Description of common congenerous heart diseases

1. Congenital Heart
Diseases in Children
Presented by-
Dr. Writtika Majumdar (DCH student)
Dr. MD. Ashik Kamal Alvee (MD Student)
Dept. of Paediatrics
Dhaka Medical College Hospital
Seminar on

2. Objectives:
Epidemiology of CHD
Developmental changes of Heart
Risk factors
Classification
Approach to a child with CHD
Discussion about common CHD including important clinical
findings, investigations, treatment and natural history.

3. What Is Congenital Heart Disease
▪ Congenital heart diseases are problems with the heart's structure
that are present at birth.
▪ These defects can involve:
-The interior walls of the heart
-The valves inside the heart
-The arteries and veins that carry blood to the heart or out to
the body

4. Epidemiology
• CHD occurs in approx. 0.8% of live births.
• Incidence is higher in stillborns (3-4%), Spontaneous abortuses
(10-25%) and premature infants.
• Approx. 2-3/1000 newborn will be symptomatic with heart
disease in 1st year of life.
• WHO reports the incidence of CHD
in Bangladesh is 6% (2015)

5. Development Of Heart

6. The Fetal Circulation

7. • Closure of umbilical arteries
• Closure of umbilical vein
• Closure of ductus venosus
• Closure of ductus arteriosus
• Closure of foramen ovale
• Enlargement of pulmonary veins
Changes in fetal circulation after birth

8. Maternal
drug use
Chromosomal
abnormality
Maternal
diseases
Risk Factors For CHDs
• Rubella infection:
PDA, PS
• SLE: Complete
Heart Block
• DM, HTN
• Maternal CHD
• Epilepsy
• Anemia
• Down syndrome:
ASD, VSD
• Turner syndrome:
CoA, Aortic stenosis
• Marfan’s syndrome
Aortic aneurysm
• Warferin therapy:
PDA,PS
• Fetal alcohol
syndrome: ASD,
VSD, TOF
• Thalidomide
• Sulfonamide
• Anti epileptic drug

9. Classification Of CHD
Acyanotic Cyanotic
Left to right
shunt
Outflow
obstruction
Ventricular septal
defect
Atrial septal
defect
Persistent ductus
arteriosus
Pulmonary
Stenosis
Aortic Stenosis
Coarctation of
aorta
Tetralogy of Fallot (TOF)
Transposition of the great arteries
Tricuspid Atresia
Truncus arteriosus
Total anomalous pulmonary venous
return
Common Ventricle

10. Relative Frequency of Common Congenital
Heart Defects
Ventricular septal defect
Atrial septal defect (Secundum)
Patent ductus arteriosus
Coarctation of aorta
Tetralogy of Fallot
Pulmonary Valve Stenosis
Aortic Valve Stenosis
Transposition of great arteries
Hypoplastic left ventricle
30-35%
6-8%
6-8%
5-7%
5-7%
5-7%
4-7%
3-5%
1-3%

11. How To Approach
a Patient With
Congenital Heart
Disease

12. Approach to a patient with CHD
History Taking
Physical
Examination
Investigations

13. HISTORY
TAKING

14. Acyanotic Heart Disease Cyanotic Heart Disease
• Feeding difficulty
• Dyspnea or shortness of
breath
• Excessive sweating during
exertion
• Recurrent respiratory
infections
• Growth impairment
• Exercise intolerance
• Easy fatigability
• Bluish or blackish
discoloration
From Birth: TGA, TAPVR
Later onset: TOF
• Adoption of squatting position
(after 18 month ususally)
• Convulsion/Unconsciousness
• Sudden weakness of one
side of body
History of present Illness

15. Birth History
Maternal history Perinatal history
• Exposure to drugs
• Alcohol intake
• TORCH infection
• Any chronic Disease
• Exposure to radiation or
chemical
• Bad obstetrical history
• Cyanosis at birth
• Gestational age
• Birth weight
• Features of heart failure
• Any birth defects (heart-
related or not)

16. Family History
• When 2 first degree relatives have CHD, there is risk of
20-30% for subsequent child.
• If 1st born has CHD, risk of a 2nd child is 2-6%
• History of chromosomal abnormality( Down, Marfan, Turner)
• History of still birth/ spontaneous abortion

