approach to chd

1. APPROACH TO
CONGENITAL
HEART DISEASE

2. Congenital Heart Defects
Most common birth
defect: more common
than neural tube defects
or cleft lip/palate
8-9/1000 newborns
LVRV
LA
RA

3. Congenital Heart Defects
prognosis in the current
era
95% of various types of CHDs have a lot of morbidity and mortality if left
untreated
If treated in time, the 5 most common CHDs have a pretty good
prognosis

4. Congenital Heart Defects
(CHD)
Common
Contribute significantly to morbidity and mortality
After treatment, high chance of normal life

5. Presenting complaints/signs
Failure to thrive
Exercise intolerence
Easy fatigability
Chest indrawing
Sweating during feeding
Bluish spells
Fever with rigor
Palpitation
Convulsion
Fast breathing
Oedema
Hepatomegaly,
spleenomegaly
Clubbing
Cyanosis
Focal neurological lesion
Other organ defects
Chromosomal anomalies

6. Prevalence
Congenital
Cyanotic: 22%
Acyanotic: 68%
◦ VSD 25%
◦ ASD 6%
◦ PDA 6%
◦ TOF 5%
◦ PS 5%
◦ AS 5%
Acquired
◦ Kawasaki disease
◦ Rheumatic
◦ Tubercular
◦ Collagen
Ceylon Med J 2001 Sep; 46 (3): 96-8; Indian J Pediatr. 2001 Aug;68 (8):757-7
Nelson’s Textbook of pediatrics; 17 ed.

7. Case 1
Mother carrying a baby walks into your clinic
Cough, fever for 3 days.
Not very high fever
Little bit of runny nose
Drinking less milk than usual
Age: “5mo completed”

8. Case 1
Mildly febrile, tachycardic
Clear nasal discharge
Mildly tachypneic; coughing, mild intercostal and subcostal retractions
Lungs: bilateral rhonchi
No murmur;
Liver is palpable ?pushed down

9. Case 1
Does he need some bronchodilator therapy?
Is this a straight forward case of a baby with bronchiolitis?
Probably….

10. Case 1
What is one important fact about the baby that we need to know?

12. Case 2
Mother carrying a baby walks into your consulting chamber.
Fever, cough for 3 days.
Not very high fever
Little bit of runny nose
Drinking less milk than usual
Age: “8months”

13. Case 2
Looks smaller than 8mo
Mildly febrile, tachycardic
Clear nasal discharge;
Mildly tachpneic, coughing, mild intercostal and
subcostal retractions,
Lungs: bilateral rhonchi
No murmur;
Liver is palpable ?pushed down

15. Case 2
Does this baby need bronchodilator therapy?
Is this a straight forward case of a baby with URI?
What is different about this baby?

16. Case 2
Check the weight of the baby
Compare with the birth weight
Birth weight should atleast double by 5months
Wt-4.5kg; Birth weight: 2.5kg

17. Case 2: 8 mo old with cough
cold and malnourishment
Take feeding history:
◦ Is there a suck-rest-cycle with breast feeds?
◦ Is there sweating with feeds?
◦ Does the mother resort to wati-spoon feeds as the baby is too ‘weak’ to breast
feed?
Take past medical history
◦ Does the baby have frequent and prolonged cough/cold?
◦ Is there any hospitalization for pneumonia?
ASK mother if she has noted any cyanosis of the nail beds, lips, palms or
planter surface

18. Case 2: 8 mo old with cough
cold and malnourishment
Examination
Compare current weight with birth weight
Assess perfusion of patient
Any wheezes in the lungs?
Listen for any horse galloping in the heart
Assess the hepatomegaly
Feel femoral pulses

19. Case 2
Suck-rest-suck cycle (S-R-S cycle)
pneumonia in past
Hyperdyamic precordium
Murmur heard
Liver 3 fingers below costal margin

20. How would you approach this case?

22. Echo: large Ventricular Septal
Defect (VSD);
When would you want to
refer to a pediatric heart
center?
Objective assessment of
the heart
Follow up evaluation

23. Ventricular Defect
Small VSD
◦ Asymptomatic
◦ A loud, harsh, or blowing holosystolic
murmur.
Large VSD
◦ dyspnea, feeding difficulties, poor
growth, profuse perspiration, recurrent
pulmonary infections, and cardiac
failure in early infancy.
80%

24. Ventricular Septal defects
Small Defects
◦ 30–50% of small defects close spontaneously, mostly - 1st 2 yr
of life
◦ Muscular VSDs - up to 80% - spontaneous closure
◦ Membranous VSDs - up to 35% - spontaneous closure
Infants with large defects have repeated episodes of respiratory
infection and heart failure despite optimal medical management
Intervention - prior to development of an irreversible increase in
pulmonary vascular resistance

