Clinical congenital heart

disease
Prof M S Ranjit MD DCH
Senior consultant paed. Cardiologist
Chennai.
 Some clinical aspects
“paediatric and adolescent accent”
 Classification (modified for simplicity)

 cyanotic - with ↑ pulm blood flow

- with ↓ pulm blood flow
- unclassifyiable – ebsteins/ TGA IVS
 acyanotic – largely shunt lesions
 stenotic - outflow & arterial obstructions
 Cyanosis
caused by > 5gm/dl reduced Hb

 Clinical detection depends on

- % arterial blood that is desaturated
- Hb Concentration !!

 If art O2 satn is 60%,

cyanosis is detectable if Hb > 12.5gm/dl !
but not if Hb < 10 gm/dl !
ie 4gm/dl insufficient for detection of cyanosis !
 Detection of cyanosis

 Astute physician/ paed cardiologist

detects when reduced Hb 3 gm/dl
Others detect at 5gm/dl

 Better to overdiagnose than underdiagnose !

 Clinical diagnosis of cyanosis is inaccurate

M Tynan in Andersons Paediatric cardiology 2007
 Cyanosis -some aspects
 Some CCHD with Rt to Lt shunt and ↑ P B flow
UO TAPVR/ Truncus/ TGA-VSD/ Single ventr Physiol etc
- may have low saturations
- but undetectable cyanosis clinically
i.e. 88-92% !!

 Polycythemic patients appear cyanosed

 Methhaemoglobinaemia !!
 Hyperoxic test
cyanosed or not

 Pulse oximeter - not always reliable

“a random number generator”
 Rt radial ABG in air and after 5-10 min O2
paO2 > 250mmHg -excludes CCHD
paO2 > 160 -CCHD unlikely
( UO TAPVR False negative !)
paO2 < 100 -CCHD likely (usually lower)
(severe Lung disease (high paCo2), PPHN/PFC)
 “Radial ABG more useful than ECG or CXR
in detection of cyanotic heart disease”

Warburton 1981
 CCHD
in 3 major circumstances
 Pulmonary obstruction with avenue for right to
left shunting

 Discordant AV connection i.e transpositions

 Common mixing situations i.e common atrium

single ventricle etc
 Unusual causes of cyanosis
without murmurs !
surviving to adolesc./ adult life

 Left SVC to LA
 IVC to LA
 Rt. SVC to LA
 Pulm. AV Fistulae (Ostler Rendu Weber syndr)
LV
Cyanosis – which category?

 Symptomatology

 Clinical examination

 Chest X ray
 Fallot physiology

 Systemic venous return unable to reach lungs

 Shunted right to left away from pulm circulation

 ASD/VSD essential for this to occur;

 Or a common chamber !
PLUS
 Fallot physiology
 Obstruction 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 flow
pulmonary oligaemia on CXR

 Symptomatology

 Inspection findings

 Auscultatory findings

 Chest Skiagram
CCHD with ↓ PBF - symptoms
 Exertional dyspnoea
 Cyanosis, spells, seizures
 CNS complications

 No recurrent RTI/ no diaphoresis

 No breathlessness at rest
except in extremes / anaemia
 CCHD - ↓ PBF
- inspection /palpatory findings

 Cyanosis & clubbing

 polycythemia
 Quiet precordium to inspection & palpation
 No Harrisons sulcus or precordial bulge
 Apex well within limits if visible

 No palpable sounds or thrills

 CCHD with ↓ PB Flow
auscultatory findings
 Normal first heart sound
 Single second heart sound
 Pulm component inaudible

 Stenotic pulmonary murmur

slightly after S1
stops short of S2
 Other murmurs – ductal/ MAPCA/ AR
 Ejection murmur in Fallot physiology

 Length & loudness inversely proportional to

severity of stenosis
In isolated PVS – the opposite !

 Absent murmur – acquired pulm atresia

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

 To & Fro – Aortic regurg / Abs PV syndrome

MAPCAS
CCHD with Pulm.blood flow

 Tetralogy of Fallot
 VSD - PS
 DORV – VSD – PS
 Tricusp. atresia - PS
 Single ventricle - PS
 TGA with VSD – PS
 Corr.transp.-VSD-PS
 ASD - PS
 Chest skiagram in CCHD with ↓ PBF

 Small heart

 Pulmonary bay

 Pulmonary oligaemia

 Right aortic arch/ RA enlargement/ differential

vascularity/ narrow pedicle in various defects
Tetralogy of Fallot
 Typical - Fallot CXR

Pulmonary Pulm bay

oligaemia

RV apex
Fallot physiology
Tricuspid atresia
Normally related great arteries
Restrictive VSD

PFO / ASD
VSD / PDA
Fallot physiology

Tricuspid atresia
Fallot physiology
Transposition of the
TGA-VSD-PS Great arteries
With V S D and P S
Fallot physiology
Double inlet Single ventricle with PS
Left ventricle
AO CCHD with PB Flow
With PS
PA

LA

RA

LV
 Atrial septal defect with pulmonic stenosis
ASD with PS
Fallot physiology

Fallot physiology
S2 variable
Pulm ESM
 Corrected Transposition with VSD and PS

Fallot Physiology Atrio-ventricular &

ventriculo-arterial
AO Discordance
PA LA

RA RV
Fallot physiology
LV
Single S2
Loud A2
pulmonic ESM
CNS complications of CCHD with ↓ PBF

 Paradoxic embolus
 Cerebral thrombosis
 Cerebral abcess
 Seizures
 Hypoxic damage
 Endocarditis & vegetations
 Postoperative strokes
 CCHD with ↑ pulm blood flow

