Cong heart diseasea

1. CONGENITAL HEART
DISEASES
Soumya Ranjan Parida
Basic B.Sc. Nursing 4th
year
Sum Nursing College

2. 01/09/15 2
Practical approach
 Congenital-Associated syndrome
 Acquired
 Rheumatic
 Cardiomyopathy ( Restrictive )
 Cardiomyopathy ( Dilated , Post viral
myocarditis )
 Pericardial diseases ( Effusion,Pericarditis)
 Kawasaki disease

3. 01/09/15 3
CLINICAL CLASSIFICATION OF
CONGENITAL HEART DISEASES
 Cardiac Malpositions- Ectopia cordis
Dextrocardia
 Acyanotic without a shunt–
Malformations on left side
Malformations on right side
 Acyanotic with a shunt
 Cyanotic

4. 01/09/15 4
Acyanotic without a shunt
 Left sided malformations
Mitral stenosis
Mitral regurgitation
Aortic stenosis
Aortic regurgitation
Coarctation of aorta

5. 01/09/15 5
Acyanotic without a shunt
 Right sided malformation
Ebsteins anomaly of Tricuspid valve
Pulmonary stenosis
Pulmonary regurgitation
Primary pulmonary hypertension

6. 01/09/15 6
Acyanotic with a shunt
 Shunt at atrial level
ASD
PAPVC
 Shunt at ventricular level
VSD
 Shunt at great artery level
PDA
AP Window
 Shunt at more than one level

7. 01/09/15 7
Acyanotic without a shunt
 Left sided malformations
Mitral stenosis
Mitral regurgitation
Aortic stenosis
Aortic regurgitation
Coarctation of aorta

8. 01/09/15 8
Aortic Stenosis
 Age
 Symptoms
 Types
 Valvular
 Supra valvular
 Sub valvular
Clinical Presentation
Management

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Coarctation of Aorta
 Age
 Types
 Pre ductal
 Post ductal
 Clinical presentation
 Associations
 Management
 Age of intervention
 Surgery // Cath based intervention

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Acyanotic without a shunt
 Right sided malformation
Ebsteins anomaly of Tricuspid valve
Pulmonary stenosis
Pulmonary regurgitation
Primary pulmonary hypertension

11. 01/09/15 11
Pulmonary stenosis
 Types
 Age
 Symptoms
 Clinical findings
 Management

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Ebsteins anomaly
 Age of presentation
 Clinical findings
 Management

13. 01/09/15 13
Acyanotic with a shunt
 Shunt at atrial level
ASD
PAPVC
 Shunt at ventricular level
VSD
 Shunt at great artery level
PDA
AP Window
 Shunt at more than one level

14. 01/09/15 14
VSD
 Types – based on location
 Size
 With or without PAH
 Associations
 Clinical features
 Management

15. 01/09/15 15
ASD
 Types
 Associations
 Clinical features
 D/D
 Management

16. 01/09/15 16

17. 01/09/15 17
PDA
 Age
 Symptoms
 Signs
 D/D
 Management

18. 01/09/15 18
Cyanosis
Definition
Central // Peripheral
Differential / Reverse differential
Causes
Non Cardiac
Respiratory
Vascular causes
Hematological
Cardiac
Congenital cyanotic heart disease
Eisenmenger’s Syndrome

19. 01/09/15 19
Cyanotic lesions
 Tetralogy of Fallots
 Tricuspid atresia
 Transposition of Great arteries
 Truncus arteriosus
 Single ventricle
 Hypoplastic left heart syndrome
 Eisenmenger Syndrome

20. 01/09/15 20
Classification of Cong Cyanotic
Heart diseases
Pulmonary stenosis, without VSD
Critical PS, Ebsteins
Pulmonary Stenosis with Large VSD
TOF
Increased Pulmonary flow with/ without PAH
TGA ( Transposition of great arteries )
Decreased pulmonary flow with PAH
Eisenmenger Syndrome
Pulm venous congestion with PAH
TAPVC, HLHS
Cyanosis without Pulm stenosis , Normal PA pressure
Single atrium, Pulm AV fistula

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Tetralogy of Fallots

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Transposition of Great Vessels