25. Pulmonary hypertensive disease is a vulture waiting
to pounce on its prey- a child waiting to become 10kg

26. Case 2: Take Home Points
1. Think of CHDs like large VSD, PDA in an infant
who has inadequate weight gain and
pneumonia
2. Inadequate weight gain is a powerful indicator
of a CHD
3. A large VSD MAY NOT produce a murmur
4. A large VSD needs surgery early after diagnosis
5. There is no such thing like the ’10kg barrier’ for
the surgery

27. Take Home Points cont’d
6. VSDs come in all shapes and sizes and locations and prognoses.
Someone familiar with pediatric cardiology can determine what is
the best management plan for each kind
7. “‘wait till 5 years for spontaneous closure. If not closed then we
will get it operated”

28. 10kg
10kg
10kg
10kg
10kg
10kg

29. Case 3Evening OPD: rush hour (8-9pm); 20 pts in waiting area
6yr old boy
CC: cough, mild fever: 2days
Clear lungs
Grade 3/6 systolic murmur heard best at Left upper sternal border

30. Case 3:
Is this an innocent murmur?
◦ 6/10 school going kids have a murmur
Should we get an echocardiogram?

31. X-Ray
Enlargement of the right ventricle
Enlargement of atrium
Large pulmonary artery
increased pulmonary vascularity
What was the Echo study?

32. Perform Echo on all murmurs
Do not discard every murmur in a healthy looking child
as a functional murmur
American College of Cardiology (ACC) guidelines: all
murmurs do not need echocardiogram
America = India

33. Indian guideline: Echo all murmurs
?
Do you have time to examine the child
completely in the middle of your busy
OPD?
Very poor follow-up: will you ever see
this patient again?
Echo here does not cost $800
Echo is now an extension of the
stethoscope

34. Do not Echo if
The murmur is soft, grade 1-2/6
Systolic only
Heart sounds are normally split
There is no cyanosis
No hepatomegaly
Peripheral pulses- the radials and the femorals are
normal
The entire history of the patient is normal
12 lead ECG is normal

35. Case 3:
Echocardiogram: large Atrial Septal Defect
(ASD) with right heart enlargement

36. Atrial Septal Defects:
secundum
Most common form of ASD
(fossa ovalis)
In large defects, a
considerable shunt of
oxygenated blood flows from
the left to the right atrium.
Mostly asymptomatic
The 2nd heart sound is
characteristically widely split
and fixed.
Secundum

37. Atrial Septal Defects:primumSituated in the lower portion of the atrial
septum and overlies the mitral and tricuspid
valves
P/O - cleft in the anterior leaflet of the mitral
valve is also noted
C/F similar to that of an ostium secundum ASD

38. Atrial Septal Defects
Secundum ASDs are well tolerated during childhood
Antibiotic prophylaxis for isolated secundum ASDs is not recommended
Surgery or transcatheter device closure is advised for all symptomatic
patients
Ostium primum defects are approached surgically

39. Case 3:
How would you approach this CHD?
Standard of Care for ASD
◦ Offer device closure of ASD if the ASD is found suitable for device closure
after echo assessment
Long term outcome: excellent

40. ASD
• As child grows in size, the hole doesn’t grow in size
•Child doesnot need to be on anti platelet agent beyond first 6
months after device placement

41. Case 3: take home points
A soft systolic murmur may be the only clue to a CHD
Echocardiogram is the gold standard to diagnose CHD
Most secundum ASDs can be device closed

42. Case - 4
Neonatal echo (day 1) done in an infant with PPHN: large ductus
Neonate discharged eventually
2mo follow up: Doing well, normal physical exam: no murmur
Follow up echo ordered: tiny PDA
????

43. Patent Ductus Arteriosus
Small defect - no symptoms
Large defect:
◦ Wide pulse pressure
◦ Enlarged heart
◦ Thrill in L second IS
◦ Continuous murmur
◦ X-ray: prominent pulmonary artery with increased vascular
markings
If murmur is heard – intervention indicated

44. Silent ductuses do not need SBE prophylaxis
Silent ductuses do not need closure

45. CASE 5

46. Case 5
4 day old neonate
Noted to be cyanosed on routine examination
Sat 65-70%
On enquiry: feeding poorly
echo

47. Case 5
D transposition of the great
arteries with an intact
ventricular septum with a small
PDA with a small PFO

48. Transposition of the great
arteries
Parental counseling
◦ Surgery in first 2 weeks of life
◦ Overall good quality of life

