Clinical congenital heart Clinical congenital heart diseasedisease

Prof M S Ranjit MD DCHProf M S Ranjit MD DCH

Senior consultant paed. CardiologistSenior consultant paed. Cardiologist

Chennai.Chennai.

Some clinical aspectsSome clinical aspects“paediatric and adolescent accent”

Classification (modified for simplicity)Classification (modified for simplicity)

cyanotic - with ↑ pulm blood flow - with ↑ pulm blood flow

- with ↓ pulm blood flow- with ↓ pulm blood flow

- unclassifyiable – - unclassifyiable – ebsteins/ TGA IVS

acyanotic – largely shunt lesions – largely shunt lesions stenotic - - outflow & arterial obstructions

CyanosisCyanosiscaused by > 5gm/dl reduced Hb

Clinical detection depends onClinical detection depends on

- % arterial blood that is desaturated- % arterial blood that is desaturated

- Hb Concentration !!- Hb Concentration !!

If art O2 satn is 60%, If art O2 satn is 60%,

cyanosis is detectable if Hb > 12.5gm/dl !cyanosis is detectable if Hb > 12.5gm/dl !

but not if Hb < 10 gm/dl !but not if Hb < 10 gm/dl !

ie 4gm/dl insufficient for detection of cyanosis !

Detection of cyanosisDetection of cyanosis

Astute physician/ paed cardiologistAstute physician/ paed cardiologist

detects when reduced Hb 3 gm/dldetects when reduced Hb 3 gm/dl

Others detect at 5gm/dlOthers detect at 5gm/dl

Better to overdiagnose than underdiagnose ! !

Clinical diagnosis of cyanosis is inaccurateClinical diagnosis of cyanosis is inaccurate

M Tynan in Andersons Paediatric cardiology 2007M Tynan in Andersons Paediatric cardiology 2007

Cyanosis -some aspectsCyanosis -some aspects

Some CCHD with Rt to Lt shunt and ↑ P B flowSome CCHD with Rt to Lt shunt and ↑ P B flow

UO TAPVR/UO TAPVR/ Truncus/ TGA-VSD/ Single ventr Physiol etcTruncus/ TGA-VSD/ Single ventr Physiol etc

- may have low saturations - may have low saturations

- but undetectable cyanosis clinically- but undetectable cyanosis clinically

i.e. 88-92% !!

Polycythemic patients appear cyanosedPolycythemic patients appear cyanosed Methhaemoglobinaemia !!Methhaemoglobinaemia !!

Hyperoxic testcyanosed or not

Pulse oximeter - not always reliable

“a random number generator”

Rt radial ABG Rt radial ABG in airin air and and after 5-10 min O2after 5-10 min O2

paO2 > 250mmHg -excludes CCHDpaO2 > 250mmHg -excludes CCHD

paO2 > 160 -CCHD unlikelypaO2 > 160 -CCHD unlikely

( UO TAPVR False negative !)( UO TAPVR False negative !)

paO2 < 100 -CCHD likely paO2 < 100 -CCHD likely (usually lower)

((severe Lung disease severe Lung disease (high paCo2), PPHN/PFC) PPHN/PFC)

““Radial ABG more useful than ECG or CXRRadial ABG more useful than ECG or CXR

in detection of cyanotic heart disease”in detection of cyanotic heart disease”

Warburton 1981Warburton 1981

C C H Din 3 major circumstances

Pulmonary obstructionPulmonary obstruction with with avenue for right to avenue for right to left shuntingleft shunting

Discordant AV connectionDiscordant AV connection i.e transpositionsi.e transpositions

CommonCommon mixing mixing situations situations i.e common atrium i.e common atrium

single ventricle etcsingle ventricle etc

Unusual causes of cyanosisUnusual causes of cyanosiswithout murmurs !without murmurs !

surviving to adolesc./ adult lifesurviving to adolesc./ adult life

Left SVC to LALeft SVC to LA IVC to LAIVC to LA Rt. SVC to LARt. SVC to LA Pulm. AV Fistulae Pulm. AV Fistulae (Ostler Rendu Weber syndr)(Ostler Rendu Weber syndr)

LV

Cyanosis – which category?

SymptomatologySymptomatology

Clinical examinationClinical examination

Chest X rayChest X ray

Fallot physiology

Systemic venous return Systemic venous return unable to reach lungsunable to reach lungs

Shunted Shunted right to leftright to left away from pulm circulation away from pulm circulation

ASD/VSDASD/VSD essential for this to occur; essential for this to occur;

Or a Or a common chambercommon chamber ! !

