Critical congenital heart disease

1. Dr.Parashuram Waddar
Pediatrician
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2. References
 Eric C. Eichenwald, Anne R.Hansen, Camilia R. Martin,
Ann R. Stark.South Asian 8th Edition of Cloherty and
Stark’s Manual of Neonatal Care.wolters kluwer.2017.
 Josheph K. Perloff, Ariane J. Marelli, Perloff’s clinical
recognition of congenital heart disease.6th edition.Elsevier
 Kleigman, St Geme, Blum, Shah, Tasker, Wilson.Nelson
textbook of pediatrics.21st international edition
 Ramesh Agarwal, Ashok Deorari, Vinod Paul, M Jeeva
Sankar, Anu Sachdeva.AIIMS protocols I neonatalogy
volume 1. 2nd edition.Noble.2019
 https://indianpediatrics.net/dec2018/dec-1075-1082.htm
 https://www.rch.org.au/cardiology/heart_defects
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3. “Neonates who present with shock /
collapse/ heart failure in the first few
weeks of life have duct-dependent blood
flow until proved otherwise”.
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4. Specific learning objectives
By the end of presentation, we will be able to
 Present scenario
 Understand anatomy and physiology of PDA.
 Define of duct dependent lesion(DDL).
 Classify and Pathophysiology of these lesions.
 Understand presentation of DDL
 Diagnose and differentiate DDL
 Manage DDL and referral.
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5. Present scenario
 Incidence rate of CHD is 8-9/1000 live births, nearly 1.8-
2lacs children are born with CHD each year in India.
 Of these, nearly 60,000 to 90,000 suffer from critical CHD
requiring early intervention.
 Approximately 10% of present infant mortality in India may
be accounted for by CHD alone.
 Lack of awareness & delay in diagnosis is biggest obstacle.
 Frontline health workers & primary caregivers are not
sensitized to the problem of CHD.
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https://indianpediatrics.net/dec2018/dec-1075-1082.htm

6. Present cont..
 78.9- 81.4% Institutional deliveries.
 20% of births in India occur at home, and the infant is
likely to die before the critical, ductus-dependent CHD
is diagnosed.
 Fortunately, the rate of hospital deliveries have
increased due to several incentivized schemes by the
Govt of India.
 Ductus-dependent CHD may still escape detection as
babies are often discharged earlier.
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https://indianpediatrics.net/dec2018/dec-1075-1082.htm

7. Present cont..
 Predischarge screening of newborns by pulse oximetry,
which may pick up these CHDs, is often not practiced,
especially in rural & semi-urban centers.
 Lack of follow up care.
 Delay in referral results in poor outcomes as co-
morbidities may have already set in.
 The risks of developing hypothermia and hypoglycemia
during long, unsupervised transport further adds to the
already serious condition of the infants with CHD.
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https://indianpediatrics.net/dec2018/dec-1075-1082.htm

8. Patent ductus arteriosus
 Short circuit channel between the
pulmonary artery and the aorta in the fetus, which
bypasses the lungs to distribute oxygen received
through the placenta from the mother’s blood.
 It normally closes once the baby is born and the
lungs inflate, separating the pulmonary and
systemic circulations, thus converting parallel
circulation into series.
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Josheph K. Perloff, Ariane J. Marelli, Perloff’s clinical recognition of congenital heart
disease.6th edition.Elsevier

9. • Functional closure of the ductus arteriosus
occurs within 10-15 hours after birth in
healthy infants born at term.
• This occurs by abrupt contraction of the medial
smooth muscular wall of the ductus arteriosus.
• Multiple factors are responsible for the
closure of ductus arteriosus. Ex- Po2, GA,
PGE2 , etc.
Patent cont…
Josheph K. Perloff, Ariane J. Marelli, Perloff’s clinical recognition of congenital heart
disease.6th edition.Elsevier
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10. Patent cont…
 Increase in the partial pressure of oxygen (PO2) from
25mmHg(in utero) to 50mmHg after lung expansion is
the strongest stimulus.
 Decrease in PGE2.
 Anatomic closure completes by end 2-3 weeks.
 Starting of ductus closure is the cause for deterioration
in these lesions.
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Josheph K. Perloff, Ariane J. Marelli, Perloff’s clinical recognition of congenital
heart disease.6th edition.Elsevier

11. Definition
 These are critical congenital heart disease (cCHD), in
which the permeability of the ductus arteriosus is
mandatory in order to maintain systemic and
pulmonary perfusion after birth.
 These are most important d/d for newborns who are
going to collapse in and around day3.
 Critical congenital heart disease (cCHD) is the most
common reason for acute cardiac failure in the neonatal
period.
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12. Introduction
 Incidence- 25% of all CHDs, nearly 25% mortality in
first year life.
 The distribution of cCHD differs from the distribution
of CHDs in general.
 Left sided heart obstructions have the largest share
with 30–40%, followed by complete transposition of
the great arteries (approx. 30%) and right sided heart
obstructions (20–30%).
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https://indianpediatrics.net/dec2018/dec-1075-1082.htm

