogyoligaemia - dark lung fields - tetralogy plethora - increased flow - mixersplethora - increased flow - mixers use to side the archuse to side the arch visceral situs in complex formsvisceral situs in complex forms

The hyperoxia testThe hyperoxia test

Cyanosis confirmed with arterial oxygen Cyanosis confirmed with arterial oxygen measurementmeasurement

to differentiate between respiratory and cardiac to differentiate between respiratory and cardiac causes of cyanosiscauses of cyanosis

administer oxygen at the highest concentration administer oxygen at the highest concentration possible (head box)possible (head box)

blood gas from blood gas from RightRight radial artery radial artery Wait for 15 minutes – repeat ABGWait for 15 minutes – repeat ABG

pOpO22 < 150 mm Hg (20 kPa) - cardiac < 150 mm Hg (20 kPa) - cardiac

pOpO22 > 250 mm Hg (33 kPa) - respiratory > 250 mm Hg (33 kPa) - respiratory

Failures of the Hyperoxia testFailures of the Hyperoxia test

Cyanotic heart defect with large pulmonary Cyanotic heart defect with large pulmonary blood flow (eg TAPVD) – pOblood flow (eg TAPVD) – pO22 may rise may rise with Owith O22 administration. administration.

Massive intrapulmonary shunts but a Massive intrapulmonary shunts but a normal heart (eg PPHN, AVM) may not normal heart (eg PPHN, AVM) may not raise the pOraise the pO22 with oxygen – pO with oxygen – pO22 wont rise wont rise with Owith O22..

Response to oxygen inhalation must be Response to oxygen inhalation must be interpreted in the light of the clinical picture interpreted in the light of the clinical picture

BEFORE REFERRAL TO BEFORE REFERRAL TO CARDIOLOGIST – ATTEMPT TO CARDIOLOGIST – ATTEMPT TO

MAKE A REASONABLE MAKE A REASONABLE DIAGOSISDIAGOSIS

CXR

Reduced PulmonaryBlood flow

Increased Pulmonary Blood flow

ECG

RVH

LVH

CVH

RVH

LVH

Tetralogy

PAtresiaTric atresia

DTGATruncus

TAPVD

TGA

Explanation of a few common Explanation of a few common cyanotic congenital heart defectscyanotic congenital heart defects

TETRALOGY OF FALLOTTETRALOGY OF FALLOT

LV

LA

RV

RA VSD

Aorta overrides septum

Infundibular “PS”

RVH

““PALLIATED” TETRAOGYPALLIATED” TETRAOGY

RA

RVLV

LA

Blalock-Taussig shunt

TETRALOGY OF FALLOT

RV HYPERTROPHY

Ejection systolic murmur

Single S2

NO RV heave

Ejection systolic murmur

Single S2

NO RV heaveINFUNDIBULAR STENOSIS

AORTIC OVERRIDE

VSD

NO CYANOSISNO CYANOSISNO CYANOSISNO CYANOSIS

MILD CYANOSISMILD CYANOSISMILD CYANOSISMILD CYANOSIS

TETRALOGY OF FALLOT

‘HYPERCYANOTIC SPELL’

CYANOSISCYANOSIS

ACIDOSISACIDOSIS

Tachypnoea

NO ejection systolic murmur

Death

Tachypnoea

NO ejection systolic murmur

DeathPOSITIVE FEEDBACK

TRANSPOSITION OF THE GREAT TRANSPOSITION OF THE GREAT ARTERIESARTERIES

RA

LA

LVRV

Aorta from RV

Pulmonary Artery from LV

TGA

SEVERESEVERECYANOSISCYANOSIS

Two separate parallel circuits

Incompatible with lifeNo murmurs

Two separate parallel circuits

Incompatible with lifeNo murmurs

LA ENLARGEMENT

LV ENLARGEMENT

CCFCCF

Aorta PA

RV LV

TGAIVC

PDA

Rashkind atrial septostomy

Followed by :

Arterial switch or

Mustard operation

Followed by :

Arterial switch or

Mustard operation

TRICUSPID ATRESIATRICUSPID ATRESIA

RALV

LA

Atretic TV

VSD

TRUNCUS ARTERIOSUSTRUNCUS ARTERIOSUS

LV

RV

VSD

“Truncus”

Aorta

Pulmonary artery

RA

after referral?after referral?

ECG can give clues to the diagnosisECG can give clues to the diagnosis echocardiography - main diagnostic toolechocardiography - main diagnostic tool catheterisation - particularly to assess catheterisation - particularly to assess

pulmonary artery structure and sizepulmonary artery structure and size balloon septostomy in TGAballoon septostomy in TGA use of IV prostaglandin to keep the duct openuse of IV prostaglandin to keep the duct open surgerysurgery

Pre Muscle resection Post Muscle resection

Post VSD Repair

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