49. Arterial Switch for dTGA
Survival:
Early mortality rates vary from 2% or less in pts with normal coronary
artery anatomy to 10%
81-90% survival at 15 yrs post op
Freedom from re operation: 88% at 5yrs, 80% at 15yrs
Most common reason for reoperation: pulm outflow tract obstruction

50. DTGA with de-saturations
PGE started
No improvement in saturation
Sat 60-65%

51. Why won't sats improve in a
dTGA after PGE?
DTGA implies parallel circulations:
Pulm Veins-LA-LV-Pulm artery-Pulm Veins
Venae cavae-RA-RV-Ao-Body-Venae cavae
Blood has to mix to survive
Liquid mixes best (to and fro) when the pressure difference
between the chambers is least

52. Balloon Atrial Septostomy
Balloon tears open the atrial
septum
Promotes mixing of the pulm
venous and systemic venous
blood
Sats improve

53. D TGA
Emergency procedure
Can be done bedside: echo guided
Stand by cath lab preferrable
Post BAS: saturation 80%

54. Case 5: DTGA
Arterial switch procedure on day 10 of life
Discharged home on day25.
Needs life long follow up

55. Case 5: Take Home Messages
DTGA has a good prognosis post surgery
BAS is an emergency procedure that will improve saturations when PFO is
restrictive
It should preferrable be performed prior to transport (long haul)
PGE may not help much
I Hope
everyone is
listening!

56. CYANOSIS
PRESENCE OF > 4g/dl of Deoxy Hb which correlates with 80-85% Spo2.
Can be missed when mild, in dark races and anemia due to decreased deoxy
Hb
Can be misdiagnosed as CCHD in acrocyanosis, non cardiac causes of cyanosis
like pulmonary causes, CNS causes and Cyanosis with normal Po2.

57. CLUES BASED ON ONSET
1st week > 1 week
D TGA TOF
Tricuspid atresia TGA
Pulmonary Atresia Admixture lesions
TAPVC
Ebstein SV
Critical PS DORV
Truncus

58. Case 6
8 months old child brought into your OPD
Cyanosis
H/o Cyanotic Spell
No cardiac evaluation done

59. Case 6
What does one do?
◦ Chest radiograph
◦ give vitamin and iron supplements
◦ Refer for echocardiography

61. Case 6
Echocardiogram - TOF

62. Case 6
Times are changing…
Suitable age for corrective surgeries coming down
Good chance of good quality of life post surgery

63. Case 7
Attending a delivery in the local nursing home: 28yr
old G1P0 mother with no antenatal issues.
Prolonged labor; C section advised. Meconium
stained amniotic fluid.
Baby’s APGARs 8,8.
Evening rounds: baby appears to be breathing a little
fast. You count 70 RR. His lips don’t appear pink….
They appear a little bluish

64. You order chest XR and
pulse oximetry:
70% on room air

65. CCHD in Newborns:
Clues based on presentation
Cyanosis
No Resp Distress
Cyanosis
+
Resp Distress
Shock
Differential
cyanosis
TGA
DDPC
TAPVC
obstructed
DDSC

66. Neonate with ductal dependant
circulation
Pulmonary circulation is duct dependant:
◦ Pulmonary atresia
◦ Critical Pulmonary Stenosis
Systemic circulation is duct dependant:
◦ Critical aortic stenosis
◦ Hypoplastic left heart syndrome
Duct is the site of mixing
◦ Transposition of the great arteries

67. Congenital Heart Defect
1. Give oxygen
2. Call for
echocardiogram
3. Prostaglandin
infusion: to give or not
to give…

68. Always given as continuous IV infusion.
Start at 0.05-0.1μg/kg/min, can be reduced to 0.005 - 0.01µg/kg/min
once duct is opened( ^ SaO2)
Trade name: Alpostin/Prostin
One vial will last 2-3 days for a 3Kg baby

69. Start PGE1 infusion
◦ 1ml Vial of 500mcg/ml
◦ Reconstitute in 50ml 10% D
◦ 0.3xwt= _ml/hr drip rate for dose of 0.05mcg/kg/min
◦ 0.6xwt=_ml/hr drip rate for a dose of 0.1mcg/kg/min
◦ For this 2.5kg baby, start 0.8ml/hr infusion.

