This document discusses common congenital and acquired heart conditions in children. It begins by describing fetal circulation and how congenital heart diseases arise from defects present at birth. The conditions are classified as acyanotic or cyanotic depending on whether they allow mixing of oxygenated and deoxygenated blood. Common acyanotic conditions discussed include atrial septal defect, ventricular septal defect, and patent ductus arteriosus. Cyanotic conditions include tetralogy of Fallot and transposition of the great vessels. The document also covers acquired conditions like rheumatic fever and infective endocarditis, as well as their presentation, diagnosis, and management.

1. Common Heart Conditons in
Children
Presenters:
• Aqim
• Analicia

2. CONGENITAL
HEART DISEASES

3. CONTENTS
Fetal Circulation
Congenital Heart Diseases (CHD)
Incidence
Mortality and Morbidity
 Etiological Factors
Classification of CHD
Cyanotic
Acyanotic
Hemodynamics of Common CHD
Assessment & Management

4. Dr.Nidhi Ahya(Asst Prof)

5. Fetal Circulation
Blood from
placenta
Inferior Vena
Cava
RightAtrium
Left atrium
via Foramen
Ovale
Left ventricle
Aorta Head & Neck
Returns via
Superior
Vena Cava
RightAtrium
Right
Ventricle
Pulmonary
Artery
Aorta via
Patent Ductus
Arteriosus
Lower
Extremities
Blood to
Placenta

6. CONGENITAL HEART DISEASES
(CHD) These are cardiac anomalies
arising as a result of a defect in
the structure or function of
the heart and great vessels which
is present at birth
These lesions either
obstruct blood flow in the heart
or vessels near it, or alter the
pathway of blood
circulating through the heart

7. CLASSIFICATION OF
CHD
ACYNOTIC
Volume or
Pressure
Overload
PDA, VSD,ASD,
Coartation ofAorta
PINK
BABY
CYNOTIC
OR
Pulmonary
Blood flow
Tetralogy of Fallot,
Transposition of
great vessels,
TricuspidAtresia
BLUE
BABY

8. Atrial Septal Defect
(ASD)  An abnormal opening in the atrial
septum which allows oxygenated
blood from the left atrium to mix
with deoxygenated blood in the right
atrium at a minor pressure
difference
 Right atrium recieves blood from
SVC,IVC as well as from left atrium
leading to volume overload and
pulmonary congestion
 Occurs in about 4-10% of CHD
 More common in female child

9. Types of ASD:
 Ostium Secundum
most common- 50-70%,
In the middle of the septum in
the region of the foramen
ovale
 Ostium primum
30% -Low position
Form of AV septal defect
 Sinus venosus
Least common-10%
Site-at entry of superior
venacava into right atrium

10. Clinical Presentation:
Most infants and children are asymptomatic but over
years to decades may experience the symptoms
depending on type and severity of ASD
 Infant gets tired during feeding
 Child gets tired with playing/eating
 Shortness of breath
 Fatigue
 Sweating
Palpitations
Stunted growth

11. Diagnosis:
On Auscultation-
S1 :normal
S2: Widely split & fixed with P2 accentuated
 Ejection systolic murmur is present

12.  Chest X-ray - Mild to moderate cardiomegaly
with enlarged right atrium & right ventricle,
prominent pulmonary artery segment,
increased pulmonary vascular markings

13. Dr.Nidhi Ahya(Asst Prof)
 ECG- Right Axis Deviation, Right ventricular strain
pattern in lead V1
 Echocardiogram- position, size, signs of LR
shunt, flow

14. Management:
 20% of atrial septal defects will close spontaneously
in the first year of life or as the child grows
 For defects of 3-8mm, or smaller, supportive
medical management – Digoxin, diuretics and
prophylactic antibiotics are sufficient up till
spontaneous closure
 If defect is >8mm, surgical repair may
be is required
 If spontaneous closure does not
occur by school- going age,
surgical repair becomes essential
to prevent lung problems that will
develop from long- time exposure
to extra blood flow
 Surgical repair