17. Physical
Examination

18. General Examination
• Appearance:
-Ill looking
-Features of Down syndrome,
Turner syndrome,
Marfan syndrome may be present
• Cyanosis: Central or Peripheral
• Pallor (due to malnutrition, shock)
• Plethora (TOF)
• Clubbing
• Edema
• Engorged neck vein (in older children)

19. Anemia Clubbing
Plethora Cyanosis

20. ▪ Respiratory rate: Tachypnea may be present
▪ Pulse:
General Examination
Bradycardia : complete heart block in TGA
High volume pulse with wide pulse pressure: AR in TOF
Collapsing pulse: PDA
Radiofemoral delay: CoA

21. • Blood Pressure
Upper extremities HTN: CoA
(CoA is suspected when systolic pressure <20mm hg in legs
than arms.)
A narrow pulse pressure: severe AS.
• Spo2
Low in cyanotic CHD or CoA,
PDA with pulmonary HTN.
General Examination

22. Anthropometry
• Growth impairment
• Low birth weight, premature infant have more
chance of CHD.

23. Precordium Examination
• Inspection
Bulged precordium- VSD, ASD, TOF
Hyperdynamic precordium- VSD ,PDA
• Palpation
Abnormal apex beat
-Tapping: Mitral stenosis
-Thrusting : MR, AR
-Heaving : AS, Systemic HTN
-Double apical impulse: Hypertrophic cardiomyopathy

24. Thrill:
-Left sternal border- VSD
-Rt upper sternal border- Severe AS
Left parasternal heave:
-Indicates RVH
Precordium Examination

25. • Auscultation
Heart sounds
Added sounds
-Murmurs
-Pericardial rub
Precordium Examination

26. • Liver: Palpable in CCF
• Spleen: Palpable in IE
• Ascites: Heart failure
• Focal neurological sign:
Cerebral abcess (TOF, VSD)
Systemic examination

27. Investigations

28. • Chest X-ray
• ECG
• Echocardiography
• Cardiac Catheterization
• Hematological investigations
-Complete blood count
-ESR, HCT
-CRP
-ASO Titre
Investigations

29. Brief Discussion About
Common Congenital Heart
Defects

30. Acyanotic
Heart Disease
Left to Right shunt

31. • The commonest congenital cardiac disease (30-35%)
• Can occur as Isolated defect or component of other
complex defect such as TOF
• May occur in any portion of ventricular septum but
most are membranous type
Ventricular septal defect

32. Types of VSD
According To Location:
▪ Membranous VSD : 70-80 %
▪ Muscular VSD : 5-20 %
▪ Supracristal :5-30 %
▪ Endocardial cushion: 5-8%
According to size:
▪ Small : < 5mm
▪ Moderate: 5-10mm
▪ Large: > 10mm

33. Hemodynamics Of VSD

34. VSD: Symptoms
Small defect Asymptomatic
Failure to thrive
Recurrent URTI
Profuse perspiration
Moderate to
Large defect

35. VSD: Physical findings
VSD
Bulged
precordium
Thrill at lower
left sternal
border
Harsh pansystolic
murmur in left 3rd-5th
ICS
Palpable P2 & left
parasternal heave

36. VSD: Investigations
Chest X-ray:
• Cardiomegaly, increase vascular marking.
ECG:
• Large VSD: LVH, Biventricular hypertrophy
Echocardiogram:
• Position, size of VSD, shunt size, pressure gradient
Cardiac catheterization:
• When laboratory data not fits or suspected
pulmonary vascular disease

37. Treatment Of VSD
Small VSD:
• Reassurance
• No restriction on physical activity

38. Moderate to large VSD:
1.Medical treatment
• Diuretic: Frusemide (1-3mg/kg/day)
• Afterload reducing agent:
-Enalapril (0.1-0.5mg/kg/day-12 hourly)
-Captopril( 0.05-0.1mg/kg/dose-8hourly)
-Digoxin: if f/o heart failure present
2.Transcatheter device closure
3.Surgical treartment
Treatment Of VSD

39. Treatment Of VSD contd.
Indications of surgical treatment:
• At any age: Large VSD where clinical symptoms & FTT
can not be controlled medically
• At 6-12 months: Large VSD with pulmonary HTN (even
if symptoms controlled by medication)
• At age >24month: Qp:Qs ratio>2:1
Contraindication of surgery:
• Severe pulmonary vascular disease, non responsive to
pulmonary vasodilator

