Cyanosis

Evaluation of the Cyanotic Evaluation of the Cyanotic InfantInfant

Bill LefkowitzBill Lefkowitz

16 NOV 200016 NOV 2000

OutlineOutline Cyanosis - define and describeCyanosis - define and describe Terms (PaOTerms (PaO22 v SaO v SaO22 v CaO v CaO22)) Differential of cyanosisDifferential of cyanosis The hyperoxia testThe hyperoxia test A few etiologiesA few etiologies Take home pointsTake home points Evaluation and intervention FlowchartEvaluation and intervention Flowchart

CyanosisCyanosis 3g/dL deoxyhemoglobin3g/dL deoxyhemoglobin Low flow areas with increased oxygen Low flow areas with increased oxygen

extraction have more extraction have more deoxyhemoglobindeoxyhemoglobin

High flow areas with less extraction High flow areas with less extraction should not have enough should not have enough deoxyhemaglobin to appear cyanoticdeoxyhemaglobin to appear cyanotic Under normal circumstances you should not be able to Under normal circumstances you should not be able to

extract enough Oextract enough O22 to have 3g/dL deoxyhemoglobin running to have 3g/dL deoxyhemoglobin running through the tongue and gums (lips OK when cold)through the tongue and gums (lips OK when cold)

Cyanosis Acro v CentralCyanosis Acro v Central

AcrocyanosisAcrocyanosis part of normal part of normal

transitiontransition may last 72hrmay last 72hr beware APGAR beware APGAR

of 10of 10 hypoperfusedhypoperfused severe anemiasevere anemia

CyanosisCyanosis

Cyanosis

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0 20 40 60 80

HCT

% S

atu

rati

on

CyanosisCyanosis

Cyanosis is Cyanosis is dependent on dependent on HCT and % SatHCT and % Sat

Florescent light Florescent light makes cyanosis makes cyanosis hard to see.hard to see.

Except in the Except in the extreme, cyanosis extreme, cyanosis is not obviousis not obvious

Any question, Any question, check a pulse check a pulse oxox

RA v. ORA v. O22 - Saturations - Saturations

TermsTerms PaOPaO22 Arterial Oxygen Arterial Oxygen PressurePressure

Measured on an ABG machineMeasured on an ABG machine Oxygen dissolved in plasmaOxygen dissolved in plasma

0.003 ml O0.003 ml O22/mmHg/dl plasma/mmHg/dl plasma

SaOSaO22 Percent Oxygen Percent Oxygen SaturationSaturation

Measured by saturation monitor (pulse-Ox)Measured by saturation monitor (pulse-Ox) ~1.34ml O2/g Hb~1.34ml O2/g Hb

PaOPaO22 and SaO and SaO22

Oxyhemoglobin Oxyhemoglobin dissociationdissociation

TermsTerms CaOCaO22

Oxygen Content of the bloodOxygen Content of the blood bound to Hb + dissolved in plasmabound to Hb + dissolved in plasma

100% saturated100% saturated Hb 18g/dlHb 18g/dl 90 mmHg in plasma90 mmHg in plasma

= [1.0*18*1.34]+[0.003*90]= [1.0*18*1.34]+[0.003*90] = [24]+[0.25]= [24]+[0.25] Physiologically, saturations much more Physiologically, saturations much more

important to oxygen content important to oxygen content (0.003*600 (0.003*600 = 1.8)= 1.8)

CaOCaO22 Samples Samples

NORMALNORMAL95%, Hb 1895%, Hb 18PaO2 85PaO2 85[.95*18*1.34]+[0.003*8[.95*18*1.34]+[0.003*8

5]5]

[23]+[0.25]=[23]+[0.25]=2233


HYPEROXIAHYPEROXIA100%, Hb 18 100%, Hb 18 PaO2 600PaO2 600[1.0*18*1.34]+[0.003*6[1.0*18*1.34]+[0.003*6

00]00]

[24]+[1.8]=[24]+[1.8]=2266


ANEMIAANEMIA95%, Hb 10 95%, Hb 10 PaO2 85PaO2 85[.95*10*1.34]+[0.003*85][.95*10*1.34]+[0.003*85]

[13]+[0.25]=[13]+[0.25]=1313


ANEMIA-ANEMIA-HYPEROXIAHYPEROXIA

100%, Hb 10 100%, Hb 10 PaO2 600PaO2 600[1.0*10*1.34]+[0.003*600][1.0*10*1.34]+[0.003*600]