16. Ventricular Septal Defect
(VSD)  An abnormal opening in the
ventricular septum which allows
oxygenated blood from the left
ventricle to mix with deoxygenated
blood in the right ventricle
 Right ventricle recieves blood from
right atrium as well as from left
ventricle leading to volume overload
and pulmonary congestion
 VSDs are the most commonly
occurring type of congenital heart
defect, occurring in 14-17 % of babies
born each year

17. Dr.Nidhi Ahya(Asst Prof) 16

18. Types of VSD:
 Supracristal VSD
occurs just beneath the
aortic valve at the left
ventricular outflow tract
 Membranous VSD
The most common type
and originate inferior to
the crista
supraventricularis
 Muscular VSD
Occur in the mid to apical
interventricular septum

19. Clinical Presentation:
 Signs and symptoms vary with the size of the
defect.
 Clinical symptoms are usually not seen at birth
because of continued high pulmonary vascular
resistance in the newborn
 Infants with moderate to large defects will become
symptomatic within the first few weeks of life.
Shortness of breath while feeding
Poor growth
Failure to gain weight
Pounding Heart
Frequent respiratory tract infections
shunt occurs- cynosis, clubbing,
If reversal of
respiratory distress

20. Diagnosis:
 On Auscultation-
Pansystolic murmur is present
S1 is masked by the murmur
S3 can be heard at the apex

21. Chest X-ray- Cardiomegaly and incresed pulmonary
vascular markings

22. Management :
 Medical management
 digoxin
Diuretics
 Adequate nutrition
high-calorie formula or breast milk
supplemental tube feedings
Prophylactic antibiotics to prevent bacterial
endocarditis

23.  Surgical repair – closed stitches
or special patch
 Interventional cardiac
catheterization – Septal occluder
 Outcome of Surgery- 95%
success rate, growth of child
catches up in 1-2 years, size of
the heart reduces, murmurs can
be heard 2-3 months post-
operative also but hold very little
clinical importance

24. Patent Ductus Arteriosis
(PDA)
 Failure of closure of ductus
arteriosus
 Incidence: Mostly in premature
infants or infants born to a mother
who had rubella during the first
trimester of pregnancy
 Through the PDA -> oxygenated
blood passes from the aorta to the
pulmonary artery & mixes with the
deoxygenated blood which goes to
the lungs -> Increased blood
volume to the lungs -> pulmonary
hypertension and congestion

25. Dr.Nidhi Ahya(Asst Prof)
 As blood is pumped at high pressure through the
PDA, the lining of the pulmonary artery will
become irritated and inflamed. Bacteria in the
bloodstream can easily infect this injured area
bacterial endocarditis

26. Clinical Presentation:
 Shortness of breath
 Congested breathing
 Disinterest in feeding, or tiring while feeding
 Poor weight gain
 Sweating
 Tachypnea
 Bounding pulse

27. Diagnosis:
 On Auscultation-
Continuous machinery murmur in the left
infraclavicular region

28.  Management:
Medical Management
•Indomethacin IV (prostaglandin inhibitor) may
help close a PDA. It works by stimulating the
muscles inside the PDA to constrict, thereby
closing the connection
•Digoxin
•Diuretics
Adequate nutrition
• High-calorie formula or breast milk
•Special nutritional supplements may be added to
formula or pumped breast milk that increase the
number of calories in each ounce

29. Surgical Management
•Repair is usually indicated in infants younger than 6
months of age who have large defects that are
causing symptoms, such as poor weight gain and
rapid breathing
 Transcatheter coil closure of the PDA
PDA ligation-involves closing the open PDAwith
stitches or the vessel connecting the aorta and
pulmonary artery may be cut and cauterized

30. CYNOTIC HEART DISEASE
These type of defects lead to either
increased or decreased pulmonary
blood flow
The primary pathology arises either
due to an obstructive lesion; or due to
abnormal anatomy or both
The shunt present is predominantly
from Right to Left leading to shunting of
venous blood without passing through
the lungs to be oxygenated
Unoxygenated blood circulates in
arteries - > cyanosis
Example: Tetralogy of Fallot,TGV

