Evaluation of a Cyanotic Evaluation of a Cyanotic Newborn Newborn Beena Kamath, MD MPH Beena Kamath, MD MPH (Special thanks to John Kinsella) (Special thanks to John Kinsella) SBN Lecture Series SBN Lecture Series January 13, 2011 January 13, 2011
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Evaluation of a Cyanotic Evaluation of a Cyanotic NewbornNewborn
Beena Kamath, MD MPHBeena Kamath, MD MPH(Special thanks to John Kinsella)(Special thanks to John Kinsella)
SBN Lecture SeriesSBN Lecture SeriesJanuary 13, 2011January 13, 2011
Background: CHDBackground: CHD
Congenital heart disease (CHD): 8/1000 Congenital heart disease (CHD): 8/1000 live birthslive births
2/1000 will present at <1 year of age2/1000 will present at <1 year of age
25% with additional non25% with additional non--cardiac anomaliescardiac anomalies
Critical CHD: 3.5/1000 live birthsCritical CHD: 3.5/1000 live births
In raw numbers, approximately 32,000 In raw numbers, approximately 32,000 children born with CHDchildren born with CHD
14,000 of these children have critical heart 14,000 of these children have critical heart diseasedisease
Congenital Heart DefectCongenital Heart Defect IncidenceIncidence
Ventricular septal defect Ventricular septal defect (VSD)(VSD)
16% (VSD is the most common congenital heart 16% (VSD is the most common congenital heart defect)defect)
Pulmonary stenosis with Pulmonary stenosis with intact ventricular septumintact ventricular septum
7.5 7.5 ––
12%12%
Tetralogy of Fallot (TOF)Tetralogy of Fallot (TOF) 88--10% (TOF is the most common cyanotic heart 10% (TOF is the most common cyanotic heart disease beyond infancy)disease beyond infancy)
Atrial septal defect (ASD) Atrial septal defect (ASD) 66--11%11%
Transposition of the great Transposition of the great arteriesarteries
55--10% (TGA is the most common cyanotic heart 10% (TGA is the most common cyanotic heart disease presenting in the first week of life)disease presenting in the first week of life)
Patent ductus arteriosusPatent ductus arteriosus 44--10% in full term infants10% in full term infants
Coarctation of the aortaCoarctation of the aorta 55--8%8%
Atrioventricular septal defectsAtrioventricular septal defects 22--5%5%
Total anomalous pulmonary Total anomalous pulmonary venous returnvenous return
11--2.5%2.5%
Truncus arteriosusTruncus arteriosus 11--4%4%
Hypoplastic left heart Hypoplastic left heart syndromesyndrome
1.5% (HLHS is the 21.5% (HLHS is the 2ndnd
most common cyanotic heart most common cyanotic heart disease presenting in the first week of life)disease presenting in the first week of life)
Genetic Syndromes: CHDGenetic Syndromes: CHD
Trisomy 21: AVSD (30%)Trisomy 21: AVSD (30%)
Turner (XO): Coarctation, ASTurner (XO): Coarctation, AS
CriCri--dudu--chat (5pchat (5p--): VSD): VSD
Williams (microdel. 7)Williams (microdel. 7)--supravalvular AS, supravalvular AS, PPSPPS
NoonanNoonan’’s (AD Chr 12)s (AD Chr 12)——Pulmonary Pulmonary stenosisstenosis
Maternal Causes: CHDMaternal Causes: CHD
Systemic illnessesSystemic illnesses
Diabetes: HCMDiabetes: HCM
SLE: Complete heart blockSLE: Complete heart block
Intrauterine infectionIntrauterine infection
Rubella: PDA, septal defects, PPSRubella: PDA, septal defects, PPS
ToxicologyToxicology
AnticonvulsantsAnticonvulsants——AS, PS, CoarctationAS, PS, Coarctation
Excessive alcoholExcessive alcohol——Septal defectsSeptal defects
Board QuestionBoard Question
Match the congenital heart disease with the syndrome, Match the congenital heart disease with the syndrome, associated clinical anomalies or maternal conditionassociated clinical anomalies or maternal condition
