3. Structures of the heart

4. Normal Heart

5. Atrial Septal defect ( ASD ) Insidence : + 10 %  :  ratio = 1,5 to 2 : 1 Anatomy :  Defect on foramen ovale : Secundum ASD  Defect at SVC and RA junction: sinus venosus ASD  Defect at ostium primum : primum ASD

6. ASD

7. Atrial Septal Defect

8. Diagram of ASD

9. LA LV RVRA PA AO Systemic Lungs Qp > Qs Atrial septal defect

10. RA RV LA LV RA RV LA LV Atrial septal Defect

11. Clinical findings Asymptomatic Auscultation : Normal 1st HS or loud Widely split and fixed 2 nd HS Ejection systolic murmur Atrial septal Defect

12. Atrial Septal Defect Auscultation :1 st HS N or loud widely split and fixed 2 nd HS Ejection Sistolic Murmur

13. ECG : IRBB, right ventricular hypertrophy Atrial Septal Defect

14. Right atrial enlargement Prominence the MPA segment Increased pulmonary vascular marking Atrial Septal Defect Chest X-Ray

15. Atrial Septal Defect Diagnosis Differential Primary Atrial Septal Defect ECG : LAD Partial Anomalous Pulmonary Vein Drainage Pulmonary Stenosis Innocent Murmur

16. Atrial Septal defect Management Surgery : Preschool age Recent treatment: transcatheter closure using ASO (Amplatzer septal occluder)

17. ASD Small Shunt Large Shunt Observation Evaluation At age 5-8 yrs Cath FR<1.5FR>1.5 Conservative Infants Children/Adults Heart Failure (-) Heart Failure (+) Age >1yrs W >10kg Transcatheter closure (Secundum ASD) / Surgical Closure(others) Conservative Anti failure FailSuccess PH (-)PH (+) PVD (-) PVD (+) Hyperoxia Reac- tive Non reactive Surgical Closure

18. Atrial septal defect

19. ASD before occlusion

20. During balloon sizing Atrial septal defect

21. ASD after occluded using ASO

22. Ventricular septal defect Insidence  20 % of all CHD  No sex influenced Anatomy  Subarterial defect : below pulmonary and aortic valve  Perimembranous defect: below aortic valve at pars membranous septum  Muscular defect

23. VSD

24. Ventricular Septal Defect

25. Systemic Lungs Qp > Qs Ventricular Septal defect

26. RA RV RA LA RVLV Ventricular septal defect

27. Ventricular Septal Defect

28. Clinical findings Day 1 st after birth: murmur (-) After 2-6 weeks : murmur (+) Murmur : pansystolic grade 3/6 or higher at LSB 3 Small muscular defect: early systolic murmur Significant defect: Mid diastolic murmur at apex

29. Small VSD Large VSD Ventricular Septal Defect Murmur: pansystolic grade 3/6 or higher at LSB 3

30. Ventricular Septal Defect Cardiomegaly Apex down ward Prominence pulmonary artery segment Increased pulmonary vascular marking

31. Ventricular septal Defect Diagnosis Differential  PDA with PH  Tetralogy Fallot non cyanotic  Inoscent murmur

32. Ventricular septal defect Management: Definitive : VSD closure  Surgery  Transcatheter closure

33. DSV Heart failure (+) Heart failure (-) Anti failure FailSuccess PAB Evaluate in 6 mths Surgical closure/Transcatheter closure Aortic valve prolaps Infundibular stenosis PHSmallerSpontaneous closure Cath PVD(-)PVD(+)Cath ReactiveNon- reactive Conservative FR>1.5 FR<1.5

34. Ventricular septal defect VSD before occlusion

35. Ventricular septal defect VSD during deploying the device

36. Ventricular septal defect VSD after occluded using ASO

37. Patent Ductus Arteriosus Insidence + 10% Female : Male = 1.2 to 1.5 : 1 Premature and LBW higher Anatomy Fetus: ductus arteriosus connects PA and aorta. If ductus does not closs  Patent Ductus arteriosus

38. PDA

39. LA LV RV RA PA AO Systemic Lungs Qp > Qs Patent Ductus Arteriosus

40. RA RV LA LV RA LA RVLV Patent Ductus Arteriosus

41. Clinical findings Small defect: Symptom (-) Growth and development normal Significant defect: Decreased exercise tolerant Weigh gained not good Frequent URTI Specific case: pulsus seler at 4 th extremities

42. Patent Ductus Arteriosus Diagnosis Pulsus seler and continuous murmur heard

43. Patent Ductus Arteriosus Chest X- Ray –Similar to VSD

44. Patent Ductus Arteriosus Auscultation : continuosus murmur at upper LSB 2

45. Diagnosis Differential AP-window Arterio-venous fistulae Management premature: indometasin PDA closure : surgery transcatheter closure Patent Ductus Arteriosus

