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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 ↑ pu...
Cyanosis
caused by > 5gm/dl reduced Hb
 Clinical detection depends on
- % arterial blood that is desaturated
- Hb Concent...
Detection of cyanosis
 Astute physician/ paed cardiologist
detects when reduced Hb 3 gm/dl
Others detect at 5gm/dl
 Bett...
Cyanosis -some aspects
 Some CCHD with Rt to Lt shunt and ↑ P B flow
UO TAPVR/ Truncus/ TGA-VSD/ Single ventr Physiol etc...
Hyperoxic test
cyanosed or not
 Pulse oximeter - not always reliable
“a random number generator”
 Rt radial ABG in air a...
 “Radial ABG more useful than ECG or CXR
in detection of cyanotic heart disease”
Warburton 1981
C C H D
in 3 major circumstances
 Pulmonary obstruction with avenue for right to
left shunting
 Discordant AV connection...
Unusual causes of cyanosis
without murmurs !
surviving to adolesc./ adult life
 Left SVC to LA
 IVC to LA
 Rt. SVC to L...
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/...
Fallot physiology
 Obstruction at
- RA outlet - i.e Tric atresia
- infund/valvar Pulm stenosis
- rarely branch PA stenosi...
CCHD with ↓ pulm blood flow
pulmonary oligaemia on CXR
 Symptomatology
 Inspection findings
 Auscultatory findings
 Ch...
CCHD with ↓ PBF - symptoms
 Exertional dyspnoea
 Cyanosis, spells, seizures
 CNS complications
 No recurrent RTI/ no d...
CCHD - ↓ PBF
- inspection /palpatory findings
 Cyanosis & clubbing
 polycythemia
 Quiet precordium to inspection & palp...
CCHD with ↓ PB Flow
auscultatory findings
 Normal first heart sound
 Single second heart sound
 Pulm component inaudibl...
Ejection murmur in Fallot physiology
 Length & loudness inversely proportional to
severity of stenosis
In isolated PVS – ...
MAPCAS
CCHD with Pulm.blood flow
 Tetralogy of Fallot
 VSD - PS
 DORV – VSD – PS
 Tricusp. atresia - PS
 Single ventricle - ...
Chest skiagram in CCHD with ↓ PBF
 Small heart
 Pulmonary bay
 Pulmonary oligaemia
 Right aortic arch/ RA enlargement/...
Tetralogy of Fallot
Typical - Fallot CXR
Pulm bay
RV apex
Pulmonary
oligaemia
Tricuspid atresia
Normally related great arteries
Restrictive VSD
PFO / ASD
VSD / PDA
Fallot physiology
Fallot physiology
Tricuspid atresia
Transposition of the
Great arteries
With V S D and P S
TGA-VSD-PS
Fallot physiology
LV
RA
LA
PA
AO
Double inlet
Left ventricle
With PS CCHD with PB Flow
Single ventricle with PS
Fallot physiology
Atrial septal defect with pulmonic stenosis
ASD with PS
Fallot physiology
Fallot physiology
S2 variable
Pulm ESM
Corrected Transposition with VSD and PS
Atrio-ventricular &
ventriculo-arterial
Discordance
LV
RVRA
LA
AO
PA
Fallot Physio...
CNS complications of CCHD with ↓ PBF
 Paradoxic embolus
 Cerebral thrombosis
 Cerebral abcess
 Seizures
 Hypoxic dama...
CCHD with ↑ pulm blood flow
 Transpositions with VSD/Duct/ASD
 Common mixing situations
atrial level – TAPVR/Comm Atr
Mi...
CCHD with ↑ Pulm blood flow
 Seldom survive to adolescence/ adulthood
 UO TAPVR/ comm atrium- the exceptions
 Most have...
CCHD ↑ P B Flow
easy diagnosis – rare
 Clinical differentiation not always possible
(Tynan M, Andersons paed cardiology 2...
CCHD with ↑ P B Flow - symptoms
 Respiratory symptoms predominate
 Growth retarded – weight & height
 Scrawny, sick, dy...
CCHD with ↑ P B Flow
inspection findings
 Sickly underweight individual
 Cyanosis & clubbing -mild to moderate
 Severe ...
CCHD with ↑ P B Flow
palpatory findings
 Active precordium
 RV impulse – DORV, TAPVR, TGA VSD PS
 LV Impulse – Single v...
Eminently operable Operable but PHT
Eisenmenger
CCHD with ↑ P B Flow
auscultatory findings
 Single second heart sound
 Loud pulm component, if heard
 Ejection click – ...