Webbed neck, congenital lymphedema, Webbed neck, congenital lymphedema, prominent ears, broad chestprominent ears, broad chest
A: Interrupted aortic valveA: Interrupted aortic valve
Hypotonia, protuberant tongue, flat facial Hypotonia, protuberant tongue, flat facial profile, duodenal obstructionprofile, duodenal obstruction
B. Coarctation of the aortaB. Coarctation of the aorta
Severe hypocalcemia and seizures, cellular Severe hypocalcemia and seizures, cellular immunity deficit, and infectionsimmunity deficit, and infections
C. Dysplastic pulmonary valveC. Dysplastic pulmonary valve
Growth retardation, microcephaly, long Growth retardation, microcephaly, long philtrum, and smooth upper lipphiltrum, and smooth upper lip
D. AV canalD. AV canal
Cryptorchidism, small penis, webbed neck, Cryptorchidism, small penis, webbed neck, pectus excavatum, pectus excavatum, ““TurnerTurner--likelike””
E. VSDE. VSD
CyanosisCyanosis
Bluish discoloration due to arterial oxygen Bluish discoloration due to arterial oxygen desaturation of any causedesaturation of any cause
Occurs when concentration of capillary Occurs when concentration of capillary deoxyhemoglobin exceeds 3deoxyhemoglobin exceeds 3--5 gm/dL5 gm/dL
NOTE: defined by absolute amount of NOTE: defined by absolute amount of reduced Hb (NOT the oxygen saturation)reduced Hb (NOT the oxygen saturation)
CyanosisCyanosis
Hb = reduced Hb + HbO2Hb = reduced Hb + HbO2
O2 saturation = HbO2 / (HbO2 + reducedHbO2)O2 saturation = HbO2 / (HbO2 + reducedHbO2)
Occurs at different saturations depending on Hb levelOccurs at different saturations depending on Hb level
So, if an anemic infant has a Hb of 8 g/dL and one would expect So, if an anemic infant has a Hb of 8 g/dL and one would expect cyanosis to appear if there is a 5 g reduced Hb/dL, the HbO2 muscyanosis to appear if there is a 5 g reduced Hb/dL, the HbO2 must be t be 3. The O2 saturation at which the infant would appear cyanotic 3. The O2 saturation at which the infant would appear cyanotic is 3/8 is 3/8 = 37%= 37%
Similarly, if a polycythemic infant had a Hb of 22 g/dL, the HbOSimilarly, if a polycythemic infant had a Hb of 22 g/dL, the HbO2 at 2 at which the infant will appear cyanotic is 17. The O2 saturation which the infant will appear cyanotic is 17. The O2 saturation at at which the infant would appear cyanotic iswhich the infant would appear cyanotic is……
77%77%
Board questionBoard question
Match the maximum saturation at which the described Match the maximum saturation at which the described infant become cyanoticinfant become cyanotic
Polycythemic infant with Hb of 21 Polycythemic infant with Hb of 21 gm/dLgm/dL
Saturation of 100%Saturation of 100%
Saturation of 85%Saturation of 85%
Normal infant with Hb of 16 gm/dLNormal infant with Hb of 16 gm/dL Saturation of 81%Saturation of 81%
Saturation of 66%Saturation of 66%
Anemic infant with Hb of 9 gm/dLAnemic infant with Hb of 9 gm/dL Saturation of 50%Saturation of 50%
PathophysiologyPathophysiology
Who are the players involved in the cyanosis?Who are the players involved in the cyanosis?
Where is there a defect in the system?Where is there a defect in the system?
Heart Heart ••
Is there a problem with the delivery of blood to the lungs?Is there a problem with the delivery of blood to the lungs?••
Is there a right to left shunt?Is there a right to left shunt?
LungsLungs••
Is there a problem with oxygenation as it passes through the Is there a problem with oxygenation as it passes through the lungs?lungs?
End organsEnd organs••
Is there a shift with oxygen dissociation?Is there a shift with oxygen dissociation?
BloodBlood••
Is there a defect in the oxygen carrying capacity?Is there a defect in the oxygen carrying capacity?
