dibu's approach to congenital heart disease
TRANSCRIPT
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Approach toCongenital Heart Diseases
Dr. Dibbendhu Khanra
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Disclosures
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What do we get?
• cyanosis• murmur• cardiomegaly• dextrocardia in CXR• CHB
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What do we want to know?
• Situs/ position?• Congenital heart disease?• Severity and operability?
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What do we have?
• Clinical features• CXR• ECG
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Today’s agenda
• Fetal circulation• Hemodynamics • Heart sounds and murmurs• ECG and CXR in cong heart disease• Classification of cong heart disease• Cardiac malpositions
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Foetal circulation
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Pressures
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Congenital heart disease
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Our approach
• When to suspect heart disease in a child?• When can we say it is congenital?• Classification of cyanotic cong heart disease• Classification of acyanotic cong heart disease• To assess severity (whether to intervene?)
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Is there any heart disease?
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Murmers
• Systolic• Diastolic• Continuous
Age • From birth: obstructive or regurgitation• Late appearance:- ASD 6m- VSD/ PDA ESM from 3-4 days shunt murmur from 6-10 wks
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Systolic murmur (acyanotic)
Pan systolic murmer• Same intensity• S1 not clear• Always organic• Limited differentials- MR (apical)- TR (parasternal)- VSD (thrill)
Ejection systolic murmer• Diamond shaped• S1 must be clear• Organic if- More than grade III- Cyanosis- Click- Cardiomegaly - Abnormal S2
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Pan-systolic murmur (cyanotic)• VSD- TA+ VSD+PS- DORV+VSD+PS• TR- ASD+ PS- Ebstein’s- AVCD + PS- ASD + severe PAH (Eisenmenger’s)- AVCD + severe PAH (Eisenmenger’s)• MR- AVCD + severe PAH (Eisenmenger’s)- AVCD + PS
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Diastolic Murmur•AR•PREarly •Stenotic lesions (MS/ TS)•Flow murmers (L-R shunt)Delayed
•Presystolic accentuation of delayed diastolic murmerLate
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Early diastolic PR murmurHypertensive• Common • PAH+• P2 loud• Parasternal heave+• Decrescendo
Non-hypertensive• Uncommon • PAH-• P2 soft• Parasternal heave absent• Diamond shaped• “Ejection shaped diastolic murmer”
- Absent pulm valve- PA dialated- Post-operative Fallot/ PS- Pulm valve IE
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Continuous murmur (acyanotic)Always organic
High pressure to low pressure (chamber or vessel)S2 is drowned
Aortic run-off
PDA - Grade 2-4 - systolic comp harsh - LSB + infraclavicular RSOV - Grade 5-6 - systolic comp loud in ass VSD - Diastolic comp loud in ass AR
No aortic run-off
- Coronary AVF (smooth systolic component)
- Systemic AVF (murmer is ‘away and delayed’; A2 audible)
- ALCAPA (ECG: ALWMI pattern)
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• Bronchial collaterals in Fallot’s• PDA in Fallot’s• Supracardiac TAPVC• Pulm AVF• PTA• Surgically created shunts
Continuous murmur (cyanotic)
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Abnormal S2“key to auscultation”
• Three components: A2, P2, split• First three months: P2>A2• In AS, A2 is never diminished until valve is immobile• In PS, P2 is usually normal or diminished d/t decreased PBF• Splitting is related to movement of P2
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Variation of P2
P2 NORMAL P2 LOUD P2 DIMINISHED P2 ABSENT
Isolated PS Associated PAH(Both hyperkinetic and obstructive)
Pulmoanry artery dialated
PS + ASD(Diminished PBF)
Dystrophic Pulmonary valve (Noonan’s syndrome)
Fallot’s (single S2)
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Pulmonary Hypertension (loud P2)HyperkineticIncreased PBF
• L-R shunt• Transposition physiology• Volume overload• Wide split- Cardiomegaly- Parsternal heave- Murmer (flow and shunt)
Obstructive Decreased PBF
• Eisenmenger’s • Obstructive physiology• Click may be present• Single P2
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Abnormal split
A2 early P2 delayed
P2 early A2 delayed
Wide split
Paradoxical split
MRVSD
LVOTOPDALVF
PSASD
TAPVC
TRPAH
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Abnormal sounds
S3• Early diastolic • Volume overload- L-R (post-tricuspid)- Severe regurgitation- LVF
S4• Late diastolic• Pressure overload- AS- PS
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Ejection Click Pulmonary Aortic
Inconstant Constant Constant
Valvular PS Obstructive PAH
Pulm art dialated
Valvular AS?