[13]+[1.8]=[13]+[1.8]=1515


CCHDCCHD75%, Hb 1875%, Hb 18PaO2 40PaO2 40[.75*18*1.34]+[0.003*40][.75*18*1.34]+[0.003*40]

[18]+[0.12]=[18]+[0.12]=1188


CCHD-ANEMIACCHD-ANEMIA75%, Hb 1075%, Hb 10PaO2 40PaO2 40[.75*10*1.34]+[0[.75*10*1.34]+[0..003*40]003*40]

[10]+[0.12]=[10]+[0.12]=1010

Differential of cyanosisDifferential of cyanosis 3g of deoxyhemoglobin/dL 3g of deoxyhemoglobin/dL

(cyanosis)(cyanosis)

- Not enough oxygen in- Not enough oxygen in - Oxygen “mal-absorption”- Oxygen “mal-absorption” - Too much oxygen out- Too much oxygen out

Not enough oxygen inNot enough oxygen in ApneaApnea

neurologic and pharmacologic causesneurologic and pharmacologic causes Diffusion barrierDiffusion barrier

RDS, aspiration, pneumoniaRDS, aspiration, pneumonia ObstructionObstruction

pneumothorax, head positionpneumothorax, head position

Oxygen “mal-absorption”Oxygen “mal-absorption” Shunting lesionsShunting lesions

cardiaccardiac non-cardiac (like PPHN)non-cardiac (like PPHN)

HematologicHematologic methemoglobinemiamethemoglobinemia carboxyhemoglobinemiacarboxyhemoglobinemia

Too much oxygen outToo much oxygen out High oxygen consumptionHigh oxygen consumption

sepsissepsis low flow, high extractionlow flow, high extraction

acrocyanosisacrocyanosis hyperviscosity/polycythemiahyperviscosity/polycythemia extravasated (ie bruising)extravasated (ie bruising)

What is the Hyperoxia test?What is the Hyperoxia test?

Hyperoxia TestHyperoxia Test Infant on Room Air, get ABGInfant on Room Air, get ABG Infant on 100% oxygen, get ABGInfant on 100% oxygen, get ABG PaOPaO22 unchanged = fixed shunt = unchanged = fixed shunt =

CCHDCCHD Max PaOMax PaO22 <100 = CCHD <100 = CCHD Max PaOMax PaO22 >200 = No CCHD >200 = No CCHD

Hyperoxia TestHyperoxia Test Giving 100% overcomes the diffusion barrierGiving 100% overcomes the diffusion barrier

Normal -----------Normal ----------- Bad Lungs -------Bad Lungs -------

(diffusion barrier)(diffusion barrier)

Shunt -------------Shunt -------------

Shunt with Bad --Shunt with Bad --

LungsLungs

Hyperoxia TestHyperoxia Test

Hyperoxia Hyperoxia ProperProper

Hyperoxia Hyperoxia CPAPCPAP

Hyperoxia Hyperoxia hyper-hyper-ventilationventilation

Hyperoxia TestHyperoxia Test Jones: 1976Jones: 1976

8/109 with CCHD had PaO8/109 with CCHD had PaO22 > > 100mmHg100mmHg

7/23 without CCHD (bad RDS etc) had 7/23 without CCHD (bad RDS etc) had PaOPaO22 < 150mmHg < 150mmHg

HLHS > 300mmHgHLHS > 300mmHg TGA, TAPVR > 200mmHgTGA, TAPVR > 200mmHg Don’t be fooled by early high PaODon’t be fooled by early high PaO22ss

Hyperoxia TestHyperoxia Test Don’t do the room air partDon’t do the room air part

Looking for minimal PaOLooking for minimal PaO22 change from 21% change from 21% to 100% fiOto 100% fiO22

Hyperoxia test developed pre pulse-oxHyperoxia test developed pre pulse-ox With pulse-ox you can tell when PaOWith pulse-ox you can tell when PaO22s are s are

not changing despite big changes in fiOnot changing despite big changes in fiO22 (for sats that are between 70 and 95%)(for sats that are between 70 and 95%)

Probably the norm to have some degree of Probably the norm to have some degree of lung disease at the time of the test anywaylung disease at the time of the test anyway