70. PGE1
500mcg/ 1amp
Cost Rs 6000
When is the echo being done?
Do you have ventilator back up?

71. Why 75-85% is ideal for this
neonate…

72. Pulmonary blood flow
Too little Just right Too much

73. Decreased Pulmonary Blood Flow
Systemic
Venous
Blood
Pulmonary
Venous
Blood
RA LA
RV LV

74. Increased Pulmonary Blood Flow
RA LA
RV LV
Systemic
Venous
Blood
Pulmonary
Venous
Blood

75. “Ideal” Saturation
Arterial Venous
100% 60%
Increased pulmonary blood flow
Decreased pulmonary blood flow
“Ideal”
80%

76. CASE 8

77. Case 8
1 day old neonate
Had respiratory distress soon after planned C section (rpt)
APGAR 8,8
O/E: no dysmorph; CR =3sec; cyanosed; sat 60%; hr 160; lungs clear,
abd soft, pulses weak and equal
CXR

78. Chest XR

79. Case 8: 1hour neonate: cyanosed and
decreased perfusion
? Congenital pneumonia
PPHN
Intubate
Oxygen
No improvement
Dopamine
ABG: metabolic acidosis and hypoxia; PO2 50mm Hg

80. Case 8 cont'd: Total anomalous pulmonary venous
connection to the portal veins; obstructed

81. TAPVC- infradiaphragmatic
Parental Counseling
Immediate surgery
Usually one time surgery
Quality of life good

82. Obstr TAPVR: mgmt
PGE not started
Given lasix
Oxygen stopped
Transferred to peds heart center
Surgery the same day
Inhaled Nitric Oxide post operatively
Dicharged 3 weeks later

83. Case :8- Take Home Messages
TAPVR of obstructed type has neonatal presentation
CXR mimics PPHN: Mec asp
Only treatment is surgery
PGE if started may actually cause clinical deterioration.
However, PGE remains neonatologist's best friend
Long term outcome is good

84. Case 9
3 day old neonate
Noted to be less active, less tone and feeding poorly since discharge
History: born FTNVD, no meconium

85. 3 day old with decreased feeding
On examination
Cool to touch, CR 3sec
Pulses feeble
limp
HR 190
No murmur
Abd normal

86. Case 9 cont'd
Sat- not picking up
Baby looks blue
IV line,
Oxygen
Blood Ix
IV antibx
Sat 85%

87. Case 9 cont'd
CXR: Cardiac size overshadowed by thymus, normal lung fields, no
patch
Baby intubated
Sats not improving beyond 90%
IV fluids given

88. Case 9: 6 hours later
Perfusion improved to some extent with fluids
Dopamine begun at 5
Bicarb given as ABG shows marked acidosis and base deficit
? CHD
Echocardiogram ordered

89. 3 day old with circ collapse
Next day portable echo done
Critical aortic stenosis with very poor flow across it. Descending
aorta partly perfused by duct shunting right to left. LV function
moderately reduced

90. Critical Aortic Stenosis
Parent counseling
Urgent intervention required
Gratifying results but long term
follow up required

91. Aortic Stenosis: steps in mgmt
PGE1 infusion started
Ideal sats: we have no control.... will stay 85-90%
ALT, AST, BUN, Cr, (head ultrasound)
Monitoring of perfusion, urine output

92. Duct dependant Aortic Stenosis: mgmt
Transport team takes pt on PGE
drip
Emergency balloon valvuloplasty
performed the same day
PGE drip discontinued in the
cath lab

93. A sad story
A 6 month boy was examined by a physician and
was suspected to have a VSD.
at that age had frequent URIs, he was a skinny child
too
He was placed on lasix, aldactone and digoxin and
asked to follow up 6monthly as it was felt that
holes in the heart may close on their own and also
that he was too small to be operated at a mere 5kg
weight.

94. He became 1 yr old
An echo showed the VSD to be as big,
He wasn’t yet ‘10kg’
Dose of lasix and digoxin was optimized
He was asked to follow up in 6mo

95. Sad story continued…
His grandmother assumed he was doing well now:
Not falling ill as often, no hospital admission since the
one when he was 8mo
He seemed to be putting on weight too
She didn’t think visiting the doctor was necessary

96. Sad story continued
He turned 2yrs old…
He was really really
naughty; very active
The family forgot that
there ever was a hole in
the heart

97. Around the time he
turned 3, his mother
noticed that he
frequently took rests
between playing
His nails looked blue and
parrot beak like
She feared for her son’s
life…

98. VSD: Eisenmenger
A large VSD may start showing R to L shunt as early as 1yr
As Pulmonary vascular resistance increases steadily, the patient no
longer has chest infections as he no longer has pulmonary
overcirculation
As the R-L shunt increases, it starts impacting pt’s activity level

99. Irreversible Pulmonary
hypertension…
… Mogambo of Pediatric
Cardiology:
…Cannot be judged by
echocardiography