HEARTHEART
Factors favoring R to L shuntingFactors favoring R to L shunting
Abnormal RV complianceAbnormal RV compliance
Atretic or stenotic pathwaysAtretic or stenotic pathways
Elevated PVRElevated PVR
Single ventricle physiologySingle ventricle physiology
AV discordanceAV discordance
Extrapulmonary RightExtrapulmonary Right--toto--Left Left ShuntShunt
Causes of HypoxemiaCauses of Hypoxemia in Neonatal Lung Diseasein Neonatal Lung Disease
Extrapulmonary Extrapulmonary
RightRight--toto--left shunting across left shunting across ““fetalfetal””
channelschannels••
Foramen ovale (FO)Foramen ovale (FO)
••
Patent ductus arteriosus (PDA)Patent ductus arteriosus (PDA)
LUNGSLUNGS
Anatomic barriersAnatomic barriers
MechanicalMechanical
Oxygen diffusion capacity is compromisedOxygen diffusion capacity is compromised
HypoxiaHypoxia
Lung ventilation distribution and Lung ventilation distribution and atelectasisatelectasis
Lung Disease Causing Hypoxemic Lung Disease Causing Hypoxemic Respiratory Failure in the NewbornRespiratory Failure in the Newborn
Meconium aspiration syndromeMeconium aspiration syndrome
Conditions characterized by diffuse, Conditions characterized by diffuse, homogeneous parenchymal disease homogeneous parenchymal disease (RDS, pneumonia)(RDS, pneumonia)
Pulmonary hypoplasia (oligohydramnios, Pulmonary hypoplasia (oligohydramnios, congenital diaphragmatic hernia [CDH])congenital diaphragmatic hernia [CDH])
Causes of HypoxemiaCauses of Hypoxemia in Neonatal Lung Diseasein Neonatal Lung Disease
IntrapulmonaryIntrapulmonary
Intrapulmonary shunting: Pulmonary arterial Intrapulmonary shunting: Pulmonary arterial blood that reaches pulmonary venous side blood that reaches pulmonary venous side without passing through ventilated areas without passing through ventilated areas of the lungof the lung
Ventilation:perfusion (V/Q) inequality: Ventilation:perfusion (V/Q) inequality: Imbalance between ventilation and Imbalance between ventilation and perfusion; increased deadperfusion; increased dead--space ventilationspace ventilation
Intrapulmonary Shunt and V/Q Intrapulmonary Shunt and V/Q MismatchMismatch
Relationship of Lung Disease Relationship of Lung Disease to Pulmonary Hypertensionto Pulmonary Hypertension
Pulmonary vascular resistance (PVR) Pulmonary vascular resistance (PVR) increases at lung volumes above and below increases at lung volumes above and below functional residual capacityfunctional residual capacity••
Underinflation in RDSUnderinflation in RDS
••
Overinflation in MASOverinflation in MAS
Extremes of lung volume contribute Extremes of lung volume contribute to high pulmonary vascular resistance to high pulmonary vascular resistance and extrapulmonary shuntingand extrapulmonary shunting
PVR Increases at Lung Volumes PVR Increases at Lung Volumes Below and Above FRCBelow and Above FRC
PVR
Lung Volume
Lung Recruitment With HFOV Augments Lung Recruitment With HFOV Augments Response to INO in Newborns Response to INO in Newborns With PPHN and Lung DiseaseWith PPHN and Lung Disease
HFOV+INOHFOV+INOINOINOHFOVHFOV
MASMAS RDSRDS IdiopathicIdiopathic
**
1010
2020
3030
4040
5050
Responders Responders (%) (%)
**
**
Kinsella et al, J. Pediatrics, 1997
End OrgansEnd OrgansHb has more affinity for Hb has more affinity for
curves to the left, which curves to the left, which means POORER means POORER oxygen deliveryoxygen delivery
(LEFT, L or LOW (LEFT, L or LOW suggesting LOW acid, suggesting LOW acid, LOW paCO2, LOW 2,3 LOW paCO2, LOW 2,3 DPG, LOW temp)DPG, LOW temp)
Hb has less affinity of Hb has less affinity of oxygen towards the oxygen towards the right, which means right, which means BETTER oxygen BETTER oxygen deliverydelivery