Information neededInformation needed Clinical appearanceClinical appearance

““comfortably tachypneic and blue”comfortably tachypneic and blue” Pulses/perfusionPulses/perfusion

differential, delayeddifferential, delayed Pulse-Ox/ABGPulse-Ox/ABG

pre and post ductal, max PaOpre and post ductal, max PaO22

AuscultationAuscultation S2, MurmurS2, Murmur

Information neededInformation needed CXRCXR

heart shapesheart shapes snowman = TAPVRsnowman = TAPVR11

boot = pulm atresia, TOF, tricuspid atresiaboot = pulm atresia, TOF, tricuspid atresia egg on string = TGAegg on string = TGA

pulmonary vascularitypulmonary vascularity EKGEKG

axisaxis increased or decreased forcesincreased or decreased forces

TAPVRTAPVR11

SnowmanSnowman 50% type 150% type 1 50% 50%

snowmansnowman venous venous

connection at connection at VV to SVCVV to SVC

TOFTOF

Boot Boot shapeshape RVH lifting RVH lifting

apexapex loss of PA loss of PA

knobknob

TGATGA

Egg on a Egg on a stringstring alignment of alignment of

PA and Ao PA and Ao narrows the narrows the mediastinummediastinum

The 6 T’sThe 6 T’s

Total Anomalous Pulmonary VeinsTotal Anomalous Pulmonary Veins Tetrology of Fallot Tetrology of Fallot Tricuspid AtresiaTricuspid Atresia TranspositionTransposition Truncus ArteriosusTruncus Arteriosus

Total AcardiaTotal Acardia

Normal AnatomyNormal Anatomy normalnormal

PPHN - PDA shuntingPPHN - PDA shunting Pre and post Pre and post

ductal satsductal sats Head and Head and

coronaries get coronaries get well well oxygenated oxygenated bloodblood


PPHN - PFO shuntingPPHN - PFO shunting Head and Head and

coronaries coronaries get get hypoxemic hypoxemic bloodblood

TAPVRTAPVR

TAPVRTAPVR Type 1 Type 1

(supracardiac)(supracardiac) 50% with 50% with

snowmansnowman can have can have

UE<LE satsUE<LE sats Type 2 (cardiac)Type 2 (cardiac) Type 3 Type 3

(infracardiac)(infracardiac) all can all can

obstruct, obstruct, infracardiac infracardiac almost always almost always doesdoes


TOFTOF OverridinOverridin

g Aortag Aorta VSDVSD PSPS RVHRVH


Tricuspid AtresiaTricuspid Atresia

TGATGA normalnormal

TGA - no PPHNTGA - no PPHN Comfortably Comfortably

tachypneic tachypneic (usually big) (usually big) child with child with sats in the sats in the 50s on room 50s on room air or air or oxygenoxygen

TGA - with PPHNTGA - with PPHN TGA and TGA and

TAPVR type 1 TAPVR type 1 can present can present with higher with higher sats in then sats in then lower lower extremities extremities than the upper than the upper

extremitiesextremities


Truncus ArteriosusTruncus Arteriosus


Total AcardiaTotal Acardia Poor Poor

prognosis, prognosis, consider consider DNRDNR

Flow diagramFlow diagram There are bad acyanotic lesionsThere are bad acyanotic lesions There are bad cyanotic lesionsThere are bad cyanotic lesions A ductal dependent lesion is one that A ductal dependent lesion is one that

depends on the ductus to get adequate depends on the ductus to get adequate blood flow to the pulmonary and blood flow to the pulmonary and systemic circuits, or provide mixingsystemic circuits, or provide mixing

PSPS CoACoA TGATGA

Summary and Take Home Summary and Take Home PointsPoints

Check a pulse ox Check a pulse ox (murmur w/u, suspicion)(murmur w/u, suspicion) murmur w/u (š Sx) murmur w/u (š Sx) Pulse-OxPulse-Ox, EKG, 4-point BPs, EKG, 4-point BPs

Don’t be fooled by a reassuring Don’t be fooled by a reassuring hyperoxia test if suspicion remains highhyperoxia test if suspicion remains high

If you think you have a ductal If you think you have a ductal dependent lesion, start prostin (don’t be dependent lesion, start prostin (don’t be afraid of prostin)afraid of prostin)

Summary and Take Home Summary and Take Home PointsPoints

If a baby presents in shock in the first If a baby presents in shock in the first week week (or two)(or two) of life think sepsis & of life think sepsis & ductal dependant lesions & CAHductal dependant lesions & CAH

Cyanosis w/u includes antibioticsCyanosis w/u includes antibiotics Make sure your pharmacy carries Make sure your pharmacy carries

prostinprostin Don’t be afraid to use itDon’t be afraid to use it Know how to mix it!Know how to mix it!

QUESTIONS?QUESTIONS?

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Cyanosis