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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