CCHD with ↑ P B Flow
auscultatory findings -2
 Pulm flow – ejection murmur
 MD murmur - if no severe PHT/ Eisenmenger
 ...
Double outlet right ventricle with VSD & PAH
Normally related great arteries
DORV – VSD - PAHCCHD with
P B Flow
RV impulse...
Common atrium
CCHD with P B Flow
Often complex venous anatomy
VSD physiology
RV impulse
Wide split S2
Tricuspid MDM
Pulm. ...
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
Supra cardiac
Cardiac
Infra cardiac
Total anomalous pulmonary venous return
TAPVR
VSD physiology
ASD on
auscultation
Mild ...
CCHD with ↑ P B Flow
radiographic findings
 Cardiomegaly (unless sev. PHT/Eisenmenger)
 Dilated PA
 Pulmonary plethora
...
Keys to clinical diagnosis
 Work in order
 Pulses,pulses, pulses
 Colour ie. Cyanosis, pallor, polycythemia
 Inspect –...
The second heart sound
the key to diagnosis of CHD
 Single
 Normal split
 Wide variable split
 Wide fixed split
 Reve...
Unclassifiable CCHD
 TGA–IVS – do not survive
 Ebsteins – may have features of CCF & ↓PBF
cyanosis, cardiomegaly
multipl...
Acyanotic CHD
Stenotic CHD
 Few issues
 ASD, VSD , PVS, AVS too well known to
talk about
AAO ARCH
DAO
PA
Coarctation of aorta
COA
Localised coarct
membrane
Collateral
circulation
in
coarctation
1
2
3
Adapted from
Amplatz radiology in CHD
Cxr coarct adult – rib notching
Coarctation of aorta
 Asymptomatic adults – collaterals
 Hypertension !
 Femorals !!
 Bicuspid AV in 80% - ejection cl...
DD of a continous murmur
 With or without cyanosis ?
 Continous or a To & Fro murmur ?
Continous murmurs without cyanosis
 PDA (Patent arterial duct)
 AP Window
 Venous Hum
 Coronary AV Fistula
 ALCAPA
 ...
AO
PA
LV
MR
AO
RA
Fi
LV
AO
LA
P
AR
RV
LA
LA
Continous murmur with cyanosis
 Duct in Tetralogy
 Pulm Atresia with Duct
 MAPCAS in Pulm atresia
 Supracard. TAPVR
 ...
Thomas-Blalock-Taussig
shuntWaterston shunt
Pott’s shunt
Central shunt
To & Fro Murmur
without cyanosis with cyanosis
 VSD AR
 MR AR
 AS AR
 PS PR
 Post op Tetralogy
 MR AR
 TR PR etc
 ...
LV
RV
PA
PR
LV
PV
AO
PA
PR
VSD
outcome
CCF > FTT > marasmus
pneumonias / death
PHT / PVOD / Eisenmenger
Infective endocarditis
Aortic prolapse & regu...
thanks
Thanks
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
A good ppt on Clinical congenital heart disease for Post Graduate
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A good ppt on Clinical congenital heart disease for Post Graduate

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Clinical congenital heart disease

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A good ppt on Clinical congenital heart disease for Post Graduate

  1. 1. Clinical congenital heart disease Prof M S Ranjit MD DCH Senior consultant paed. Cardiologist Chennai.
  2. 2. 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
  3. 3. 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 !
  4. 4. 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
  5. 5. 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 !!
  6. 6. 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)
  7. 7.  “Radial ABG more useful than ECG or CXR in detection of cyanotic heart disease” Warburton 1981
  8. 8. C C H D 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
  9. 9. 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)
  10. 10. LV
  11. 11. Cyanosis – which category?  Symptomatology  Clinical examination  Chest X ray
  12. 12. 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
  13. 13. 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 !!
