Critical CHD Vietnam

Critical
Congenital Heart
Disease
in Neonates:
Early detection & Initial Treatment
Sukman T Putra, MD, FACC, FESC
Senior Lecturer, Chairman of Cardiology
Department of Pediatrics, University of
Indonesia,Integrated Cardiovascular Center, Dr.Cipto
Mangunkusumo National General Hospital, Jakarta,
INDONESIA
E-mail : stpfika@dnet.net.id
19th Vietnam Congress of Pediatrics,
HCMC ,27-28 Dec 2008
n o t
r e n are
Chil d
d u l t s
t l e a
lit
2
3
THE REGIONAL HEART CENTER IN
INDONESIA
Population : 230 million
46.000 CHD babies born/year
19.000 islands 2
THE PURPOSE
 Early detection &

Recognition
of CCHD in Neonates
 The initial treatment /
Management by
Prim.Physician
 Timing of referral of the
Neonates with CCHD

HCMC, 27-28 December 2008
5
OUTLINE
 Backgrounds (Facts & Figures)
 Diagnosis, Early detection
&Recognition CCHD in Neonates
 Initial Treatment & Management
Timing of Referral of the Neonates
with CCHD
 Conclusion

6
d Issues of CHD in
u n Developing
gro Countries
ck
Ba
 The magnitude of the problems
Type of CHD at birth and
survival patterns
 CHD as a contributor to IMR
 Resources for CHD treatment
Congenital CardiologyToday 7
Congenital Heart Disease
in the Developing World
-q u a l it y
w h i g h
. v e r y f e c i l i t i e s
t e l y . . . .. s i v e f a
r t u n a p r e h e n
“ Unfo s w i t h c o m
ld r e n w i th
t h e
i o n c h i o f
Institut ake care of e x i s t o u t s id e
to t e a s e
e a r t D is o r l d
e n i t a l H p e d W
Co n g Deve l o
Kumar, K. CongenitalCardiology Today,Vol 3, April 2

DEFINITION
TES OF CHD
“ A gross abnormality of the heart or
intrathoraxic great vessels that is
actually or potentially of functional
significance”
Mitchel et al , Circulation
1971;43: 323-32
19th Vietnam Congress of Pediatrics,HCMC 27-28 Dec.2008 9
CONGENITAL HEART
DISEASE
• The global prevalence of
CHD
4-5/1000 live birth – 12-14 / live
birth
Constant in different geographic &
ethnics backgrounds
• Contributor to IMR
7% of neonatal death : major
congenital malformation
Lancet ( of 10
Facts & Figures of CHD
in Early life
 One in three infants (30%)

with
a potentially life threatening
cardiovascular malformat
left hospital undiagnosed !!
 Prenatal diagnosis improves
post-natal outcome
(TGA,HLHS
Coarctation, etc.) 11
in Early Life
 Routine Neonatal Examination
fails to detect > 50% of CHD in
neonates
 Fails to detect >1/3 by 6 weeks
 Normal findings examination
does not exclude heart disease
 Babies with murmur at neonatal
or 6 weeks should be referred for
cardiac evaluation
Arch Dis Child Fetal Neonatal Ed 12
in Early Life
229
Full-term
Infant
CCHD
Acta Paediatrica 2006; 95:407-13

13
in Early Life
Of 669 infants with life-threatening
cardiovascular malformation :
 55 (8%) had an antenatal diagnosis
 416 (62%) had postnatal diagnosis
before
discharged from hospital
 168 (25%) was diagnosed in living
infant
after discharged
 30 (5%) were diagnosed at autopsy
14
DIAGNOSIS
EARLY DETECTION &
RECOGNITION
Critical Congenital Heart
Disease
in Neonates
15
Disease (CCHD)in Neonates
DEFINITION OF CCHD
Congenital Heart Disease that
are ductal dependent or may
equired surgical or invasive intervention
or resulted in death in the first
30 days of life
Pediatrics,
2008;121:751-757
19th Vietnam Congress of Pediatrics,HCMC 27-28 Dec.2008 16
Early identification of the
infant with serious or life
threatening
heart disease) is essential for the
OPTIMAL OUTCOME
Evaluation should
focus on
3 cardinal signs
 Cyanosis
 Decreased systemic
perfusion
 Tachypnea (due to
excessive
pulmonary blood flow ) 17
Diagnostic TOOLS
of Congenital Heart Disease
• History of illness
• Physical Examination
• Electrocardiogram (ECG)
• Chest X-Ray
• Echocardiogram
• Cardiac Catheterization
HCMC, 27-28 December 2008 18
Chest X-Ray