(RIGHT, R for release with (RIGHT, R for release with Inc 2,3 DPG, Inc H+ Inc 2,3 DPG, Inc H+ and Inc Temp)and Inc Temp)
BLOODBLOOD
HemoglobinopathiesHemoglobinopathies
Sickle cell diseaseSickle cell disease
Methemoglobinemia: KNOW THIS: the iron Methemoglobinemia: KNOW THIS: the iron of Hb changes from ferrous (reduced) to ferric of Hb changes from ferrous (reduced) to ferric (oxidized), decreasing ability of Hb to bind O2.(oxidized), decreasing ability of Hb to bind O2.••
So you get a normal paO2, but decreased oxygen So you get a normal paO2, but decreased oxygen saturationssaturations
ThalassemiaThalassemia
AnemiaAnemia
PolycythemiaPolycythemia
Initial Evaluation of the TermInitial Evaluation of the Term Newborn with CyanosisNewborn with Cyanosis
Term Newborn with Cyanosis
PrenatalPrenatal ultrasound study results
History of oligohydramnios and durationHistory of fetal brady/tachyarrhythmiaMaternal illnesses, drugs, medications
History of fetal distressRisk factors for infection
DeliveryHistory of positive pressure ventilation in DR
Meconium stained amniotic fluidHemorrhage
TraumaApgar score
History and Risk Factor AssessmentHistory and Risk Factor Assessment
Physical ExaminationPhysical Examination
Respiratory Distress(retractions, grunting, nasal flaring)
Suggests lung parenchymal disease (compliance),airway disease or metabolic acidemia
Physical exam
No Significant Respiratory Distress(tachypnea alone)
Suggests hypoxemia caused by cyanoticheart disease without lung disease
Physical Exam
Role of Pulse OximetryRole of Pulse Oximetry
Preductal / postductal SaO2
Preductal SaO2 < postductal SaO2
1) TGV with pulmonary hypertension2) TGV with coarctation of aorta
Preductal SaO2 > posductal SaO2
1) PVR>SVR with RL PDA: PPHN with MAS, RDS, CDH,
or idiopathic
2) Ductal-dependent Qs: HLHS, AS, IAA, coarctation
3) Anatomic pulm. vasc. disease: alveolar-capillary dysplasia, pulmonary venous stenosis, TAPVR with obstruction.
Preductal SaO2 = postductal SaO2
1) Intrapulmonary shunt:(PVR<SVR)
2) Cyanotic CHD with left-to-right PDA: (Ductal-dependent Qp: e.g. PS,
tricuspid atresia, Ebstein's)
3) PPHN: extrapulmonary shunt at FO:(PVR>SVR with closed DA)
Response to Supplemental OxygenResponse to Supplemental Oxygen
Marked improvement in SaO2
Parenchymal lung disease, CHD withductal-dependent systemic blood flow
Acute response to high FiO2 (hood, mask)
Minimal or transient change in SaO2
Cyanotic heart disease, PPHN
Acute response to high FiO2 (hood, mask)
CXR and Laboratory EvaluationCXR and Laboratory Evaluation
ABG: Assess respiratory and metabolic acidemia
CBC: For evidence of infection
BP: Ductal-dependent systemic blood flow and closing PDA: (e.g. coarctation, IAA)
Chest Radiograph ABG, CBC, 4 ext. BP
Hypoxemia out of proportion to radiographic changes
CHD with ductal-dependent pulmonary blood flow orextrapulmonary right-to-left shunting with PPHN
ABG, CBC, 4 ext. BPChest radiograph
Idiopathic PPHNIdiopathic PPHN
PneumoniaPneumonia
Meconium Aspiration SyndromeMeconium Aspiration Syndrome
Hyperoxia testHyperoxia test
ABG preABG pre--ductal sample from the right radial ductal sample from the right radial arteryartery
FiO2 0.21FiO2 0.21 FiO2 1.00FiO2 1.00
pO2 50 pO2 50 --
150150 pO2 50 pO2 50 --
150150 CardiacCardiac
pO2 50 pO2 50 --
150150 pO2 150 pO2 150 ––
300300 PulmonaryPulmonary
pO2 50 pO2 50 --
150150 pO2 > 300pO2 > 300 NormalNormal
Role of EchocardiographyRole of Echocardiography
Echocardiogram
RL shunting at PDA and/or FOwith normal LV performance
Consider iNO use aftereffective lung recruitment.