23. 01/09/15 23
Hypoplastic Left Heart Syndrome

24. 01/09/15 24
Clinical Characteristics of TOF
Prominent a wave in JVP
Normal heart size
Mild parasternal impulse
Systolic thrill – though uncommon
Single second sound ( P 2 absent )
Ejection systolic murmur
Diastolic period clear
Decreased pulmonary flow on Chest X ray

25. 01/09/15 25
Congenital Cyanotic Heart diseases
Ejection Systolic Murmur / Pan systolic murmur
Continuous murmur
TOF with
PDA
Bronchial collaterals
Surgically created shunts ( BT shunt )
Peripheral PS

26. 01/09/15 26
DIFFERENTIAL DIAGNOSIS
Tetralogy of Fallot
Transposition of Great Arteries, VSD, PS
Tricuspid atresia, VSD, PS
Single ventricle, PS
Double outlet right ventricle, VSD, PS
Corrected transposition of great arteries, VSD,PS
AV Canal defects with PS
Eisenmenger’s Syndrome

27. 01/09/15 27
Assessment of Severity
Cyanosis – More the cyanosis more severe the disease
but mild cyanosis also to be taken seriously
Age of onset – earlier the onset more severe the lesion
Symptoms – more the symptoms more severe the
disease

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Investigations
 ECG
 X Ray Chest PA view
 Echocardiography
 Holter monitoring
 MRI
 Cardiac Cath

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ECG in CHD
 Rate, Rhythm
 Look at P wave in Lead I
 PR interval
 QRS axis ( Left / Right )
 RVH, LVH, Bivebtricular Hypertrophy
 Incomplete / Complete RBBB

30. 01/09/15 30
X Ray Chest
 Cardiac Size
 C T Ratio
 Pulmonary Vascularity
 Classical images

31. 01/09/15 31
Echocardiography
 2DEcho
 M mode
 Doppler
 Pulsed wave, Continuous wave, Colour
 Tranesophageal ( TEE )
 3 D Echo

32. 01/09/15 32
Management of TOF
 Age of presentation
 Severity of symptoms
 Anatomical considerations
 Palliative - Shunts
 Definitive

33. 01/09/15 33
Management of Complex
CHD
 Define anatomy by Echo, if required
Cath/ MRI/ CT Angio
 Decide whether Two Ventricle repair is
possible or not
 Glenn shunt
 Fontan repair

34. 01/09/15 34
Bidirectional Glenn
 W What is the purpose
of the Glenn?
 I

35. 01/09/15 35
FontanFontan

36. 01/09/15 36
Reversal of shunt in following with development of
Pulmonary arterial hypertension
Eisenmenger’s Syndrome
Shunt at atrial level
ASD
PAPVC
Shunt at ventricular level
VSD
Shunt at great artery level
PDA
AP Window
Shunt at more than one level
AV Canal defect
Patients with Cyanotic Heart diseases with Increased pulmonary blood
flow – TGA, TAPVC

37. 01/09/15 37
Characteristics with
Eisenmenger physiology
History of frequent chest infections
Age of onset of cyanosis?
No cardiomegaly or thrill
No parasternal heave
Constant ejection click of PAH
Palpable P 2
Diastolic murmur of PR or systolic
murmur of TR

38. 01/09/15 38
Management of
Eisenmenger’s Syndrome
 Manage Polycythemia
 Drug management of PAH
 Heart Lung Transplantation

39. 01/09/15 39
Common Pediatric Cardiac
Emergency Conditions
 Heart failure
 Cyanotic Spell
 Duct dependent Circulation –
Pulmonary atresia , aortic atresia , TGA
 Cardiac Arrhythmias
 Post operative state

40. 01/09/15 40
CHF in Fetal Life
 Supraventricular Tachyarrythmias
 AV Block
 AV Regurgitation
 Severe PR / TR
 EFE
 Severe anaemia
 Cardiomyopathy
 Myocarditis, Storage Diseases

41. 01/09/15 41
CHF on Day 1 of Life
 Structural Heart Defects
 Rhythm Abnormalities
 AV fistulas
 Heart muscle dysfuntion