Bicuspid Aortic valve
Dialated asc aorta d/t
- Aneurysm- Fallots- PTA
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Cyanosis
• Bluish discoloration• Clubbing• Polycythemia• Absent thymus • SpO2 low and not corrected with O2
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Hypoxic spells• Crying, feeding, deep sleep• 2m-2yrs• rate and depth of breathing• Deepening cyanosis
Squatting • To relieve SOB• Grown up children• During exercise/ sports
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Heart failure
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Arrythmia
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ECG• Age• Cyanosis• Heart size in CXR
• Axis• LAE/ RAE• LVH/ RVH
Fallot’s Transposition Eisenmenger’s
RAD RVH TOF (90-120)DORV (>150)TGA
TGATAPVC
VSDPDAAPWOS-ASD
RAD LVH SV RV SV RV SV RV
LAD LVH TA TA TA
LAD RVH SV AVC SV AVC SV AVC
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CXR
• Situs/ position• Thymus • Cardiomegaly • MPA• Aortic arch • Aortic knuckle• Pulmonary vascularity• Rib notching
• Age • Cyanosis
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Is there any heart disease?
CONTINOUS MURMER
CLICK
ABNORMAL P2 or SPLIT
CARDIAC ARRYTHMIAHYPOXIC SPELLS
RVH IN ECG
CARDIOMEGALY WITH ABNORMAL PULM VASCULARITY
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Is it congenital?
• Low age group• FTT/ CHF at pediatric age• Parasternal murmer• Central cyanosis• Cardiac arrythmia• Extracardiac abnormalities• Infection/ drug intake during pregnancy
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Classification
• Cyanotic• Acyanotic
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Cyanotic CHD
PULMONARY STENOSISPulm ESM
NOPULMONARY
STENOSIS
NO VSD VSD PULMONARYHYPERTENSION
NOPULMONARY
HYPERTENSION
INCREASEDPBF
DECREASEDPBF
PULMONARYVENOUS
HYPERTENSION
ASD+PS(Triology)
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Fallot’sPhysiology
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Transposition physiology
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Eisenmenger’s physiology
4Obstructive
TAPVC5
PAVFSV to LA
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Acyanotic CHD
L-R• Pre-Tricuspid (ASD)• Post-Tricuspid (VSD, PDA)
Obstructive• Aortic/ pulmonary Stenosis- Valvular- Supravulvular- Subvalvular• CoA
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Congenital Cyanotic heart disease
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PS + Cyanosis (R-L shunt)NO VSD• ASD + PS (triology)• Ebstein’s + PS
VSD• TOF• DORV + VSD + PS• SV + VSD + PS• TA + VSD + PS
No hypoxic spells Hypoxic spellsJVP RAISED JVP NORMAL
CARDIOMEGALY NO CARDIOMEGALYPARASTERNAL IMPULSE NO PARASTERNAL IMPULSE
WIDE SPLIT SINGLE S2DIASTOLIC SOUND DIASTOLE SILENT
LONG ESM SHORT ESMDelayed transition in ECG Early transition in ECG
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R-L shunt at atrial level
ASD + PS (TRIOLOGY)• Commoner • RV apex• Parasternal heave faint• S1S2• Incomplete RBBB with R in VI>15mm
• Enlarged pulmonary artery
EBSTEIN’s anomaly of TC valve• Rare• LV apex• No parasternal heave• 4 or more sounds• Complete RBBB with R in VI<7mm• Broad S in V6• Abnormal P• Box - heart & narrow pedicle
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Fallot’s physiologydifferential diagnosis by ECG
• TOF: RVH+ RIGHT AXIS 90-120’• DORV: RVH+ RIGHT AXIS > 150’ (D/D TGA+ FALLOT)
• SV: RVH+ LEFT AXIS / LVH+ RIGHT AXIS• TA: LVH + LEFT AXIS
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Cyanosis without PScyanosis + loud P2
Transposition Eisenmenger’s PulmonaryVenous
Hypertension