Anatomic abnormalities
Ductal-dependent systemic blood flow:HLHS, critical AS, coarctation, IAA
Functional abnormalites
LV dysfunction with mitral insufficiency:LR atrial shunting with RL ductal
shunting=pulmonary venous hypertension
? RV dependent systemic blood flow
Echocardiography
What do you want to know from an What do you want to know from an echo??echo??
Structural abnormalitiesStructural abnormalities
Heart function: LV dysfunctionHeart function: LV dysfunction
ShuntsShunts
Intracardiac (extrapulmonary)Intracardiac (extrapulmonary)••
PFOPFO
••
PDAPDA
Measurement of PA pressureMeasurement of PA pressure
Tricuspid regurgitationTricuspid regurgitation
Cardiopulmonary Interactions in PPHNCardiopulmonary Interactions in PPHN
(Structural changes; (Structural changes; altered reactivityaltered reactivity
to dilator and to dilator and constrictor stimuli)constrictor stimuli)
PVRPVR
RightRight--toto--left shunting left shunting at PDA and FOat PDA and FO
Hypoxia, hypercarbia, Hypoxia, hypercarbia, acidosisacidosis
LungLung Lung volumeLung volume
ComplianceCompliance
Intrapulmonary Intrapulmonary shuntshunt
PulmonaryPulmonary VascularVascular HeartHeart
(RV pressure (RV pressure overload and overload and LV LV
dysfunction)dysfunction)
SVR SVR
Relationship of Cardiac DysfunctionRelationship of Cardiac Dysfunction to Pulmonary Hypertensionto Pulmonary Hypertension
Left ventricular dysfunction causes mitral Left ventricular dysfunction causes mitral insufficiency, increased left atrial end diastolic insufficiency, increased left atrial end diastolic pressure, and pulmonary venous hypertensionpressure, and pulmonary venous hypertension
Increasing pulmonary blood flow does not Increasing pulmonary blood flow does not improve systemic oxygenation in this settingimprove systemic oxygenation in this setting
Lesion ClassificationLesion Classification
Too much pulmonary blood flowToo much pulmonary blood flow——50%50%
AcyanoticAcyanotic
Too little pulmonary blood flowToo little pulmonary blood flow
CyanoticCyanotic
Obstructive physiologyObstructive physiology
Low cardiac outputLow cardiac output——variable cyanosisvariable cyanosis
Transposition/mixing physiologyTransposition/mixing physiology
Variable to severe cyanosiVariable to severe cyanosi
CombinationsCombinations
DuctalDuctal
DependenceDependence
PDAPDA
Pulmonary blood flowPulmonary blood flow MixingMixing Systemic blood flowSystemic blood flow
Pulmonary Obstruction
Not ductal
dependent
TOF
Pulmonary stenosis
Ductal
dependent
Critical PS
Severe TOF
Pulmonary atresia
Tricuspid atresia
Systemic Obstruction
Not ductal
dependent
AS
Coarctation
Ductal
dependent
Critical AS
Critical coarctation
IAA
HLHS
Algorithm for diagnosis of CHDAlgorithm for diagnosis of CHD
Five “T’s”, “DO” and “ESP” = TGA, Tetralogy, TAPVR, Tricuspidatresia, truncus arteriosus, DORV, Ebstein’s, Single ventricle, and pulmonary atresia
Ductal
PBF Ductal
SBF Mixing
CardiacOutput
Perfusion Normal Decreased Normal to decreased
Acidosis Normal Severe Mild
Cyanosis Amount Moderate 70-80%
Mild80-90%
Severe40-70%
Differential None ?Decreased lower ext
Rare
CXR Heart size Normal Increased Normal
Vascularity Decreased Normal to increased
Increased
Transposition of the Great ArteriesTransposition of the Great Arteries
Know the difference between D and LKnow the difference between D and L--TGA (D has AV TGA (D has AV concordance)concordance)
Most common cyanotic lesion in first week of lifeMost common cyanotic lesion in first week of life
Greater in males 3:1Greater in males 3:1
TranspositionTransposition
““egg on a stringegg on a string””
Severe cyanosis at birth as Severe cyanosis at birth as a result of separate a result of separate circulations and poor circulations and poor mixing, particularly if mixing, particularly if restrictive PFOrestrictive PFO