42. 01/09/15 42
CHF in First week of Life
Structural Abnormalities
 Critical Aortic Stenosis
 Coarctation of Aorta
 Interrupted Aortic Arch
 Hypoplastic Left Heart Syndrome
 Critical Pulmonary Stenosis
 PDA in premature Babies

43. 01/09/15 43
CHF in First 2 months of Life
Structural Abnormalities
 Aortic level shunts
 Ventricular level shunts
 Left sided obstructive lesions
 Atrial level shunts
 Anomalous origin of Coronary
Arteries

44. 01/09/15 44
Clinical Recognition
 Respiratory rate & Effort
 Heart Rate
 Feeding Difficulties
 Exercise intolerance
 Excessive sweating
 Weight gain
 Cardiomegaly
 Hepatomegaly

45. 01/09/15 45
Principles of Therapy
 Removal of underlying cause
- Surgical Correction
- Medical m/m of IE
 Removal of Precipitating cause
- Intercurrent Infections
- Arryhthmias
- Anemia

46. 01/09/15 46
DUCT DEPENDENT PULMONARY
CIRCULATION
 DEFINITION
Complete absence or severe restriction
of antegrade pulmonary blood flow
resulting in severe hypoxemia with
dependance on a patent arterial duct to
maintain pulmonary perfusion
compatible with life

47. 01/09/15 47
Duct Dependant Pulmonary
Circulation
 ETIOLOGY
A) Anatomical restriction/discontinuity of
ventricle and pulmonary artery
B) Admixture lesions- TGA
C) Functional pulmonary atresia
 Severe Ebsteins anomaly
 Hypoplastic right ventricle without PS

48. 01/09/15 48
DUCT DEPENDANT PULMONARY
CIRCULATION
Clinical Presentation
 90% have progressive hypoxemia,
cyanosis,acidosis 1-7 days after birth
 5% present in
infancy/childhood,adolescent
 Rarely heart failure ( PS with TR, PA
intact septum)

49. 01/09/15 49
DUCT DEPENDANT PULMONARY
CIRCULATION
Clinical Presentation
 Blood Gas
 Low Po2, Normal PCO2,Metabolic acidosis
 Hyperoxia test- Po2 < 150Torr

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DUCT DEPENDANT PULMONARY
CIRCULATION
DIAGNOSIS
 Echocardiography
 Defines anatomy
 Duct size
 Pulmonary artery anatomy

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DUCT DEPENDANT PULMONARY
CIRCULATION
MANAGEMENT
 Early recognition- Key
 Structural diagnosis- Less crucial

52. 01/09/15 52
DUCT DEPENDANT PULMONARY
CIRCULATION
MANAGEMENT
 Secure good I/V and I/A line
 Correction of acidosis
 Volume -colloid(5% Albumin 5-10ml/Kg)

53. 01/09/15 53
DUCT DEPENDANT PULMONARY
CIRCULATION
MANAGEMENT
 Prostaglandin- Life saving
 Oxygen(?)
 Balloon Dilation of duct
 Stenting of Duct
 Surgery

54. 01/09/15 54
DUCT DEPENDANT PULMONARY
CIRCULATION
MANAGEMENT
 Prostaglandin
 Before transfer-0.01mcg/kg/min I/V
 Tertiary centre- 0.1mcg/kg/min scale to
0.05-0.01mcg/kg/min I/V
 Oral- 12-65 mcg/kg at 4hrly interval

55. 01/09/15 55
DUCT DEPENDANT PULMONARY
CIRCULATION
MANAGEMENT
 Prostaglandin- predictors of
response
 Widely open duct- Nil
 Closed duct-Nil
 Constricted duct- Best response

56. 01/09/15 56
DUCT DEPENDANT
PULMONARY CIRCULATION
Prostaglandin - side
effects
 Apnea
 Hypotension
 Fever
 Edema
 Periostiitis (20%)
 Subcutaneous fat
necrosis
 Fragile ductus
 Aneurysm, rupture

57. 01/09/15 57
DUCT DEPENDANT PULMONARY
CIRCULATION
 VENTILATION
 Transport to tertiary centre
 Apnea of prostaglandin( premature, LBW)
 Stabilisation of the sick child