PLUSPLUS

Fallot physiology

Obstruction atObstruction at - RA outlet - i.e Tric atresia

- infund/valvar Pulm stenosis

- rarely branch PA stenosis/ DCRV

- High PVR – Eisenmenger !

obstructed pulm arterioles !!

CCHD with ↓↓ pulm blood flowpulmonary oligaemia on CXR

SymptomatologySymptomatology

Inspection findingsInspection findings

Auscultatory findingsAuscultatory findings

Chest SkiagramChest Skiagram

CCHD with ↓ PBF - symptoms

Exertional dyspnoeaExertional dyspnoea Cyanosis, spells, seizuresCyanosis, spells, seizures CNS complicationsCNS complications

No recurrent RTI/ no diaphoresisNo recurrent RTI/ no diaphoresis No breathlessness at restNo breathlessness at rest

except in extremes / anaemiaexcept in extremes / anaemia

CCHD - ↓ PBF - inspection /palpatory findings

Cyanosis & clubbingCyanosis & clubbing polycythemiapolycythemia Quiet precordium to inspection & palpationQuiet precordium to inspection & palpation No Harrisons sulcus or precordial bulgeNo Harrisons sulcus or precordial bulge Apex well within limits if visibleApex well within limits if visible

No palpable sounds or thrillsNo palpable sounds or thrills

CCHD with ↓ PB Flowauscultatory findings

Normal first heart soundNormal first heart sound Single second heart soundSingle second heart sound Pulm component inaudiblePulm component inaudible

Stenotic pulmonary murmurStenotic pulmonary murmur

slightly after S1slightly after S1

stops short of S2stops short of S2 Other murmurs – Other murmurs – ductal/ MAPCA/ AR ductal/ MAPCA/ AR

Ejection murmur in Fallot physiology

Length & loudness inversely proportional to Length & loudness inversely proportional to severity of stenosisseverity of stenosis

In isolated PVS – the opposite !In isolated PVS – the opposite !

Absent murmurAbsent murmur – – acquired pulm atresiaacquired pulm atresia

- MAPCA murmur over back- MAPCA murmur over back - soft ductal murmur (tortuous)- soft ductal murmur (tortuous)

To & FroTo & Fro – – Aortic regurg / Abs PV syndromeAortic regurg / Abs PV syndrome

MAPCASMAPCAS

CCHD withCCHD with Pulm.blood flow Pulm.blood flow

Tetralogy of FallotTetralogy of Fallot VSD - PSVSD - PS DORV – VSD – PSDORV – VSD – PS Tricusp. atresia - PSTricusp. atresia - PS Single ventricle - PSSingle ventricle - PS TGA with VSD – PSTGA with VSD – PS Corr.transp.-VSD-PSCorr.transp.-VSD-PS ASD - PSASD - PS

Chest skiagram in CCHD with ↓ PBF

Small heartSmall heart

Pulmonary bayPulmonary bay

Pulmonary oligaemiaPulmonary oligaemia

Right aortic arch/ RA enlargement/ differential Right aortic arch/ RA enlargement/ differential vascularity/ narrow pedicle in various defectsvascularity/ narrow pedicle in various defects

Typical - Fallot CXR

Pulm bay

RV apex

Pulmonaryoligaemia

PFO / ASDVSD / PDA

Fallot physiology

Fallot physiologyFallot physiology

Tricuspid atresia

Fallot physiology

Fallot physiology

Fallot physiologyS2 variablePulm ESM

Corrected Transposition with VSD and PS

Atrio-ventricular &ventriculo-arterialDiscordance

LV

RVRA

LA

AO

PA

Fallot Physiology

Fallot physiologySingle S2Loud A2pulmonic ESM

CNS complications of CCHD with ↓ PBF

Paradoxic embolusParadoxic embolus Cerebral thrombosisCerebral thrombosis Cerebral abcessCerebral abcess SeizuresSeizures Hypoxic damageHypoxic damage Endocarditis & vegetationsEndocarditis & vegetations Postoperative strokesPostoperative strokes