31. Tetralogy of Fallot (TOF)
 A complex condition of several
congenital defects that occur due to
abnormal devlopment of the fetal
heart during the first 8 weeks of
pregnancy
 ‘Tetra’ meaning ‘four’
Ventricular septal defect (VSD)
Pulmonary valve stenosis
Overriding aorta
Right ventricular hypertrophy

33.  Due to pulmonary artery stenosis, RV has to work
harder to push blood into the lungs, thereby
increasing the RV pressure and size
 Presence of VSD facilitates blood to pass from the
RV into the left ventricle, and mixing of blood takes
place.
 Overriding of aorta- The aorta sits above both the
left and right ventricles over the VSD, rather than
just over the left ventricle. As a result, oxygen poor
blood from the right ventricle can flow directly into
the aorta instead of into the pulmonary artery to the
lungs
 Decresed pulmonary blood flow and poorly
oxygenated blood circulating throughout the body
leads to CYNOSIS

34. Clinical Presentation:
Cyanosis - (bluish color of the skin, lips, and nail
beds) that occurs with such activity as crying or
feeding
 Irritability
 Lethargic
 Reduced physical activity
 Fainting
 Clubbing of nails of fingers/toes
 Breathing difficulty

35. Diagnosis:
On Auscultation- An ejection systolic murmur is
present at the Left parasternal region 3rd ICS due
to pulmonary stenosis.

36. Investigations
Chest radiograph
• Boot shaped heart
• Unturned apex
• Pulmonary artery bay
• Oligemic lung fields
• Enlarged aorta

37. Investigations
• ECG
• RVH (p-pulmonale (Right atrium),
tall R waves in V1, upright T wave
in V1.
• Marked right axis deviation
• Echo
• Detection of main features
• Quantification of right ventricular
outflow pressure gradient
• May be performed with cardiac
catheterization

38. Management :
 Requires surgical repair usually undertaken at 6-18
months age
 It involves- closure of VSD with a tangential patch
to correct the override and the pulmonary stenosis
is relieved with a patch).

39. Transposition of Great Vessels
(TOG)  The aorta is connected to the right
ventricle, and the pulmonary artery is
connected to the left ventricle
 Oxygen-poor (blue) blood returns to
the right atrium from the body - >
passes through the right atrium and
ventricle, -> into the misconnected
aorta back to the body.
 Oxygen-rich (red) blood returns to the
left atrium from the lungs - > passes
through the left atrium and ventricle, ->
into the pulmonary artery and back to
the lungs.

41. Clinical Presentation:
Cyanosis - (bluish color of the skin, lips, and nail
beds) that occurs with such activity as crying or
feeding
 Rapid and laboured breathing
 Cold and clammy skin
Failure to thrive

42. Investigations
• Echocardiography
(confirmatory)
• Chest radiograph
1. Egg on a string heart
2. Increased pulmonary
vascular markings
• ECG : normal

43. Management:
 Admitted to NICU
 On ventilator support
 Cardiac Catheterization
 Ballon Atrial Septostomy
 I.v. Prostaglandins administered
 By 2nd week of life, TGA repair
is done
 ‘Switch’ operation

44. Hypercyanotic spell

45. Introduction
• Sudden severe episodes of intense cyanosis caused by reduction of
pulmonary flow in patients with underlying Tetralogy of Fallot or other
cyanotic heart lesions.
• This is due to spasm of the right ventricular outflow tract or reduction in
systemic vascular resistance (e.g. hypovolaemia) with resulting increased in
right to left shunt across the VSD.

46. Clinical Presentation
• Peak incidence age: 3 to 6 months.
• Often in the morning, can be precipitated by crying,
feeding, defaecation.
• Severe cyanosis, hyperpnoea, metabolic acidosis.
• In severe cases, may lead to syncope, seizure, stroke or
death.