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TRANSPOSITION COMPLEX
TGA intact septum/Small VSD
Diagnosis- Echocardiography
 100% detection rate
 ASD size- need for septostomy
 PDA size-need for prostaglandin

59. 01/09/15 59
TRANSPOSITION COMPLEX
TGA intact septum/Small VSD
Management
a) Stable, no acidosis - BAS Plan Sx
b) Acidosis,severe hypoxemia- PGE
BAS
c) Transfer- PGE (?Ventilation) BAS

60. 01/09/15 60
Surgical management
 Arterial Switch Operation
 Venous Switch Operation

61. 01/09/15 61
CARDIAC ARRHYTMIAS
 Complete Heart Block
 Supraventricular Tachycardia
 Ventricular Tachycardia - Rare

62. 01/09/15 62
CARDIAC ARRHYTMIAS
COMMONEST ARRHYTHMIA - SUPRAVENTRICULAR
 Well tolerated in majority
 In neonates and infants difficult to
differentiate from physiological
response- fever, stress
 Prolonged unrecognized SVT- DCM

63. 01/09/15 63
Clinical Presentation
 Irregular Heart beat
 Unexplained cardiac failure
 Underlying structural heart disease
 Syncopal attacks
 Palpitations ,Chest discomfort
 Haemodynamic Instability
 Family H/O sudden cardiac events

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Long term treatment
Long term treatment till cath ablation is
safe or LV dysfunction
 Digoxin/B blockers
 Verapamil
 Amiodarone/Sotalol
 Flecanide

65. 01/09/15 65
 Symptomatic second or third degree AV
block
 SA node Dysfunction , symptoms
correlating with ↓ HR
 Persistent ( > 7days) post op second or
third degree AV block
contd….
Indications of Permanent Pacemaker Implantation

66. 01/09/15 66
Indications of Permanent Pacemaker
Implantation
 Congenital AV Block with
a. Wide QRS escape
b. HR < 50-55 bpm in infancy
without
ass structural CHD
c. HR < 70 bpm with ass structural
CHD

67. Therapeutic Cardiac
Catherisation in Pediatrics

68. 01/09/15 68
GENERAL PRECAUTIONS
 Maintenance of ABC
 Maintenance of temperature
 Heparinization-100 units/kg- repeat if
reqd
 Arterial/ Venous approach
 Prostaglandins
 Oxygen

69. 01/09/15 69
INTERVENTIONS
 Creation of a defect as palliation
 Balloon dilatation of stenotic valves
 Coil embolization / Balloon angioplasty
 Stenting of PDA
 Device closures

70. 01/09/15 70
Balloon Atrial Septostomy
 Indications
 Procedure

71. 01/09/15 71
BALLOON ATRIAL
SEPTOSTOMY
ECHO GUIDEECHO GUIDE

72. 01/09/15 72
SUCCESSFUL
SEPTOSTOMY
 Cinical improvement
 Equalization of atrial pressures
 Change in arterial O2 saturation
 Angiogram before and after septostomy
 Increase in arterial pressure
 Decrease in pulmonary arterial pressure
 Balloon calibration of defect
 Echo visualization of defect

73. 01/09/15 73
BALLOON DILATATION OF
STENOTIC VALVES
 Balloon dialtation of
 Aortic valve
 Pulmonary valve
 Coarctation of aorta

74. 01/09/15 74
BALLOON DILATATION OF
THE AORTIC VALVE
 Palliative procedure
 Procedural success is almost 100%
 Mortality may be high if associated conditions
 Endocardial fibroelastosis
 LV hypoplasia
 Others
 Risks- aortic regurgitation

75. 01/09/15 75
BALLOON DILATATION OF
AORTIC VALVE
 Indications
 Procedure

76. 01/09/15 76
AORTIC VALVULOPLASTY
PRE DILATATIONPRE DILATATION BALLOON ACROSSBALLOON ACROSS
THE AORTIC VALVETHE AORTIC VALVE
POST DILATATIONPOST DILATATION
NO ARNO AR