46. PDA Neonates/InfantsChildren/Adults Heart failure (+)Heart failure (-) Premature Full term Anti failure Indometacin SuccessFail Spontaneous closure Anti failure SuccessFail Surgical ligation Transcatheter closure PH (-) PH (+) LRLR RLRL Hyperoxia Reactive Non reactive Conservative Age >12wks W >4kg

47. Patent Ductus Arteriosus

49. PDA before occluded using ADO

50. Patent Ductus Arteriosus PDA after occluded using ADO

51. Patent Ductus Arteriosus PDA before occluded using coil

52. Patent Ductus Arteriosus PDA after occluded using coil

53. Pulmonary Stenosis Incidence : 8-10% Anatomy: Pulmonary stenosis valvular :  Bicuspid pulmonary valve  Valve leaflet thickening and adhession Pulmonary stenosis infundibular :  Hyperthropy infundibulum

54. Pulmonary Stenosis Clinical findings Valvular s tenosis Mild : Ejection systolic Wide 2 nd HS ejectiin click Moderate: ejection systolic, early systolic click Severe : ejecstion systolic, ejection click (-) Stenosis infundibular Ejection click ( - ) 1 st HS normal, 2 nd HS weak, ejection systolic Pulmonary stenosis periphery 1 st & 2 nd HS normal, ejection systolic

55. Pulmonary Stenosis Mild : ejection systolic 2 nd HS wide split ejection click Moderate: ejecsi systolic, early ejection click Severe : ejection systolic, click ejection (-)

56. Poulmonary Stenosis Diagnosis A symptomatic patient: click systolic (stenosis valvular) systolic murmur wide split 2 nd HS vary with respiration

57. Poulmonary Stenosis Normal or mild cardiomegaly Marked pulmonary valve post stenotic dilatation Normal pulmonary vascularity

58. ECG : RAD Echocardiograhhy : confirmation diagnosis Catheterization: increased RV pressure without increased oxygen saturation Pulmonary Stenosis

59. Management Medicamentosa : useless Mild stenosis: intervention (-) Moderate stenosis: observation Severe stenosis: balloon valvuloplasty

60. Pulmonary Stenosis

61. Before ballooning

62. Pulmonary Stenosis During ballooning

63. Pulmonary Stenosis After ballooning

64. Coarctation Aorta Incidence In Western country 5 % of all CHD In Asian Country incidence lower underdiagnosis ? Anatomy Stenosis at any where in the aorta (from aortic valve to abdominalis aorta) More frequent at ductus arteriosus Botalli and pulmonary artery junction

65. Coarctation Aorta

66. Clinical findings Severe coarctation in neonates period can cause heart failure in 1 st weeks of life Clinical manifestation in children: arterial hypertension commonly asymptomatic Different pulses felt at upper and lower extremities Examination : increased left ventricular activity, thrill systolic, 1 st and 2 nd HS normal, ejection systolic murmur Coarctation Aorta

67. Diagnosis Clinically : lower extremities pulses are weak CXR : Mild cardiomegaly Prominence of aortic knob Normal pulmonary blood flow ECG : normal or LVH Echocardiography: a discrete shelf-like membrane Cardiac catheterization and angiography: to confime diagnosis Coarctation Aorta

68. Management Neonates : PGE1 to maintain PDA Diuretic Correction acid-base imbalance Prepared to undergo surgery Big children: Surgery should be done as soon as diagnosis made Balloon angioplasty Coarctation Aorta

72. Before ballooning

73. Coarctation Aorta During ballooning

74. Coarctation Aorta After ballooning

75. Tetralogy Fallot Insidence 5-8% from all CHD Anatomy Cause: Left-anterior deviation of infundibular septum Sindroma consist of 4 items:  VSD  pulmonary stenosis  aortic over-riding  RVH

76. Tetralogy Fallot

77. Hemodynamic acyanotic Hemodynamic cyanotic

78. Tetralogy Fallot Diagnosis Clinically : cyanosis Single 2 nd HS, ejection systolic murmur

79. Tetralogy Fallot Single 2 nd HS, ejection systolic murmur

80. Tetralogi Fallot

81. CXR : Boot-shaped Concave pulmonary segment Apex upturned Decreased pulmonary blood flow Tetralogy Fallot

82. ECG : RAD Echocardiography : to confirm diagnosis

83. Tetralogy Fallot Diagnosis Differential  Pulmonary Atresia  Double outlet right ventricle and pulmonary stenosis  Transposisi of great arteri and pulmonary stenosis Management  Paliative treatment: Blalock-Taussig shunt  Definitive: total correction