47. Acute management
1. Knee-chest/squatting position
2. Give IV/IM/SC morphine 0.1 – 0.2 mg/kg
If the above measures fail:
1. Give IV Propranolol 0.05 – 0.1 mg/kg slow bolus over 10 mins.
2. Alternatively, IV Esmolol 0.5 mg/kg slow bolus over 1 min, followed by 0.05
mg/kg/min for 4 mins.
3. Volume expander (crystalloid or colloid) 20 ml/kg rapid IV push to increase
preload.
4. Give IV sodium bicarbonate 1 – 2 mEq/kg to correct metabolic acidosis.
5. Heavy sedation, intubation and mechanical ventilation.

48. Acute management
In resistant cases, consider
• IV Phenylephrine (0.01 – 0.02 mg/kg slow bolus)
• or Noradrenaline infusion (0.1 – 0.5 mcg/kg/min)
• Emergency Blalock Taussig shunt.
Notes:
• A single episode of hypercyanotic spell is an indication for early surgical referral (either total
repair or Blalock Taussig shunt).
• Oral propranolol 0.2 – 1 mg/kg/dose 8 to 12 hourly should be started soon after stabilization
while waiting for surgical intervention.

49. Acquired Heart Disease

50. Contents
• Rheumatic fever
• Infective endocarditis
• Kawasaki disease

51. Rheumatic Fever
• Rare in the developed world but remains the most
important cause of heart disease in children
worldwide.
• Acute rheumatic fever is a short-lived, multisystem
autoimmune response to a preceding infection with
group A beta-haemolytic streptococcus.
• The disease mainly affects children aged 5-15 years.
• It progresses to chronic rheumatic heart disease in up
to 80% of cases.

52. Clinical features
• After a latent interval of 2-6 weeks following a
pharyngeal or skin infection, polyarthritis, mild
fever and malaise develop.
• Making the diagnosis (Jones Criteria)
[ 2 major criteria or 1 major + 2 minor
criteria + evidence of a preceding group A
streptococcal infection]

54. Treatment
•Aim to suppress inflammatory response so as to minimize cardiac
damage, provide symptomatic relief and eradicate pharyngeal
streptococcal infection.
• Bed rest. Restrict activity until acute phase reactants return to normal.
• Anti-streptococcal therapy:
- IV C. Penicillin 50000U/kg/dose 6H or Oral Penicillin V 250mg 6H
(<30kg), 500mg 6H (>30kg) for 10 days
- Oral Erythromycin for 10 days if allergic to penicillin.
• Anti-inflammatory therapy
- Mild/ no carditis (Oral aspirin 80-100mg/kg/day in 4 doses for 2-4
weeks, tapering over 4 weeks)
- Pericarditis, or moderate to severe carditis (Oral Prednisolone 2
mg/kg/day in 2 divided doses for 2-4 weeks, taper with addition of
aspirin as above.

55. • Anti-failure medications
- Diuretics, ACE inhibitors, digoxin (to be used with
caution)
**Consider early referral to a Paediatric cardiologist if
heart failure persists or worsens during the acute phase
despite aggressive medical therapy. Surgery may be
indicated.
• Secondary prophylaxis of rheumatic fever
- IM Benzathine Penicillin 0.6 mega unit (<30kg) or 1.2
mega units (>30kg) every 3 to 4 weeks.
- Oral Penicillin V 250mg twice daily.
- Oral Erythromycin 250mg twice daily if allergic to
Penicillin.
• Duration of prophylaxis
- Until age 21 years or 5 years after last attack of ARF
whichever was longer.
- Lifelong for patients with carditis and valvular
involvement.