77. 01/09/15 77
BALLOON DILATATION OF
PULMONARY VALVE
Neonatal critical Pulmonary stenosis
 Majority present during first week
 Duct dependant (require PGE1)
 RV morphology- well developed or
poorly developed
 Echo assessment
 Immediate success rate > 80%

78. 01/09/15 78
x
VALVULOPLASTY

79. 01/09/15 79
Pulmonary valvotomy and VSD closurePulmonary valvotomy and VSD closure
Effective balloon diameter should be 1.2 to 1.4 timesEffective balloon diameter should be 1.2 to 1.4 times
the measured Pulmonary Valve Annulusthe measured Pulmonary Valve Annulus

80. 01/09/15 80
Pulmonary valvotomy and VSD closurePulmonary valvotomy and VSD closure
Effective balloon diameter should be 1.2 to 1.4 timesEffective balloon diameter should be 1.2 to 1.4 times
the measured Pulmonary Valve Annulusthe measured Pulmonary Valve Annulus

81. 01/09/15 81
Pulmonary valvotomy and VSD closurePulmonary valvotomy and VSD closure
Effective balloon diameter should be 1.2 to 1.4 timesEffective balloon diameter should be 1.2 to 1.4 times
the measured Pulmonary Valve Annulusthe measured Pulmonary Valve Annulus

82. 01/09/15 82
BALLOON DILATATION OF
COARCTATION
 Controversial role in neonates
 Indications
 Symptomatic newborn
 CCF
 Failure to thrive
 Upper extremity hypertension
 Severe LV dysplasia
 Severe PAH

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88. 01/09/15 88
COIL EMBOLIZATION
 Indications
 Large AVM
 Aortopulmonary collaterals
 Systemic venous anomalies
 PDA (if large, unrestrictive- surgery ideal)

89. 01/09/15 89
STENTING OF PDA
 Ductal dependent pulmonary blood flow
 Difficult procedure
 Limited experience
 Intimal proliferation is universal thus
need for reintervention

90. 01/09/15 90
Device Closures
 PDA
 ASD
 MUSCULAR VSD
 PERI MEMBRANOUS VSD
 IN OTHER PLACES e.g
Coronary AV fistula
Peri valvular leaks

91. 01/09/15 91
Closure of PDA
 Device closure – for almost any duct
 Contra indications
Large PDA in a neonate
Associated reversal of shunt

92. 01/09/15 92

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94. 01/09/15 94
Coil closure of PDA
 Coil closure for small ducts
 Coil made up of stainless steel wire with
Dacron strands – promote thrombosis
 Cheap
 PROCEDURE

95. 01/09/15 95
DETACHABLE COIL

96. 01/09/15 96
Device closure of ASD
 Amplatzer septal occluder device is most
widely used
 Only used for Secundum ASD
 Continuous TEE monitoring
 Adequate rim is required otherwise any size
of ASD can be closed
 PROCEDURE

97. 01/09/15 97
(ASD)(ASD)

98. 01/09/15 98
Approach

99. 01/09/15 99
The Device
The device is made
up of a NITINOL
(Nickel-Titanium
Naval Ordanance
Laboratory).
Dacron fibre - within
the device

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Device closure of VSD
 Amplatzer device for Muscular VSD
 Amplatzer device for Peri membranous
VSD – defect >5 mm away from aortic
valve
 PROCEDURE

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Pediatric Cardiology: Summary
•1
•11938-Essentially no Rx for CHD
–PPioneers-pathologists, Pediatricians, Cardiologists,
Surgeons, Imaging experts, Intensivists, Interventionalists
•22008-Rx for virtually all CHD, BUT mort./morb.:
–vVentricular function
–aArrhythmia
– Valves/conduits
–pPulm hypertension
•N

105. 01/09/15 105
FUTURE OF INTERVENTIONAL
CARDIOLOGY
1. Percutaneous pulmonary valve replacement
2. Percutaneous aortic valve replacement
3. VSD closure devices
4. Percutaneous banding devices
5. RF perforation
6. Drug eluting stents
7. Fetal intervention- therapy for aortic
stenosis

106. THANK YOU
09922923383
drprabhat_cardio@yahoo.co.in