Cardiomegaly Yes No No Pulm vascularity Increased Decreased Increased PAH Hyperkinetic Obstructive Obstructive Age Neonate Adult Neonate Failure Present Absent Present Parasternal heave Present Absent Absent Ejection click Absent Present (constant) Abesnt Murmer Present Absent Absent CXR Egg on end Large hilar vessel
with sudden cut-offGround glass
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Eisenmenger’sASD VSD PDA
Cyanosis Uniform Uniform Differential JVP May be raised Not raised Not raisedSuprasternal pulse
No No Present
Parasternal heave
Present No No
S2 Wide fixed Single Normal splitTR Present Absent Absent
Cardiomegaly May be present No No
Ascending aorta Normal Normal Large
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Cyanosis Normal ausculatation
Normal ECGNormal CXR
SVC to Left atrium Pulmonary AVFContrast Echo LA-RA (no ASD) RA-LA
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Congenital Acyanotic heart disease
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Acyanotic Congenital Heart Disease
L-R shunt• Volume overload(forceful ill-sustained apex)• Precordial bulge• CXR – Cardiomegaly• Flow/ shunt murmer• No thrill• Pulmonary plethora on CXR• Dilated right pulmonary
artery
Obstructive • Pressure overload(forceful well-sustained apex)• Normal Precordium• ECG – Ventr. Hypertrphy• Ejection click/ murmer• Thrill• Normal vasculature in CXR• Normal right pulmonary
artery
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L-R shunt
No pressure differenceL-R shunt at RV diastole
RV diastolic vol overload
Depends on sizeL-R shunt at RV systole
LV diastolic vol overload
Depends on sizeDP both systole & diastoleLV diastolic vol overload
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L-R shunt• PVR dependent• Pressure dependent (obligatory)
PAH• Increase PBF + large L-R shunt = hyperkinetic (loud P2, split)• Increased PVR + small L-R shunt = obstructive (loud P2, single P2)
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L-R shunt
Pre-tricuspid • ASD• No shunt murmur• RV volume overload• Parasternal heave• Apex – RV type• RBBB/ Dilated PA, RA• Forward flow maintained• Less symptoms
Post tricuspid • VSD, PDA• Shunt murmur• LV volume overload• No parasternal heave• Apex – LV type• LVH/ BVH• Forward flow compromised• symptomatic
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ASD
OS-ASD• Commoner • No MR• Right axis• Eisenmenger’s late
OP-ASD• Less common• MR• Left axis deviation• Early Eisenmenger
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ASD ALOGARITHM
ASD
Clockwise loop
II° ASD P -wave axis normal
Crochetage+
SV ASDP- wave axis
superiorCrochetage+
CounterclockwiseLoop
I° ASDLAD/Notching of s in inf leads
LVH/LAE
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Post-tricuspid L-R shunt
VSD• Holosystolic murmer• Wide split• No click
PDA• Continuous murmer• Normal split• Constant click• Dilated aortic knuckle• Dilated MPA• Aortic runoff• Early Eisenmenger’s
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Specific about VSDVSD S2
Shunt PAH Split P2L-R No Wide Normal
Large L-R Yes Wide Accentuated
Small L-R Yes Normal Accentuated
R-L (Eisenmenger’s)
Yes Single S2 (fusion of A2 and P2)
Loud P2
L-RMild to mod PS
- Wide Diminished
R-LSevere PS (Fallot’s)
- Single S2 (A2) Inaudible
Small defect Normal Normal
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LOCATIONPERIMEMBRANOUS VSD
INLET VSD MUSCULAR VSD
With septal aneurysm-left axis deviation
Counterclockwise loop, LAD and prolonged PR interval
Clockwise loop with left axis deviation
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VSD
LEFT AXIS
Clockwise loop-L-TGA
MULTIPLE MUSCULAR