Manage with PGE, Manage with PGE, Rashkind procedure if Rashkind procedure if inadequate mixing, and inadequate mixing, and eventual arterial switch eventual arterial switch (Jatene)(Jatene)
Tetralogy of FallotTetralogy of Fallot
Primary defect: Primary defect: underdevelopment of the right underdevelopment of the right ventricular infundibulm which ventricular infundibulm which causes:causes:
Anterior malalignment VSDAnterior malalignment VSD
Pulmonary stenosisPulmonary stenosis
OverOver--riding aortariding aorta
Right ventricular hypertrophyRight ventricular hypertrophy
Associated with right AA(25%), Associated with right AA(25%), 22q11 (16%), Trisomy 21 (7%), 22q11 (16%), Trisomy 21 (7%), Alagille SyndromeAlagille Syndrome
TetralogyTetralogy
““boot shaped heartboot shaped heart””
Spectrum of clinical presentation is Spectrum of clinical presentation is related to degree of RVOTrelated to degree of RVOT
Hypercyanotic spells, or Hypercyanotic spells, or ““tettet””
spellsspells
Balance between SVR and PVRBalance between SVR and PVR
↓↓PVR: comfort, feed, sedate, oxygenPVR: comfort, feed, sedate, oxygen
↑↑SVR: knee to chest, phenylephrineSVR: knee to chest, phenylephrine
PGE, palliative BT shunt to eventual PGE, palliative BT shunt to eventual pulmonary arterioplasty and VSD pulmonary arterioplasty and VSD patchpatch
Tricuspid Tricuspid AtresiaAtresia
Complete absence of Complete absence of tricuspid valvetricuspid valve
With minimal VSD: leads With minimal VSD: leads to poor RV development to poor RV development because of lack of blood because of lack of blood flow in utero, ductal flow in utero, ductal dependent, requires shuntdependent, requires shunt
With VSD: VSD With VSD: VSD determines size of RVdetermines size of RV
PA is commonPA is common
Tricuspid Tricuspid atresiaatresia
PBF is supplied by L to R shunt at PDA or VSDPBF is supplied by L to R shunt at PDA or VSD
If baby has small VSD or PSIf baby has small VSD or PS——need PGEneed PGE——may need BT may need BT shuntshunt
If large VSD and no PS, as PVR falls, may have If large VSD and no PS, as PVR falls, may have increased PBF and be at risk for CHFincreased PBF and be at risk for CHF——may need PA may need PA bandband
TruncusTruncus
arteriosusarteriosus
Single great vessel Single great vessel gives rise to both gives rise to both aorta and aorta and pulmonary arteriespulmonary arteries
Large VSD is Large VSD is always present, always present, leading to complete leading to complete ventricular mixingventricular mixing
As PVR falls, further As PVR falls, further increase in PBFincrease in PBF
TruncusTruncus
arteriosusarteriosus
Cyanosis and CHF Cyanosis and CHF depends on PBFdepends on PBF
Clinical signs: low Clinical signs: low diastolic pressure, diastolic pressure, widened pulse pressure widened pulse pressure due to diastolic runoff due to diastolic runoff into PA, loud murmur, into PA, loud murmur, increased heart sizeincreased heart size
EbsteinEbstein’’ss
anomalyanomaly
Tricuspid valve is Tricuspid valve is displaced into RVdisplaced into RV
Portion of RV wall above Portion of RV wall above the displaced leaflets the displaced leaflets becomes becomes ““atrializedatrialized””
TV regurgitationTV regurgitation
AtrialAtrial
communication if communication if 80% with R to L shunt, 80% with R to L shunt, thus requires PDA for thus requires PDA for adequate PBFadequate PBF
EbsteinEbstein’’ss
AnomalyAnomaly
If severe, cyanosis If severe, cyanosis secondary to decreased secondary to decreased PBFPBF
CXR shows dramatic CXR shows dramatic cardiomegalycardiomegaly
Treat with PGE if need Treat with PGE if need to increase PBG, treat to increase PBG, treat CHF, assess for airway CHF, assess for airway compression, limited compression, limited surgical optionssurgical options
TAPVRTAPVR
SupracardiacSupracardiac
(most common): (most common): PV via vertical vein to PV via vertical vein to innominateinnominate, , azygousazygous