HCMC, 27-28 Dec 2008
19
Four-Chamber Views
Imaging planes
20
CRITICAL CHD
Birth/ Surgical/
Delive Interventi
ry on
Hemodynamic Instability
21
Unrecognized CCHD
 Profound metabolic acidosis
 Hypoxic-ischemic
encephalopathy,
 Intracranial haemmorhage
 Entrocolitis,
 Cardiac Arrest even Death
(when ductus constricts)
Ho Chi Minh City 27-28 Dec 2008 22
Early Detection of CCHD
(1) Prenatal diagnosis (Fetal

Echo)
(2) Post natal : PE,
ECG,Echo, CXR
3 (Three) Cardinal Sign of

CCHD
 Cyanosis
 Decreased systemic 23
CCHD in Neonates
 Decreased systemic perfusion
1. Coarctation of the Aorta
2. Hypoplastic LH syndrome
3. Cardiomyopathy
 Cyanosis
1. Decreased pulmonary blood flow
2. Norma/increased PBF (TGA)
 Tachypnea (excessive PBF)
Left to right shunt
24
CRITICAL CONGENITAL HEART
DISEASE in Neonates
HEART MURMUR
• Murmur detected : 54% due
to cardiovascular problems
• Hars murmur in neonates:
stenotic lesions (AS, PS)
J Pediatr Child
Health 2001;37:331-36
25
DISEASE in NEONATES
Shock syndrome
Severe clinical state (cyanosis,
weak pulses, hepatomegaly,
oliguria)
Blood pressure : 4 extremities
( Symtomatic: “duct dependent
19th Vietnam Congress
lesions’’ )of Pediatrics, 26
HCMC, 27-28 Dec 2008
in Neonates
Distribution of CHD based on Age at
Diagnosis
0-6 days : Transposition Great
Arteries (19%)
Hypoplastic Left
HSyndrome(14%)
Tetralogy of Fallot (8%)
Coarctation of aorta (7%)
27
in Neonates
Distribution of CHD Based on Age at
DIAGNOSIS
7-13 days : Coarctation of Aorta (16%)
Ventricular Septal Defect
(14%)
HLHS (8%)
TGA (7%)
28
Disease
in Neonates
Distribution of CHD Based on
Age at
Diagnosis
14- 28 days : VSD (16%)
Coarctation of the aorta
(12%)
TGA (7%)
Marion BS at.al :Clinics in Perinatology
Patent 2001
Ductus :
29
TRICUSPID
ATRESIA
•Hypoplastic RV
•R-L shunt atria level
•B-T shunt
and finally FONTAN
30
Hypoplastic Left Heart
Syndrome (HLHS)
• DUCT DEPENDENT
SYSTEMIC
CIRCULATION
• Clinical
manifestations:
Day > 6
•19th
Norwood Operation
Vietnam Congress of Pediatrics,
• Unfavour Prognosis
31
Coarctation of the Aorta
(CoA)
19th Vietnam Congress of Pediatrics, 32

Post Natal & Fetal
Circulation
 19th Vietnam Congress of Pediatrics,HCMC 27-28 Dec.2008
33
CONGENITAL HEART DISEASE
in Neonates
Transitional Circulation soon After
BIRTH
• Increasing pulmonary blood flow (20 x
of PBF in fetal circulation)
• Significant changes of central
circulation to be a “serial circulation” (
closing of ducts, foramen ovale).
• Increased ventricular output for
respiratory efforts and thermoregulati
34
Interventional Cardiology
Procedures in Neonates
with CCHD
19th Vietnam Congress of Pediatrics
City 27-28 Dec 2008
Sirkulasi Janin (Fetal
Circulation)
36
37
RV-graphy before Ballooning
BALLOONING PULMONARY
VALVE
RV GRAPHY AFTER BALLONING
INITIAL TREATMENT
OF CRITICAL CONGENITAL
HEART DISEASE
19th Vietnam National Pediatric Congress,
Ho Chi Minh City 27-28 Dec 2008
41
Patent Ductus
Arteriosus
Echocardiogram of 50 normal
Neonates :
 42% closed in 24 hours
 Undetected at the age of 96 hrs
J Pediatr
1981;98:443-48
TIMING OF REFERRAL
CRITICAL CONGENITAL
HEART DISEASE
Ho Chi Minh City 27-28 Dec 2008
43
URGENT REFERRAL of
CCHD
Potentially lethal CHD (15%

CHD)
 Cyanosis
 Shock
 Pulmonary edema
44
45
PALLIATIVE
PROCEDURES
for increasing
Pulmonary Circulation
19th Vietnam Congress Pediatrics,