  14. 14. CCHD with ↓ pulm blood flow pulmonary oligaemia on CXR  Symptomatology  Inspection findings  Auscultatory findings  Chest Skiagram
  15. 15. CCHD with ↓ PBF - symptoms  Exertional dyspnoea  Cyanosis, spells, seizures  CNS complications  No recurrent RTI/ no diaphoresis  No breathlessness at rest except in extremes / anaemia
  16. 16. 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
  17. 17. 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
  18. 18. 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
  19. 19. MAPCAS
  20. 20. 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
  21. 21. Chest skiagram in CCHD with ↓ PBF  Small heart  Pulmonary bay  Pulmonary oligaemia  Right aortic arch/ RA enlargement/ differential vascularity/ narrow pedicle in various defects
  22. 22. Tetralogy of Fallot
  23. 23. Typical - Fallot CXR Pulm bay RV apex Pulmonary oligaemia
  24. 24. Tricuspid atresia Normally related great arteries Restrictive VSD PFO / ASD VSD / PDA Fallot physiology
  25. 25. Fallot physiology Tricuspid atresia
  26. 26. Transposition of the Great arteries With V S D and P S TGA-VSD-PS Fallot physiology
  27. 27. LV RA LA PA AO Double inlet Left ventricle With PS CCHD with PB Flow Single ventricle with PS Fallot physiology
  28. 28. Atrial septal defect with pulmonic stenosis ASD with PS Fallot physiology Fallot physiology S2 variable Pulm ESM
  29. 29. Corrected Transposition with VSD and PS Atrio-ventricular & ventriculo-arterial Discordance LV RVRA LA AO PA Fallot Physiology Fallot physiology Single S2 Loud A2 pulmonic ESM
  30. 30. CNS complications of CCHD with ↓ PBF  Paradoxic embolus  Cerebral thrombosis  Cerebral abcess  Seizures  Hypoxic damage  Endocarditis & vegetations  Postoperative strokes
  31. 31. 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
  32. 32. 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
  33. 33. 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
  34. 34. 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.
  35. 35. 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
  36. 36. 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
  37. 37. Eminently operable Operable but PHT Eisenmenger
  38. 38. CCHD with ↑ P B Flow auscultatory findings  Single second heart sound  Loud pulm component, if heard  Ejection click – pulmonary/ truncal
  39. 39. 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
  40. 40. Double outlet right ventricle with VSD & PAH Normally related great arteries DORV – VSD - PAHCCHD with P B Flow RV impulse Single S2 - loud Pulm ESM Mitral MDM
  41. 41. Common atrium CCHD with P B Flow Often complex venous anatomy VSD physiology RV impulse Wide split S2 Tricuspid MDM Pulm. ESM
  42. 42. Transposition of the Great arteries With V S D
  43. 43. Truncus arteriosus CCHD with P B Flow Brisk pulses Ejection click Non specific ESM EDM if truncal regurg. Mitral MDM
  44. 44. Supra cardiac Cardiac Infra cardiac Total anomalous pulmonary venous return TAPVR VSD physiology ASD on auscultation Mild cyanosis
  45. 45. 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
  46. 46. 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
  47. 47. 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
  48. 48. 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
  49. 49. Acyanotic CHD Stenotic CHD  Few issues  ASD, VSD , PVS, AVS too well known to talk about
  50. 50. AAO ARCH DAO PA Coarctation of aorta COA
  51. 51. Localised coarct membrane
  52. 52. Collateral circulation in coarctation 1 2 3 Adapted from Amplatz radiology in CHD
  53. 53. Cxr coarct adult – rib notching
  54. 54. Coarctation of aorta  Asymptomatic adults – collaterals  Hypertension !  Femorals !!  Bicuspid AV in 80% - ejection click !  Collateral murmur over back  AVS
  55. 55. DD of a continous murmur  With or without cyanosis ?  Continous or a To & Fro murmur ?
  56. 56. Continous murmurs without cyanosis  PDA (Patent arterial duct)  AP Window  Venous Hum  Coronary AV Fistula  ALCAPA  RSOV  Periph Pulm. Stenosis  Systemic AV Fistula  Collaterals in COA  Mammary Souffle  Aortico-LV tunnel
  57. 57. AO PA LV MR AO RA Fi
  58. 58. LV AO LA P AR RV LA LA
  59. 59. 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
  60. 60. Thomas-Blalock-Taussig shuntWaterston shunt Pott’s shunt Central shunt
  61. 61. To & Fro Murmur without cyanosis with cyanosis  VSD AR  MR AR  AS AR  PS PR  Post op Tetralogy  MR AR  TR PR etc  Tetralogy with AR  Truncus with regurg  Absent PV syndrome
  62. 62. LV
  63. 63. RV PA PR LV PV AO PA PR
  64. 64. VSD outcome CCF > FTT > marasmus pneumonias / death PHT / PVOD / Eisenmenger Infective endocarditis Aortic prolapse & regurg. Mitral regurgitation. LV to RA shunts RSOV Infundibular pulm. stenosis VSD gets smaller spontaneous closure Surgical closureArrhythmias LV dysfunction Subaortic membrane
  65. 65. thanks Thanks

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