CCHD with ↑ pulm blood flow

TranspositionsTranspositions with VSD/Duct/ASD with VSD/Duct/ASD

Common mixingCommon mixing situations situations

atrial level – atrial level – TAPVR/Comm Atr

Mixing atMixing at ventric level – ventric level – DORV/Single ventricDORV/Single ventric

arterial level – arterial level – comm art trunkcomm art trunk

Mild cyanosis, CCF, resp symptoms, ex dyspnoea

CCHD with ↑ Pulm blood flow

Seldom survive to adolescence/ adulthoodSeldom survive to adolescence/ adulthood UO TAPVR/ comm atrium- the exceptionsUO TAPVR/ comm atrium- the exceptions

Most have Eisenmenger by thenMost have Eisenmenger by then

and those features dominateand those features dominate

CCHD ↑ P B Floweasy diagnosis – rare

Clinical differentiation not always possible (Tynan M, Andersons paed cardiology 2007)(Tynan M, Andersons paed cardiology 2007)

Brisk pulses, ej click, to& fro murmur – TruncusBrisk pulses, ej click, to& fro murmur – Truncus

Sm. pulses, RV impulse, wide split S2,TV MDM – TAPVRSm. pulses, RV impulse, wide split S2,TV MDM – TAPVR

AV regurg murmur, wide split, TV MDM – comm. atriumAV regurg murmur, wide split, TV MDM – comm. atrium

Sing S2, cont murmur over back – p atr / Sing S2, cont murmur over back – p atr / MAPCASMAPCAS

CCHD with ↑ P B Flow - symptoms

Respiratory symptomsRespiratory symptoms predominate predominate Growth retardedGrowth retarded – weight & height – weight & height Scrawny, sick, dyspnoeic patientScrawny, sick, dyspnoeic patient Recurrent LRTI/PneumoniasRecurrent LRTI/Pneumonias Chronic lung disease- bronchiectasis etcChronic lung disease- bronchiectasis etc Diaphoresis/ breathlessness at restDiaphoresis/ breathlessness at rest Exertional dyspnoea, limited activity.Exertional dyspnoea, limited activity.

CCHD with ↑ P B Flow inspection findings

Sickly Sickly underweightunderweight individual individual

Cyanosis & clubbing -Cyanosis & clubbing -mild to moderatemild to moderate

Severe PHT, Eisenmenger – Severe PHT, Eisenmenger – modifies findingsmodifies findings

Harrisson’s sulcus, precordial bulgeHarrisson’s sulcus, precordial bulge

Active precordium, RV, LV, PA pulsationsActive precordium, RV, LV, PA pulsations

Obvious cardiomegalyObvious cardiomegaly

CCHD with ↑ P B Flow palpatory findings

Active precordiumActive precordium

RV impulse – RV impulse – DORV, TAPVR, TGA VSD PSDORV, TAPVR, TGA VSD PS

LV Impulse – LV Impulse – Single ventricle, AVSD-AV regurgSingle ventricle, AVSD-AV regurg

Palpable second soundPalpable second sound / / Thrills rare

Eminently operable Operable but PHT

Eisenmenger

CCHD with ↑ P B Flow auscultatory findings

Single second heart sound Loud pulm component, if heard Ejection click – pulmonary/ truncal

CCHD with ↑ P B Flow auscultatory findings -2

Pulm flow – ejection murmur MD murmur - if no severe PHT/ Eisenmenger PR/ TR murmurs may dominate To & fro murmurs in- Truncus/ abs PV syndr. MR murmur in complex AVSD /comm Atrium

RV impulseSingle S2 - loudPulm ESMMitral MDM

Often complex venous anatomyVSD physiology

RV impulseWide split S2Tricuspid MDMPulm. ESM

Brisk pulsesEjection clickNon specific ESMEDM if truncal regurg.Mitral MDM

VSD physiologyASD on auscultationMild cyanosis

CCHD with ↑ P B Flow radiographic findings

Cardiomegaly (unless sev. PHT/Eisenmenger)

Dilated PA

Pulmonary plethora

Atrial enlargement

RV/LV/ Biventric. -Depends on anatomy/age

Keys to clinical diagnosisKeys to clinical diagnosis Work in orderWork in order Pulses,pulses, pulsesPulses,pulses, pulses Colour ie. Cyanosis, pallor, polycythemiaColour ie. Cyanosis, pallor, polycythemia Inspect – for chest form, pulsationsInspect – for chest form, pulsations Palpate to determine – which ventricle ?Palpate to determine – which ventricle ? Forget the murmurForget the murmur !! !! Listen first to S1, and then to S2Listen first to S1, and then to S2 Can you split the second sound ??Can you split the second sound ?? Then concentrate on the componentsThen concentrate on the components Finally the murmursFinally the murmurs – systolic – ejection or pansyst. – systolic – ejection or pansyst. Is there a diastolic murmurIs there a diastolic murmur