HCMC, 27-28 Dec 2008
46
HLHS
19th Vietnam Congress Pediatrics,

HCMC, 27-28 Dec 2008
47
DISEASE in NEONATES
INITIAL TREATMENT
• To prevent deterioration
• Should follow the general
approach guidelines for
critically ill neonates.
• Should be initiated as soon as
the diagnosis established. 48
DISEASE in Neonates
INITIAL TREATMENT
Basic advanced life support
• Maintaining the ductus &
stable airway (PGE1 or
Stenting PDA)
• Blood gas & monitoring blood
pressure 49
DISEASE in Neonates
TREATMENT
Primary treatment
• CONSERVATIVES (O2,mecahnical
ventilation )
• PALLIATIVES : Ballooon atrial
septostomy in TGA
50
DISEASE in NEONATES
TREATMENT
The second step
• SURGERY (BT shunt / Repair)
• PDA Stenting ( before surgery)
an alternative to surgical shunt
Alwi at.al JACC
2004;44:438-45
51
DISEASE in Neonates
• Prostaglandin E1
Administered for duct
dependent lesions: 10-20
nanogram/min.
Side effects: apnea 10-15%
cases Additional : diuretic &
inotropic
• Effective : age less than 2 52
DISEASE in Neonates
• Oxygen : consider the goal of
therapy and adverse effects
(maintain O2 sat & PaO2)
• Fluids : fluid status & urine
output. Day 1 & 2 same fluid and
glucose requirement as normal
(depend on the type of defects
for the next days)
53
Case Illustration (Real Case)
Fullterm baby :
• Born by S,C , weight 3200 gram, AS 9/10
12 hours after birth : mild cyanosis, tachypnoe
• PE: central cyanosis, RR 48x/m,
HR 144x/m, no murmur, normal pulses
• ECG : RAD, no hypertrophy, normal
• CXR : normal pulm vasc.”egg on side”
• Blood Gas Analysis : pH : 7.35 , PaO2 : 66 mmHg
O2 Sat 79%
Consulted to Pediatric Cardiologist
ECHO : Transposition Great Arteries, PDA, small ASD
Surgery : 3 weeks of age
54
“ Egg on Side”
appearance
55
TGA
56
“Booth Shape” appearance
57
DISEAS in NEONATES
TIMING OF REFERRAL
“ A professional decision
making”
• Symtomatic neonates
• Asymtomatic neonates
58
DISEAS in NEONATES
TIMING OF REFERRAL
“ A professional decision
making”
• Asymtomatic neonates
59
in NEONATES
TIMING OF REFERRAL
• Should be a professional decision
Start initial treatment
immediately.
PGE1 started before referral
• As soon as the baby stable
60
DISEASE in NEONATES
Hypercyanotic Spells
Rare in the newborn period,begin
With irritability & crying.
• Increased cyanosis
• Placing knee-chest (>> syst.vasc
resist)
• Morphine 0.1 mg/kg i.v
• If unresponsive: start vasopressor
(phenyleprine) to decrease R-L
61
DISEASE in NEONATES
CONCLUSION
• Early detection of Critical CHD in
neonates is very important for the
optimal outcome.
• Initial evaluation of neonates
suspected CHD should include :
history, physical exam, ECG, CXR,
echocardiogram and Hyperoxic
test 19th Vietnam Congress of Pediatrics,
62
HCMC ,27-28 Dec 2008
DISEASE in NEONATES
CONCLUSION
• More than 50% of CHD fails to
detect on routine neonatal
examination.
• Hyperoxic test is important to
differentiate cyanosis due to
cardiac and non cardiac origin.
63
DISEASE in NEONATES
CONCLUSION
• PGE1 should be started immediately in
neonates with critical CHD
• Initial treatment consist of: PGE1, fluids
and medication
• The timing of referral should be a
“professional decision” which much
depend on the diagnostic and initial
treatment
• Pediatricians : should be able to detect
early signs and symptom of Critical CHD
in neonates.
Ho Chi Minh City 27-28 Dec 2008 64
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Critical

 Early detection &

Neonates with CCHD

19th Vietnam Congress of Pediatrics,

Kumar, K. CongenitalCardiology Today,Vol 3, April 2

 One in three infants (30%)

Acta Paediatrica 2006; 95:407-13

19th Vietnam Congress of Pediatrics,

(1) Prenatal diagnosis (Fetal

3 (Three) Cardinal Sign of

19th Vietnam Congress of Pediatrics, 32

 19th Vietnam Congress of Pediatrics,HCMC 27-28 Dec.2008

Potentially lethal CHD (15%

19th Vietnam Congress Pediatrics,

19th Vietnam Congress Pediatrics,

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