Counter clockwise-DORV
INLET VSDTRICUSPID ATRESIA
RAD
Severe PAH
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VSD
LVH
MODERATELY RESTRICTIVE
WITH RAE-GERBODES
BVH
NONRESTRICTIVE
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NONRESTRICTIVE-BVH
Q IN LATERAL LEADS
PRESENT-simple VSD ABSENT-LTGA
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Obstructive diseaseLVOTO
• Heaving apex• systolic thrill at aortic area• Paradoxical split• A2 loud until calcified• S4 (LVEDP 13 mmHg)• Narrow pulse pressure/ low vol pulse
• Constant ejection click• ESM with radiation• Dilated asc aorta• LVH
RVOTO
• Left parasternal heave• Systolic thrill at pulm area• Wide split• P2 normal to soft• S4• Tall a wave in JVP• Loud inconstant click• Ejection systolic murmur• Post stenotic dilation of MPA• RVH (delayed transition at V5,V6)
Dysplastic pulmonary valve1. noona’s2. FH3. ESM absent4. P2 absent5. Pulm art not dialated in CXR6. ECG – LEFT axis
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Aortic StenosisValvular Supravulvular Subvulvular
Constant ejection click(until valve is calcified)Murmer at 2nd LICS
Higher pressure in right arm compared to left arm
No clickMurmer at LSBDynamic auscultaitonAR may be present
Pulmonary StenosisValvular Infandibular Subinfandibular
Murmer at 2nd LICS Murmer at 3rd, 4th LICS Murmer at 4th 5th LICSOr subxiphoid
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Coarctation of aorta• Systemic hypertension• Prominent suprasternal pulsation• Femoral pulse weak and delayed (>15 ms)• BP in upper limb is higher (> 15-20 mmHg)• Palpable collateral over chest walls• LV type apical impulse• Palpable suprasternal pulsation• Constant aortic ejection click• ESM at left inters-capular area (T4-T5)• Continuous murmur for collaterals• Strain in LVH before 15 years of age (ECG)• Prominent ascending and arch of aorta (FIGURE OF 3)• Rib notching (R3-R8) appears around 10 years of age
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Assessment of severity
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L-R shuntASD VSD PDA
Heart size - Cardiomegaly Cardiomegaly
Split Wide fixed always Wide Paradoxical
S3 (shunt 1.5-2:1)
S3 S3
Flow mumer(shunt >2:1)
Tricuspid Mitral Mitral
Shunt murmer Absent Ejection systolic murmer
Systolic murmer
Hyperkinetic PAH + + +
Cyanosis + + +
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PAH & operability
• Hyperkinetic PAH• Age • Lesion • Cardiomegaly and plethoric lung field in CXR• Flow murmur• SpO2
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Obstructive disease
LVOTO• Symptoms (incl failure)• S4• Length of murmur• Delayed peaking of murmur
• Paradoxical split• LVH in ECG• low vol & late peaking pulse• Narrow pulse pressure
RVOTO• Symptoms • S4• Length of murmur (crosses A2), also
intensity• Might peak later in systole to assume
an asymmetric kite shape• Delayed peaking of murmur• S1-click interval low• Width of the split (A2 may be soft)• RVH in ECG• Large a wave in JVP• Presystolic hepatic pulsation• f/o RV failure, TR murmer, v wave in
JVP
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ECG SEVERITY OF PSMILD MODERATE SEVERE Gradient 40mm mmHg RVSP<50% of LVSP
Right axis <100°
R in v1<10-15mm
Upright right precordial T waves
Gradient>40 mm Hg RVSP>50% of LVSP
Right axis 100-150
R in v1 <20mm
rsR’ or a small r is present on upstroke of R’
r/s in v1>4:1
50%-upright T waves
RVSP=LVSP or more Gradient >80 mm Hg
RAD>150°
R >20mm inV1
Monophasic R or qR P in lead 2 tall and
peaked, P maybe negative in V1
Deep inverted T waves ST depression beyond v2 T wave axis leftward QRS axis rightward QRT-T angle widens