40. Prognosis of VSD
• Small VSD: closed spontaneously 30-50% during 1st 2 year
of life ( muscular upto 80%, membranous 35%)
• Moderate to large VSD closes upto 8%
• Supracristal variety: never closes spontaneously

41. Atrial Septal Defect
• ASD is an opening permitting shunting of blood
between two atria
• Female: male -3:1
Types of ASD:
▪ Ostium Secondum
(commonest)
▪ Ostium Primum
▪ Sinus Venosus type

42. Hemodynamics of ASD

43. ASD: Symptoms
Small defect Asymptomatic
Failure to thrive
Recurrent URTI
Moderate to
Large defect

44. ASD: Physical findings
ASD
Bulged
precordium
Widely splitted S2
Ejection systolic murmur
in ULSB
Palpable P2 & left
parasternal heave

45. ASD: investigation
Chest x-ray:
• Cardiomegaly with
increased pulmonary
vasculature marking.

46. ECG:
• Large defect: Right axis
deviation due to RVH
Confirmatory diagnosis:
Echocardiography
ASD investigation

47. Treatment of ASD
Medical treatment:
-Diuretics ( Frusemide or Thiazide or combination)
-Digoxin
Surgical treatment:
• Small secondum ASD with minimal shunt without right
ventricular enlargement: No need of closure
• Device or surgical closure

48. Transcatheter or surgical closure:
-For all symptomatic patient
-Asymptomatic with Qp:Qs ratio at least 2:1
-Patient with right ventricular enlargement;
Timing of closure:
preferably after 1st year and before entry into school.
Treatment of ASD

49. Devices Used For ASD Closure

50. Prognosis of ASD
• Spontaneous closure of osmium secundum defect is rare
beyond 1st year of life.
• Secundum ASD is well tolerated during childhood ,usually
go asymptomatic detected 3rd decade or later
• Congestive cardiac failure is the most common cause of
death in secundum ASD

51. Patent Ductus Arteriosus (PDA)
▪ PDA results from persistence of ductus arteriosus after birth
▪ Aortic blood shunted left to right into pulmonary artery.
▪ Female :male - 3: 1
▪ Common problem in premature infant.
▪ Maternal rubella infection associated with PDA

52. Haemodynamics of PDA

53. PDA: Symptoms
Small defect Asymptomatic
Failure to thrive
Exercise intolerance
Easy fatiguability
Repeated RTI
Moderate to
Large defect

54. PDA: Physical findings
PDA
High volume collapsing
pulse
Wide Pulse pressure
Apex beat heaving in
nature
Thrill at 2nd left ICS
Continuous machinery
murmur in 2nd left ICS
radiate to left clavicle
Hyper dynamic
precordium

55. PDA: Investigations
Chest X-ray:
• Cardiomegaly
• Increased Pulmonary vascular markings
ECG:
• left ventricular hypertrophy, biventricular hypertrophy
Confirmatory Diagnosis:
• Echocardiogram
• Cardiac catheterization

56. PDA: Diagnosis

57. PDA: Management
1. Medical Treatment:
(effective if given within 72 hours)
• Indomethacin :3 doses, 0.2mg/kg 12-24 hours apart
I/V- slowly over 30 minutes
• Ibuprofen : Oral, For 3 days .
1st day 10mg /kg, 2nd and 3rd dose 5mg/kg
2. Transcatheter device
3. Surgical closure
4. Prophylaxis for infective endocarditis

58. PDA: Management
Surgical Treatment:
• By Transcatheter device or by lateral thoracotomy
• Preferably before 1 year, as Heart failure develops
earlier in PDA.
• Small PDA: By intravascular coils
• Moderate to large PDA: By umbrella like device.