or directly or directly to SVC, to SVC, ““snowman CXRsnowman CXR””
Cardiac: PV into RA directly or Cardiac: PV into RA directly or indirectly via coronary sinusindirectly via coronary sinus
InfracardiacInfracardiac: PV cross : PV cross diaphragm and drain into portal diaphragm and drain into portal vein or hepatic vein or IVCvein or hepatic vein or IVC
MixedMixed
TAPVRTAPVRCharacteristicsCharacteristics Obstructive TAPVRObstructive TAPVR NonobstructiveNonobstructive
TAPVRTAPVR
PatencyPatency InfracardiacInfracardiac
((subdiaphragmaticsubdiaphragmatic) ) almost always obstructivealmost always obstructive
Majority of Majority of intracardiacintracardiac
and and supracardiacsupracardiac
Clinical findingsClinical findings Pulmonary edema, CHFPulmonary edema, CHFCyanosis, respiratory distressCyanosis, respiratory distressDecreased systemic perfusionDecreased systemic perfusion
Increased PBF, CHFIncreased PBF, CHFMild to moderate cyanosisMild to moderate cyanosisSystolic murmurSystolic murmur
CXRCXR Normal or small heart because Normal or small heart because decreased PBFdecreased PBFLung fields similar to aspiration PNALung fields similar to aspiration PNA
Increased heart size, Increased heart size, pulmonary congestionpulmonary congestion
ECGECG RVH, or may be normalRVH, or may be normal Right axis deviation, RVH, Right axis deviation, RVH, RAERAE
ManagementManagement Medical treatment of CHFMedical treatment of CHFTreatment with PGE may worsen Treatment with PGE may worsen symptoms by increasing PBF, in symptoms by increasing PBF, in some cases PGE may be helpful by some cases PGE may be helpful by dilating dilating ductusductus
venous and improving venous and improving venous return, surgery is venous return, surgery is urgenurgen
Medical treatment of CHFMedical treatment of CHFSurgery not always needed Surgery not always needed immediatelyimmediately
DORVDORV
Aorta and pulmonary Aorta and pulmonary artery arise from RVartery arise from RV
VSD is required to VSD is required to provide an outlet provide an outlet from LV, and defines from LV, and defines type of DORVtype of DORV
VSD can be VSD can be subaorticsubaortic
(TOF or (TOF or
VSD), VSD), subpulmonicsubpulmonic (TGA), doubly (TGA), doubly
committed or remotecommitted or remote
Single ventricleSingle ventricle
Both AV valves empty Both AV valves empty into as single ventricleinto as single ventricle
Think tricuspid Think tricuspid atresiaatresia physiology or HLHS physiology or HLHS
physiologyphysiology
Oxygen saturations and Oxygen saturations and clinical symptoms clinical symptoms depend on balance depend on balance between between QpQp/Qs/Qs
If increased PBF: CHF, If increased PBF: CHF, mild cyanosismild cyanosis
If decreased PBF: If decreased PBF: similar to TOF with similar to TOF with moderate, severe moderate, severe cyanosiscyanosis
Board questionBoard question
A full newborn has moderate respiratory distress and A full newborn has moderate respiratory distress and is cyanotic. The pO2 from the umbilical artery catheter is cyanotic. The pO2 from the umbilical artery catheter is 80 mmHg, at the same time the pO2 from the is 80 mmHg, at the same time the pO2 from the umbilical venous catheter is 200 mmHg. What is the umbilical venous catheter is 200 mmHg. What is the most likely diagnosis?most likely diagnosis?
A.A.
Persistent fetal circulation (pulmonary hypertension)Persistent fetal circulation (pulmonary hypertension)B.B.
TGATGAC.C.
TruncusTruncus
arteriosusarteriosusD.D.
InfraInfra--diaphragmatic TAPVRdiaphragmatic TAPVRE.E.
HLHSHLHS
Answer:DCBA
AaDO2 = (713 x FiO2) – (pCO2 / 0.8) – (paO2).
L to R shunt if Qp/Qs >1R to L shunt if Qp/Qs < 1
The answer is D.
The answer is D.The answer is D.
The answer is B.The answer is B.
27.27.
DD28.28.
EE
29.29.
AA30.30.
CC
The answer is BThe answer is B
The answer is A.The answer is A.
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