 Transpositions with VSD/Duct/ASD

 Common mixing situations

atrial level – TAPVR/Comm Atr
Mixing at ventric level – DORV/Single ventric
arterial level – comm art trunk

 Mild cyanosis, CCF, resp symptoms, ex dyspnoea

 CCHD with ↑ Pulm blood flow

 Seldom survive to adolescence/ adulthood

 UO TAPVR/ comm atrium- the exceptions

 Most have Eisenmenger by then

and those features dominate
 CCHD ↑ P B Flow
easy diagnosis – rare
 Clinical differentiation not always possible
(Tynan M, Andersons paed cardiology 2007)

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

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

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

 Sing S2, cont murmur over back – p atr / MAPCAS

CCHD with ↑ P B Flow - symptoms

 Respiratory symptoms predominate

 Growth retarded – weight & height
 Scrawny, sick, dyspnoeic patient
 Recurrent LRTI/Pneumonias
 Chronic lung disease- bronchiectasis etc
 Diaphoresis/ breathlessness at rest
 Exertional dyspnoea, limited activity.
 CCHD with ↑ P B Flow
inspection findings
 Sickly underweight individual

 Cyanosis & clubbing -mild to moderate

 Severe PHT, Eisenmenger – modifies findings

 Harrisson’s sulcus, precordial bulge

Active precordium, RV, LV, PA pulsations
Obvious cardiomegaly
 CCHD with ↑ P B Flow
palpatory findings
 Active precordium

 RV impulse – DORV, TAPVR, TGA VSD PS

 LV Impulse – Single ventricle, AVSD-AV regurg

 Palpable 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
 Double outlet right ventricle with VSD & PAH
Normally related great arteries
CCHD with DORV – VSD - PAH
P B Flow

RV impulse
Single S2 - loud
Pulm ESM
Mitral MDM
Often complex venous anatomy
VSD physiology
Common atrium
CCHD with P B Flow

RV impulse
Wide split S2
Tricuspid MDM
Pulm. ESM
Transposition of the
Great arteries
With V S D
 Truncus arteriosus
CCHD with P B Flow

Brisk pulses
Ejection click
Non specific ESM
EDM if truncal regurg.
Mitral MDM
 Total anomalous pulmonary venous return
TAPVR
Supra cardiac

Infra cardiac

Cardiac

VSD physiology
ASD on
auscultation
Mild 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 diagnosis
 Work in order
 Pulses,pulses, pulses
 Colour ie. Cyanosis, pallor, polycythemia
 Inspect – for chest form, pulsations
 Palpate to determine – which ventricle ?
 Forget the murmur !!
 Listen first to S1, and then to S2
 Can you split the second sound ??
 Then concentrate on the components
 Finally the murmurs – systolic – ejection or pansyst.
 Is there a diastolic murmur
 The second heart sound
the key to diagnosis of CHD
 Single
 Normal split
 Wide variable split
 Wide fixed split
 Reverse split
 Loud A2
 Loud P2
 Unclassifiable CCHD

 TGA–IVS – do not survive

 Ebsteins – may have features of CCF & ↓PBF

cyanosis, cardiomegaly
multiple sounds, wide split, soft P2, Sail sound
TR murmur, MDM, scratchy sounds

 P Atresia IVS – seldom survive infancy

 Acyanotic CHD
Stenotic CHD

 Few issues
 ASD, VSD , PVS, AVS too well known to
talk about
AAO ARCH

COA
PA
DAO

Coarctation of aorta
Localised coarct
membrane
 1

Collateral
circulation
in 3
coarctation 2

Adapted from
Amplatz radiology in CHD
Cxr coarct adult – rib notching
 Coarctation of aorta
 Asymptomatic adults – collaterals
 Hypertension !
 Femorals !!
 Bicuspid AV in 80% - ejection click !
 Collateral murmur over back
 AVS
 DD of a continous murmur

 With or without cyanosis ?

 Continous or a To & Fro murmur ?

 Continous murmurs without cyanosis

 PDA (Patent arterial duct)  Periph Pulm. Stenosis

 AP Window  Systemic AV Fistula
 Venous Hum  Collaterals in COA
 Coronary AV Fistula  Mammary Souffle
 ALCAPA  Aortico-LV tunnel
 RSOV
 PA

AO

MR
Fi

AO
RA
LV
 P
AO

LV AR
LA

RV

LA
LA
Continous murmur with cyanosis
 Duct in Tetralogy
 Pulm Atresia with Duct
 MAPCAS in Pulm atresia
 Supracard. TAPVR
 Pulm AV Fistulae
 Post BT shunt (Thomas-Blalock-Taussig shunt)
 Post - Pott’s, Waterston, Central shunts
 Pott’s shunt
Thomas-Blalock-Taussig
Waterston shunt shunt

Central shunt
 To & Fro Murmur
without cyanosis with cyanosis

 VSD AR
 MR AR  Tetralogy with AR
 AS AR
 PS PR  Truncus with regurg
 Post op Tetralogy
 MR AR  Absent PV syndrome
 TR PR etc
LV
PA PV

LV
RV
PR

PR

AO

PA
VSD CCF > FTT > marasmus
outcome pneumonias / death
PHT / PVOD / Eisenmenger
Infective endocarditis
Aortic prolapse & regurg.
RSOV
Mitral regurgitation.
LV to RA shunts
Subaortic Infundibular pulm. stenosis
membrane VSD gets smaller
Arrhythmias
spontaneous closure
Surgical closure
LV dysfunction
 thanks

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