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Approach to the Cyanotic Approach to the Cyanotic Infant Infant Billie Parsley Billie Parsley

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Approach to the Cyanotic InfantApproach to the Cyanotic InfantBillie ParsleyBillie Parsley

GoalsGoals

Recognition of a cyanotic infantRecognition of a cyanotic infant

What to do in any setting from ICU to rural What to do in any setting from ICU to rural community pediatric practicecommunity pediatric practice What questions to askWhat questions to ask What to look forWhat to look for What imaging to orderWhat imaging to order What test to orderWhat test to order

CaseCase

39 week male born at home in uncomplicated 39 week male born at home in uncomplicated vaginal delivery vaginal delivery

Presented at ACH ED 24 hours old due to Presented at ACH ED 24 hours old due to temp of 35.1 rectaltemp of 35.1 rectal

Sats in 50s, HR 100, RR 80, BP 76/59Sats in 50s, HR 100, RR 80, BP 76/59

Cyanotic on general appearanceCyanotic on general appearance

What is cyanosis?What is cyanosis?

Cyanosis is a bluish discoloration of the tissue that Cyanosis is a bluish discoloration of the tissue that results when the absolute level of reduced results when the absolute level of reduced hemoglobin (when not combined with oxygen) in hemoglobin (when not combined with oxygen) in the capillary bed exceeds 3 g/dL. The the capillary bed exceeds 3 g/dL. The appearance of cyanosis depends upon the total appearance of cyanosis depends upon the total amount of reduced hemoglobin rather than the amount of reduced hemoglobin rather than the ratio of reduced to oxygenated hemoglobin.ratio of reduced to oxygenated hemoglobin.

CyanosisCyanosis

Two mechanisms result in cyanosis:Two mechanisms result in cyanosis: Peripheral cyanosisPeripheral cyanosis

Normal systemic arterial oxygen saturation and Normal systemic arterial oxygen saturation and increased tissue oxygen extraction that leads to a increased tissue oxygen extraction that leads to a widened systemic arteriovenous oxygen difference of widened systemic arteriovenous oxygen difference of >60% (normal 40%) resulting in an increased >60% (normal 40%) resulting in an increased concentration of reduced hemoglobin on the venous concentration of reduced hemoglobin on the venous side of the capillary bed.side of the capillary bed.

Central cyanosis caused by systemic arterial oxygen Central cyanosis caused by systemic arterial oxygen desaturationdesaturation

Don’t confuse central cyanosis with acrocyanosis!!!! Don’t confuse central cyanosis with acrocyanosis!!!! Acrocyanosis-common physical finding in newborns as Acrocyanosis-common physical finding in newborns as

a result of peripheral vasoconstrictiona result of peripheral vasoconstriction

CyanosisCyanosis

Best indicator is the tongue-due to free of Best indicator is the tongue-due to free of pigmentation and has rich vascular supply.pigmentation and has rich vascular supply.

Also seen in lips, nails, earlobes, mucous Also seen in lips, nails, earlobes, mucous membranesmembranes

CyanosisCyanosis

Depends on multiple Depends on multiple factorsfactors HemoglobinHemoglobin Fetal hemoglobinFetal hemoglobin Skin PigmentSkin Pigment Factors affecting the Factors affecting the

hemoglobin dissociation hemoglobin dissociation curvecurve

CauseCause

Alveolar hypoventilationAlveolar hypoventilation Nml response is hypercarbia but can also cause Nml response is hypercarbia but can also cause

hypoxemiahypoxemia

Ventilation Perfusion mismatchVentilation Perfusion mismatch Nml is for areas with decreased ventilation to have Nml is for areas with decreased ventilation to have

decreased blood flow, but alterations in this cause decreased blood flow, but alterations in this cause hypoxemiahypoxemia

Diffusion ImpairmentDiffusion Impairment Oxygen molecules must diffuse from alveoli to pulmonary Oxygen molecules must diffuse from alveoli to pulmonary

capillary to oxygenate hemoglobincapillary to oxygenate hemoglobin

Right to Left ShuntRight to Left Shunt Systemic venous blood bypasses ventilated alveoli and Systemic venous blood bypasses ventilated alveoli and

returns to the left heart without being oxygenatedreturns to the left heart without being oxygenated