The second heart soundThe second heart soundthe key to diagnosis of CHDthe key to diagnosis of CHD

Single Single Normal splitNormal split Wide variable splitWide variable split Wide fixed splitWide fixed split Reverse splitReverse split Loud A2Loud A2 Loud P2Loud P2

Unclassifiable CCHDUnclassifiable CCHD

TGA–IVSTGA–IVS – do not survive – do not survive

EbsteinsEbsteins – may have features of CCF & ↓PBF – may have features of CCF & ↓PBF

cyanosis, cardiomegalycyanosis, cardiomegaly

multiple sounds, wide split, soft P2, Sail soundmultiple sounds, wide split, soft P2, Sail sound

TR murmur, MDM, scratchy soundsTR murmur, MDM, scratchy sounds

P Atresia IVS – seldom survive infancyP Atresia IVS – seldom survive infancy

Acyanotic CHDAcyanotic CHDStenotic CHDStenotic CHD

Few issues Few issues ASD, VSD , PVS, AVS too well known to ASD, VSD , PVS, AVS too well known to

talk abouttalk about

AAO ARCH

DAO

PA

Coarctation of aorta

COA

Localised coarct membrane

Collateral circulation

in

coarctation

1

23

Adapted fromAmplatz radiology in CHD

Cxr coarct adult – rib notching

Coarctation of aortaCoarctation of aorta

Asymptomatic adults – collateralsAsymptomatic adults – collaterals Hypertension !Hypertension ! Femorals !!Femorals !! Bicuspid AV in 80% - ejection click !Bicuspid AV in 80% - ejection click ! Collateral murmur over backCollateral murmur over back AVSAVS

DD of a continous murmurDD of a continous murmur

With or without cyanosis ?With or without cyanosis ?

Continous or a To & Fro murmur ?Continous or a To & Fro murmur ?

Continous murmurs without cyanosisContinous murmurs without cyanosis

PDA PDA (Patent arterial duct)(Patent arterial duct)

AP WindowAP Window Venous HumVenous Hum Coronary AV FistulaCoronary AV Fistula ALCAPAALCAPA RSOVRSOV

Periph Pulm. StenosisPeriph Pulm. Stenosis Systemic AV FistulaSystemic AV Fistula Collaterals in COACollaterals in COA Mammary SouffleMammary Souffle Aortico-LV tunnelAortico-LV tunnel

AO

PA

LV

MR

AORA

Fi

LV

AO

LA

P

AR

RV

LALA

Continous murmur with cyanosisContinous murmur with cyanosis

Duct in TetralogyDuct in Tetralogy Pulm Atresia with DuctPulm Atresia with Duct MAPCAS in Pulm atresiaMAPCAS in Pulm atresia Supracard. TAPVRSupracard. TAPVR Pulm AV FistulaePulm AV Fistulae Post BT shunt Post BT shunt (Thomas-Blalock-Taussig shunt)(Thomas-Blalock-Taussig shunt) Post - Pott’s, Waterston, Central shuntsPost - Pott’s, Waterston, Central shunts

Thomas-Blalock-Taussig shuntWaterston shunt

Pott’s shunt

Central shunt

To & Fro MurmurTo & Fro Murmurwithout cyanosis with cyanosiswithout cyanosis with cyanosis

VSD ARVSD AR MR ARMR AR AS ARAS AR PS PRPS PR Post op TetralogyPost op Tetralogy MR ARMR AR TR PR etcTR PR etc

Tetralogy with ARTetralogy with AR

Truncus with regurgTruncus with regurg

Absent PV syndromeAbsent PV syndrome

LV

RV

PA

PR

LV

PV

AO

PA

PR

VSDoutcome

CCF > FTT > marasmus pneumonias / death

PHT / PVOD / EisenmengerInfective endocarditis

Aortic prolapse & regurg.

Mitral regurgitation.

LV to RA shunts

RSOV

Infundibular pulm. stenosis

VSD gets smallerspontaneous closure

Surgical closureArrhythmiasLV dysfunction

Subaortic membrane

thanksthanks

Thanks