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Cyanotic congenital heart disease
• Degree of cyanosis• Age• Symptoms• Failure• Cardiomegaly• PAH
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Cardiac malpositions
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Nomenclature
Situs • Position of thoracic and
abdominal viscera- liver/ spleen, fundal gas- Bronchus - Aortic arch
Cardiac position• Position of heart in which
hemithorax (in CXR)• Cardiac apex• Hemidiaphragm attached to
cardiac apex will be lower
Loop - Bend of heart tube- LV/ RV- D/L: Relative position of
Ao/PA
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Cong cyan heart disease
Systemic or pulmonary venous
abnormalities
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Atrio ventricular
discordance
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Polysplenia syndrome
Asplenia syndrome
Situs solitus + dextrocardiaSitus inversus + levocardia
Centraly placed heartCentrally palpable liver
Symmetrical tracheaRatio of left bronchus is to right bronchus 1.5 or less
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Identification
• Right and left atrium• Right and left ventricles• D/L loop• Great arteries• Ventriculo arterial connections
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Right and left atriumClinically • Liver/ right atrium• Stomach/ spleen/ left atriumCXR• Fundal gas• Bronchus ECG• P in I, aVL and aVR• P wave axisEcho• Aorta/ IVC• IVC to RA• Coronary sinus in LA• Appendage • Contrast
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D-L loop
CXR• AortaECG• Q in V1 V2Echo
D-Lloop
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Right and left ventricleClinically • Apex• Split S2CXR• apex/ DiaphragmECG• R wave progressionEcho• LV – thick walled papillary muscle• RV – corase trabeculations & moderator band• TC valve attachment is more apical than Mitral valve• Septal valve in TC• Fish mouth mitral valve• Mitral valve posterior to TC valve
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Great arteries
NORMAL DTGA LTGA
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TGA
Complete TGAWith restrictivePFO
Complete TGAWith nonrestrictiveVSD
DynamicSubpulmonary stenosisnonrestrictiveVSD
Complete TGAWith nonrestrictiveASD
Complete TGAWith nonrestrictivePDA
Cyanosis cyanosisFrom D1
Mild cyanosis Cyanosis MILD CYANOSIS
Mild to mod
Failure No + No - +
Bounding pulse - -- + No No
Apex LV LV RV
S2 Loud single A2 Loud singleA2
Murmur NIL HSM (LSB) PESM (weeks) No No cont murmer
CXR N Egg shaped Wide pedicle Egg shaped
ECG N RAD/ BVH RAD/ RVH RAD/ RVH
PVOD Early = TOF
1. Male2. Large birth weight3. Neanatal cyanosis/ failure
4. Increased PBF/ egg on side/ narrow pedicle
5. Absent thymus6. Echo confirmation
Triuspid atresia by Kuhne
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TANormal GARESTRICTIVE VSD
DTGAnonRESTRICTIVE VSD
Associated PS
Cyanosis Prominent Mild cyanosis Mild to severe
Failure prominent
murmur HSM HSM PESM
Apex RV LV LV
ECG Tall pLADCounterclockwise depolarisationAdult progression of precordial QRS
Biatrial PAdult progression of precordial QRS
TALL PAdult progression
CXR Hump shaped rt border Narrow pedicleHigh PBF
Narrow pedicleLow PBF
Echo Echodense band at atretic TCVRestrictive VSDSmall RVN GARestrictive PFO/ ASD
Echodense band at atretic TCVRestrictive VSDWell formed RV
atretic TCVTGANR VSD
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Summary
• Age• Cyanosis• S2/ split• Murmur• CXR – PBF• ECG – axis• Echo – great arteries• Abdominal viscera
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Thank you