59. PDA: Management

60. Complication of PDA
Blood Ductus
Heart
lungs
• Pulmonary
HTN
• Pulmonary
artery
embolism
• Infective
endocarditis
• Congestive
Cardiac
failure
Systemic
embolism
Calcification
Of ductus

61. Eisenmenger syndrome
• Shunt reversal
• May occur in
VSD
PDA
AV canal defect

62. Cyanotic Heart
Disease
Right to left shunt

63. Tetralogy OF Fallot (TOF)
• First described by Etienne L A Fallot
• Accounts for 5-7% of all congenital heart diseases.
Four components:
• Obstruction of Right ventricular outflow or Pulmonary stenosis
• VSD
• Overriding of the aorta (over the VSD)
• RVH

64. Hemodynamics Of TOF

65. TOF: Pathological Effects
• Less blood in pulmonary circulation
(Oligemic lung field)
• Low oxygen saturation in systemic
circulation (Cyanosis)
• Polycythemia
• Growth failure

66. Symptoms of TOF
• Cyanosis
• Paroxysmal hyper cyanotic attack
• Easy fatigability and dyspnea on exertion
• Growth failure or developmental delay

67. TOF: Hypercyanotic Spell
• Hall mark of severe TOF
• Usually occurs during first 2 year of life, most
commonly at 4-6 month of life.
• Most frequently occurs in the morning on
awakening
or vigorous cry

68. TOF: Hypercyanotic Spell
Hypoxic spells are characterized by:
• Sudden onset of hyperapnea, sometimes
gasping respiration and syncope
• Sudden deepening of cyanosis
• Alteration of consciousness, sometimes
convulsion and hemiparesis
• Temporary disappearance or decrease in the
intensity of the systolic murmur.
• Metabolic acidosis

69. TOF: Signs
General examination: Clubbing
Cyanosis
Polycythemia
Stunting
Examination of Precordium:
• Bulged chest.
• Tapping apex beat
• Left parasternal heave
• Systolic thrill at left upper ICS
• S1 is normal, S2 is loud & single
• A loud ejection systolic murmur at upper sternal border

70. TOF: Diagnosis
CBC:
Hb: Elevated
Haematocrit: Elevated
PBF:
Microcytic hypochromic
anemia
Chest X-ray :
Boot shaped heart
Oligemic lung field

71. TOF: Diagnosis
ECG:
Right axis deviation
RVH
Confirmatory:
Echocardiography

72. Management of TOF
Non-surgical management
• Iron, vitamin and mineral supplementation
• Adequate fluid intake
• High calorie diet
• Oral propranolol 0.25 -1 mg/kg/day prophylaxis

73. Management of TOF
Neonates with severe cyanosis is treated with I/V infusion
of prostaglandin E1
Treatment of cyanotic spell
• Place the infant in knee-chest position.
• Older children squat spontaneously
• Oxygen inhalation
• Inj Morphine

74. Management of TOF
• I/V normal saline 10ml/kg bolus followed by
maintenance fluid
• I/V NaHCO3
• I/V Propranolol
If not controlled: Phenylephrine 10-20
microgram/kg bolus IM or SC, then 0.1-0.5
mircrogram/kg/min infusion IV

75. Management of TOF
Surgical management
▪ Total correction:
▪ Palliative surgery :
Blalock Taussig Shunt

76. Complications of TOF
Blood Growth &
Development
Heart
Brain
• Cerebral
abcess
• Thrombo-
embolism
• Stroke
• Infective
endocarditis
• Cardiac
failure
Severe
polycythemia
Failure of
growth and
development

77. Transposition Of Great Arteries (TGA)
• 2nd most common
cyanotic heart
defect.
• The Aorta arises
from right ventricle
• The pulmonary
trunk arises from
left ventricle

78. TGA: Clinical features
Symptoms
• Cyanosis
• Tachypnea
Signs
• Left parasternal heave may present
• 2nd heart sound single, loud and may split.
• Murmur absent or a soft ejection systolic
murmur may present

79. Chest X ray:
• Egg on string
appearance of heart
• Normal to increased
pulmonary vascular
marking
• Mild cardiomegaly
Investigations of TGA

80. Echocardiogrm
• Transposed
ventricular-arterial
connection
(Confirmation of
diagnosis)
Investigations of TGA

81. Management
Non-invasive management
• IV Prostaglandin
Invasive management
• Balloon atrial septostomy
• Permanent correction
by Atrial switch operation
Balloon atrial septostomy

82. Treatment availability Of CHDs in Bangladesh
Device closure available at:
NICVD, NHF&RI, BSMMU, BICH, BIRDEM, CMH, APOLLO
hospital
Surgical treatments are available at:
Inside Dhaka:
NICVD, NHF&RI, BSMMU, BICH, BIRDEM, Lab Aid
Outside Dhaka:
Fortis Escort Hospital ( Chittagong & Khulna)
Metropolitan Hospital ( Chittagong )

83. 7th -14th February

84. Thank
You…