56. Infective
Endocarditis

57. Definition – Risk factors
Is an infection of the endocardial surface of the heart which
frequently involves the heart valves
• Highest risk when there is a turbulent jet of blood:
• VSD
• Coarctation of the aorta
• PDA
• Insertion of prosthetic materials

58. Strep Viridans:
• Principal cause in
children with congenital
heart conditions without
previous history of
surgery.
Staph Aureus and CONS:
• Especially following
cardiac surgery and in
the presence of
prosthetic cardiac and
endovascular materials.
Candida:
• Especially in premature
infants with central
venous catheter / on
parenteral nutrition

59. Pathogenesis
Turbulent blood
flow
Endothelial damage
Lesion can be infected
Vegetation, usually occurring on
valve leaflet
-Immune complex formation
-mobilization of vegetation
-destruction of infected valve
Presence of transient
bacteremia

60. Clinical Signs
• Fever
• Anemia
• Splinter hemorrhages
• Clubbing (late)
• Necrotic skin lesions
• Changing cardiac signs
• Splenomegaly
• Retinal infarcts
• Hematuria (microscopic)

62. Pre-existing risk factors
•Congenital heart disease; whether unrepaired or repaired
•Prosthetic heart valves and intracardiac devices
•Previous history of infective endocarditis
•Native valvular heart diseases such as rheumatic heart disease
•Presence of chronic intravenous access such as indwelling
central venous catheters, chemoports and haemodialysis
catheters
•Immunocompromised patients

63. Investigation
•The diagnosis of IE requires combination of clinical features,
microbiological findings and identification of endocardial
involvements and extracardiac complications by imaging
tools.

64. Blood cultures
•Remains the cornerstone of diagnosis of IE
•At least 3 sets (to increase yield and reduce false positive rate by
skin contaminants)
•There is no necessity to wait for spikes of fever (due to continuous
nature of bacteraemia)
•Should be taken at 30 mins intervals between samples
•Should be obtained from peripheral veins and not from central
venous catheter using aseptic technique
•Should be taken before commencement of antibiotics
• Each set should include 1 aerobic and 1 anaerobic bottle with
minimal of 3 ml of blood

65. Echocardiography
• Findings suggestive of IE include vegetation, abscess,
pseudoaneurysm, new dehiscence of prosthetic valve, fistula, valve
leaflet perforation and aneurysm
• If clinical suspicion of IE remains high despite an initial negative
TTE, a repeat TTE or transoesophageal echocardiogram (TEE) is
recommended within a week
• TEE is advisable in cases with prosthetic valves, prosthetic cardiac
material and those with poor TTE acoustic window
• TTE is recommended at completion of antibiotic treatment to
assess treatment response

67. Modified Duke Criteria

70. Management
General principles:
•Use bactericidal instead of bacteriostatic agents
• Initial high dose parenteral route to achieve high bactericidal
effects
• Adequate duration to ensure complete eradication (4 to 6 weeks)

78. Surgical Intervention
Surgical intervention is indicated in the following cases:
• Heart failure: severe valvular regurgitation, obstruction or fistula
causing refractory pulmonary oedema, cardiogenic shock or severe
heart failure symptoms.
• Uncontrolled infection: infection caused by fungi, local extension of
infection (abscess, pseudoaneurysm, fistula, enlarging vegetation),
persistent positive blood cultures despite appropriate antibiotic therapy
and prosthetic valve endocarditis caused by staphylococci or non-
HACEK gram-negative bacteria.
• Prevention of embolism: Left-sided vegetation > 10 mm after 1 or
more embolic episode, very large vegetation > 30 mm.

79. Prophylaxis
The most important factor in prophylaxis against
endocarditis is good dental hygiene that should
be strongly encouraged in all children with
congenital heart disease along with avoidance of
body piercing and tattoos.
• dental treatment, however, trivial
• surgery which is likely to be associated with
bacteraemia.