Disturbed HemoglobinDisturbed Hemoglobin Decreased oxygen affinityDecreased oxygen affinity

HistoryHistory

PregnancyPregnancy Maternal Diabetes-associated with cyanotic heart Maternal Diabetes-associated with cyanotic heart

dz.dz. Polyhydramnios-associated with fetal airway, Polyhydramnios-associated with fetal airway,

esophageal, neurologic abnormalitiesesophageal, neurologic abnormalities Oligohydramnios-associated with renal defects and Oligohydramnios-associated with renal defects and

pulmonary hypoplasiapulmonary hypoplasia Drug Use during pregnancyDrug Use during pregnancy Maternal SerologyMaternal Serology US resultsUS results Previous complications with pregnancy Previous complications with pregnancy

(stillborn/multiple spontaneous abortions)(stillborn/multiple spontaneous abortions) Medication Use during pregnancyMedication Use during pregnancy

HistoryHistory

Perinatal/PeripartumPerinatal/Peripartum Gestational AgeGestational Age Prolonged/Premature Rupture of MembranesProlonged/Premature Rupture of Membranes What/How treatment given for illness during What/How treatment given for illness during

pregnancy (GBS)pregnancy (GBS) Maternal feverMaternal fever Birth TraumaBirth Trauma Neonatal polycythemiaNeonatal polycythemia HypoglycemiaHypoglycemia Meconium Staining-associated with Meconium Meconium Staining-associated with Meconium

Aspiration syndrome and P-HTNAspiration syndrome and P-HTN Hospital course post deliveryHospital course post delivery

HistoryHistory

HPIHPI FeverFever ExposureExposure TraumaTrauma Well waterWell water SeizureSeizure Poor FeedingPoor Feeding Decreased urine outputDecreased urine output MedicationsMedications Different Temperatures between extremitiesDifferent Temperatures between extremities Cough/Congestion Cough/Congestion Rashes Changes in skinRashes Changes in skin ““birth marks”birth marks” Abnormal breathing-pauses in breathingAbnormal breathing-pauses in breathing Weight loss or gainWeight loss or gain EdemaEdema

HistoryHistory

DietDiet What (could mom be taking in meds that get into What (could mom be taking in meds that get into

breastmilk)breastmilk) How long does it take?How long does it take? Perspiration with feedsPerspiration with feeds Coughing/Gagging/Gurgling with feedsCoughing/Gagging/Gurgling with feeds

MedicationsMedications

Family HistoryFamily History HemoglobinopathiesHemoglobinopathies Congenital Cardiac AbnormalitiesCongenital Cardiac Abnormalities Cystic FibrosisCystic Fibrosis Neuromusclar DisordersNeuromusclar Disorders

Physical ExamPhysical Exam

*The presence and degree of respiratory distress are very *The presence and degree of respiratory distress are very important factors in differentiating your 2 main causes of important factors in differentiating your 2 main causes of Cyanosis-Cardiac vs. RespiratoryCyanosis-Cardiac vs. Respiratory

General exam: Comfort, WOB, LOC, Color, General exam: Comfort, WOB, LOC, Color,

Head: Fontanel, Shape of Head, eccymosis, lacerations, Head: Fontanel, Shape of Head, eccymosis, lacerations, Pupils, nose shape and location, shape of chin and size, mouth Pupils, nose shape and location, shape of chin and size, mouth size, moisture, cleft palate, size, moisture, cleft palate,

Neck: size of vessels, abnormal massesNeck: size of vessels, abnormal masses

Abdomen: HSM, ascities, Abdomen: HSM, ascities,

Skin: Rashes, birthmarks , mottled, slate gray color associated Skin: Rashes, birthmarks , mottled, slate gray color associated with methemolglobinemiawith methemolglobinemia

Limb abnormalitiesLimb abnormalities

Physical ExamPhysical Exam

Respiratory Exam Respiratory Exam Respiratory Rate (newborn nml 40-60)Respiratory Rate (newborn nml 40-60)

Low RR-alveolar hypoventilationLow RR-alveolar hypoventilation Tachypnea-Cardiac or Pulmonary cause Tachypnea-Cardiac or Pulmonary cause