84. Tetralogy of Fallot < 1 yr> 1 yr spell (+) spell (-) propranolol failed succeed BTS total correction cath small PAgood sized PA clinically ECG CXR echo age 1 yr cath BTS/ PDA Stent evaluation

85. Tetralogy Fallot

87. Transposition of Great Artery Insidence 5% of CHD Anatomy Abnormality of formation of trunkal septum that cause aorta arising from RV and PA arising from LV

88. Transposition of Great artery

89. Hemodynamic normal Hemodynamic of TGA “series”“parallel” Transposition of Great artery

90. TGA without VSD In adequate MixingAdequate Mixing Transposition of Great artery

91. TGA with large VSDTGA with VSD and PS Transposition of Great artery

92. Clinical aspects More frequent in male Birth weight usually normal normal or bigger Cyanotic vary from mild to severe Auscultation : single 2 nd HS and loud Murmur vary from silent to pansystolic murmur or continuous murmur Transposition of Great artery

93. Diagnosis Clinically : Suspicious if neonates presents with cyanotic with birth weight normal or bigger Murmur (-) Single 2 nd HS and loud Transposition of Great artery

94. Murmur (-) Single 2 nd HS and loud Transposition of Great artery

95. CXR : Cardiomegaly Egg-on-side heart Increased pulmonary vascular marking

96. Transposition of Great artery ECG : RAD RVH Echocardiography : to confirm diagnosis Cardiac catheterization: usually is not needed

97. Diagnosis Differential  trunkus arteriosus  trikuspid atresia  pulmonary atresia Management Surgery: arterial switch Paliative : Blalock-Taussig shunt Transposition of Great artery

98. Transposition of Great Artery PGE1 VSD(-)VSD(+)  1mth > 1mth Cath LV  2/3 syst LV<2/3 syst PAB Arterial Switch LVOTO(-) LVOTO(+) >3 mths  3 mths Cath PARI<8 PARI  8 Arterial Switch and Perforated VSD Dynamic & resectable Un- resectable BTS/ PDA stent Cath Rastelli Senning BAS/Blallock Hanlon

99. Transposition of Great artery

100. Truncus Arteriosus Insidence around 1 % of CHD Anatomy  Failure of septation of truncus arteriosus form aorta and pulmonary artery  There are 3 type:  Type 1 : MPA arises from the truncus and then divides into the RPA and LPA  Tipe 2 : The PAs arise from the posterior aspect of the truncus  Tipe 3 : The PAs arise from the lateral aspects of the truncus  Tipe 4: Arteries arising from the descending aorta supply the lungs

101. Truncus Arteriosus

104. Diagnosis Clinically suspected if: neonates present with cyanotic and single 2 nd HS murmur vary CXR:cardiomegaly increased pulmonary vascular marking ECG: biventricular hypertrophy Echocardiografhy: to confirm diagnosis Catheterization: decreased oxygen saturation at right heart and aorta Truncus Arteriosus

105. Diagnosis Differential  Transposisi of great artery  Total anomalus pulmonary vein drainage Management Medicamentosa : temporary Surgery: Rastelli Palliative: pulmonary artery banding Truncus Arteriosus

107. Tricuspid Atresia Incidence –1 % from all CHD Embriology –Valve formed at 5 th weeks –Fussion of part of endocardial cushion, ventricular septum and miocardium

108. Anatomy –Valve leaflet adhession one to another, difficult to open –ASD essentially required to drain blood from RA to LA –Classified into 2 group Normal related great artery Transposed grat artery Tricuspid Atresia

109. Tricuspid Atresia with normal related great artery Tricuspid atresia with transposed geat artery Tricuspid Atresia

110. Manifestasi klinis –Cyanosis early after birth –Increased RV activity –Increased LV activity –Auscultation Single 1 st and 2 nd HS Tricuspid Atresia

111. Clinical manifestation –In almost all patients murmur is silent –If murmur present Diastolic murmur due to relative MS Pansystolic murmur due to VSD Tricuspid Atresia

113. Diagnosis and diagnosis differential –Clinically: Cyanosis with or without murmur Tricuspid Atresia

114. CXR: Heart minimally Enlarged The PVMs are Decreased The MPA segment is concave Tricuspid Atresia

115. –ECG: LAD Left ventricular hypertrophy With or without LAE Tricuspid Atresia

116. Echocardiography: Essential to make diagnosis Catheterization –Catheter can not be passed from RA to RV –Increased RA and LA pressure –Decreased oxygen saturation in LA –Angiography: definitive diagnosis Tricuspid Atresia

117. Diagnosis differential –Transposition of great artery –Truncus arteriosus –Tetralogy of Fallot –Total Anomalous pulmonary vein drainage Tricuspid Atresia

118. Management –Fontan operation Tricuspid Atresia

124. Modification of Fontan operation Tricuspid Atresia