80. KAWASAKI DISEASE

81. INTRODUCTION
• Kawasaki disease is a systemic vasculitis that predominantly affects
the medium size arteries ( most common: coronary artery)
• IMPORTANT DIAGNOSIS to make because aneurysms of the
coronary arteries are a potentially devastating complication
• Age: 6 months- 4 years ( peaks at the 1st year)
• Young infants are more severely affected than older children (
tend to not have all the cardinal features present)
• Specific cause is unknown, likely to be the result of immune
hyperactivity to a variety of triggers in a genetically susceptible
host

82. INTRODUCTION
• NO DIAGNOSTIC TEST, IT IS A CLINCAL DIAGNOSIS

83. PATHOGENESIS
• 3 phase process
Neutrophilic necrotizing arteritis
Begins in the endothelium and moves through the
coronary wall
Saccular aneurysms may form from this arteritis
( first 2 weeks)
Subacute/ chronic vasculitis driven by lymphocytes,
plasma cells and eosinophils
( lasts for weeks to years)
Smooth muscle cell myofibroblasts develops which causes
progressive stenosis.
Thrombi may form in the lumen and obstruct blood flow
stenosi
s

84. CLINICAL FEATURES
Bilateral, non-purulent
conjunctivitis
(beginning
periungually)
(usually truncal)
Unremitting and unresponsive
to antibiotics

85. CLINICAL FEATURES
Clinical phases
Acute febrile
phase
Characterized by fever and the other acute signs of illness and usually
lasts 1-2 weeks
Subacute phase Associated with desquamation, thrombocytosis, the development of CAA,
highest risk of sudden death in whom aneurysms have developed, lasts
about 3 weeks
Convalescent
phase
When all clinical signs have disappeared and continues until ESR returns
to normal, about 6-8 weeks after the onset of illness

86. CLINICAL FEATURES
Bilateral, non-purulent
conjunctivitis
Strawberry tongue
Polymorphous rash- can be
macular, maculopapular,
but NEVER vesicular

87. CLINICAL FEATURES
Periungual
desquamation

88. CLINICAL FEATURES
Diagnosis
Kawasaki disease Fever + 4/5 of the above symptoms
Incomplete Kawasaki disease Prolonged unexplained fever in an infant/ child
< 4 of the symptoms
Atypical Kawasaki disease Present atypically: eg: with renal impairment

89. CLINICAL FEATURES
• Other helpful signs are:
- Irritability/ altered mental state, aseptic
meningitis
- Erythema/ induration at the BCG site
- Perianal excoriation
- Transient arthritis
- Diarrhoea, vomiting, abdominal pain
- Hepatosplenomegaly
- Hydrops of gallbladder
- Sterile pyuria

90. COMPLICATIONS
• Coronary vasculitis ( usually within 2 weeks of illness, affecting
up to 25% of untreated children)
• MI
• Pericarditis
• Myocarditis
• Endocarditis
• Heart failure
• Arrhythmia

91. INVESTIGATIONS
• Lab investigations
- FBC ( anemia, leucocytosis, thrombocytosis )
- Albumin levels < 3g/dL
- Raised alanine aminotransferase
- Urine > 10 wbc/hpf (normal= 0-5)

92. INVESTIGATIONS
• Imaging
- Chest X-ray ( coronary artery calcification)
- ECG ( look for MI, pericarditis, arrhythmia)
- Echocardiogram ( look for heart failure, coronary artery
dilatation/ aneurysms)- do in the acute phase and repeat 6-8
weeks later
Echo indications ( with prolonged fever plus)
2 other criteria
Subsequent periungual desquamation
2 criteria + thrombocytosis
Rash without any other explaination

93. MANAGEMENT
PRIMARY TREATMENT
- IV immunoglobulin 2g/kg infusion
over 10-12 hours ( if therapy is
started < 10 days of onset of the
disease it is effective in preventing
coronary vascular damage)
- Oral aspirin ( anti-inflammatory
dose ) 30-50mg/kg/day in 3 divided
doses till day 14 of illness or until
patient is afebrile for 2-3 days
NOT RESPONDING TO PRIMARY
TREATMENT
- Repeat IVIG 2g/kg over 10-12 hours
Persistent fever > 36
hours after completion of
initial dose of IVIG

94. MANAGEMENT
MAINTENANCE
- Oral aspirin ( anti platelet dose)
3-5mg/kg daily
- For 6- 8 weeks or until ESR and
platelet count normalizes
- If coronary aneurysm is present,
then continue aspirin until resolved
Delay live vaccination for 11
months as the use of IVIG may
impair efficacy