Regularity of RR Regularity of RR apnea events apnea events Grunting (prevents end expiratory alveolar collapse)Grunting (prevents end expiratory alveolar collapse) Nasal flare (reduces nasal resistance)Nasal flare (reduces nasal resistance) StridorStridor Tracheal DeviationTracheal Deviation Chest wall movement Chest wall movement Retractions Retractions Accessory muscle use Accessory muscle use Auscultation Auscultation

Physical ExamPhysical Exam

Cardiac Exam-not as severe respiratory Cardiac Exam-not as severe respiratory distressdistress Heart RateHeart Rate PulsesPulses PerfusionPerfusion AuscultationAuscultation Four Extremity Blood PressureFour Extremity Blood Pressure Pre/Post Ductal oxygen SaturationPre/Post Ductal oxygen Saturation Point of Maximal ImpulsePoint of Maximal Impulse

LabsLabs

ABG-for arterial oxygenationABG-for arterial oxygenation Painful and can cause increasing agitationPainful and can cause increasing agitation Methemoglobinemia oxygen saturation will be low but Methemoglobinemia oxygen saturation will be low but

measured PO2 will be normalmeasured PO2 will be normal Elevated arterial PCO2 suggest pulmonary causeElevated arterial PCO2 suggest pulmonary cause Metabolic acidosis indicates poor perfusion-possible due to Metabolic acidosis indicates poor perfusion-possible due to

poor cardiac output or oxygen delivery or shock. poor cardiac output or oxygen delivery or shock.

CBCCBC Can show high hgb/hct in polycythemiaCan show high hgb/hct in polycythemia I/T ratioI/T ratio

Blood glucoseBlood glucose

Blood CultureBlood Culture

ImagingImaging

TraumaTrauma

AirwayAirway

Situs of heart, stomach, liver-any abnormalities in Situs of heart, stomach, liver-any abnormalities in location point towards cardiac diseaselocation point towards cardiac disease

Heart size and shapeHeart size and shape

Lung fieldsLung fields

Vascular MarkingsVascular Markings Decreased vascular markings can be seen in Decreased vascular markings can be seen in

cyanotic cardiac lesions and pulmonary cyanotic cardiac lesions and pulmonary hypertensionhypertension

Other TestOther Test

Hyperoxia testing—Right radial artery PaO2 Hyperoxia testing—Right radial artery PaO2 (preductal) measured on RA and after 10 (preductal) measured on RA and after 10 minutes on 100%FiO2 minutes on 100%FiO2

if right radial arterial PaO2 on 100% FiO2 is if right radial arterial PaO2 on 100% FiO2 is less than 150mmHg, severe congenital heart less than 150mmHg, severe congenital heart disease is likely. The infant is presumed to disease is likely. The infant is presumed to have ductal dependant congenital heart have ductal dependant congenital heart disease and the low PaO2 is attributed to the disease and the low PaO2 is attributed to the obligatory mixing of oxygenated with obligatory mixing of oxygenated with deoxygenated blood within the circulatory deoxygenated blood within the circulatory system.system.

Other TestOther Test

EchoEcho TranspositionTransposition Tetralogy of FallotTetralogy of Fallot Truncus arteriosusTruncus arteriosus Total anomalous pulmonary venous ReturnTotal anomalous pulmonary venous Return TricuspidTricuspid ““Tons” of othersTons” of others

EKGEKG

ManagmentManagment

ABCsABCs

Access-venous and arterial if possibleAccess-venous and arterial if possible

Ventilation as neededVentilation as needed

Cautious fluid resuscitationCautious fluid resuscitation

AntibioticsAntibiotics

Prostaglandin InfusionProstaglandin Infusion

ProstaglandinProstaglandin

Goal is to prevent closure of the PDA if this is Goal is to prevent closure of the PDA if this is where mixing is occurring.where mixing is occurring.

Improvement normally seen within 1-2 hoursImprovement normally seen within 1-2 hours

Side effects: APNEA and peripheral Side effects: APNEA and peripheral vasodilatation with hypotension vasodilatation with hypotension

Case ReviewCase Review

Mom GBS + treated with oral clindamycinMom GBS + treated with oral clindamycin

Baby with severe Pulmonary HTN with near Baby with severe Pulmonary HTN with near systemic pressuressystemic pressures

By 48 hours of life on ECMO on way to By 48 hours of life on ECMO on way to EdmontonEdmonton