13. Classification
These duct dependent lesions are classified into 3
categories
1) Left sided obstructive lesions
(Duct dependent systemic circulation)
2) Right sided obstructive lesions
(Duct dependent pulmonary circulation)
3) Transposition physiology
(Duct dependent systemic and pulmonary
circulation)
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Eric C. Eichenwald, Anne R.Hansen, Camilia R. Martin, Ann R. Stark.South Asian 8th Edition of
Cloherty and Stark’s Manual of Neonatal Care.wolters kluwer.2017

14. Duct dependent
lesions
Duct dependent
systemic
circulation
1) HLHS
2) Interrupted Aortic
arch
3) Severe Coarctation
of Aorta
4) Critical Aortic
stenosis
5) Shone complex
Transposition
physiology
Transposition
of great
arteries
Duct dependent
pulmonary
circulation
1) Severe pulmonary
stenosis
2) PA with intact IVS
3) Tricuspid atresia
4) Severe Ebstein
Anamoly
5)Severe TOF
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14
Eric C. Eichenwald, Anne R.Hansen, Camilia R. Martin, Ann R. Stark.South Asian
8th Edition of Cloherty and Stark’s Manual of Neonatal Care.wolters kluwer.2017

15. Hypoplastic left heart syndrome
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Ductus arteriosus
https://www.rch.org.au/cardiology/heart_defects

16. Interrupted aortic arch
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https://www.rch.org.au/cardiology/heart_defects

17. Severe coarctation of aorta
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https://www.rch.org.au/cardiology/heart_defects

18. Critical Aortic stenosis
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https://www.rch.org.au/cardiology/heart_defects

19. Transposition of great arteries
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https://www.rch.org.au/cardiology/heart_defects

20. Severe pulmonary stenosis
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https://www.rch.org.au/cardiology/heart_defects

21. Pulmonary atresia with intact IVS
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https://www.rch.org.au/cardiology/heart_defects

22. Tricuspid atresia
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https://www.rch.org.au/cardiology/heart_defects

23. Severe Ebstein anamoly
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23
Kleigman, St Geme, Blum, Shah, Tasker, Wilson.Nelson textbook of pediatrics.21st
international edition

24. Severe Tetrology of Fallot
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https://www.rch.org.au/cardiology/heart_defects

25. Diagnostic gap
 Newborns with duct dependent lesions are going to
present to ED between birth to 1st week of life, usually
with nonspecific symptoms.
 Sn of the clinical examination in the first days of life
for detection of cCHD is <50%.
 Possible symptom-free interval is due to the delayed
change from fetal to neonatal circulatory physiology.
 The sensitivity of prenatal diagnostics for critical heart
defects is reported to be up to a maximum of 51% .
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26. Diagno cont..
 Fetal CVS physiology with a R -> L shunting through
foramen ovale (FO) and ductus arteriosus (DA) with a
high Rp and low systemic vascular resistance (Rs) allows
to a large extent a normal fetal development.
 Umbilical clamp placement terminates the placental
circulation.
 This transition from the fetal parallel to the adult serial
circulation might lead to a life-threatening condition in
the presence of critical heart defects.
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27. Diagno cont..
 Open DA and FO can lead to inconspicuous clinical
findings in newborns with critical CHD. This period is
therefore also referred to as “diagnostic gap”.
 Pulse oximetry screening is a proposed method by
which this postnatal “diagnostic gap” should be
reduced.
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28. Presentation of duct depedent
SBF lesion
 The symptoms range from signs of acute cardiac
failure up to the complete picture of a cardiogenic
shock.
 Tachypnea is the early and therefore leading symptom,
followed by tachycardia, prolonged CRT, hypotension,
pallor and profound shock.
 Usually referred due to acute cardiogenic shock or any
other degree of cardio-vascular failure.
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29. Present cont…
 Inaudible murmur is not a criteria for exclusion, but
disappearance of previously audible murmur points for
urgent intervention.
 H/o diagnosed cardiac diseases in ANC and fetal
Doppler.
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30. Present cont…
 Newborns with critical aortic valve stenosis are mostly
symptomatic within the first week of life.
 A critical aortic coarctation mostly within the first 4
weeks with a typical history of a “3-week-old baby
referred, after failure of a sepsis therapy.”
 In a newborn whose condition worsens clinically in the
first days of life, d/d of a cCHD must always be considered
in addition to the suspected diagnosis of sepsis.
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31. Presentation of duct depedent
PBF lesion and TGA
 Incremental cyanosis is the main & common symptom
followed by varying degree of respiratory distress due to
under perfused lungs, not benefited by O2
administration.
 Right heart failure- dyspnea, hepatomegaly, raised JVP
and dependent edema.
 Shock and metabolic acidosis.
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32. Present cont…
“The cause of a cyanotic but vital newborn is a heart
defect until the opposite is proven”.
 Most critical part is to rule out duct dependent lesion in
all cases of cardiovascular collapse in few first weeks of
life.
 Ask relevant history and progression of condition, history
of CHD in siblings or unexplained deaths in past
postnatal periods and lastly Pulse oxymetry.
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33. Diagnosis
 ABC – stabilize newborn
 History and clinical examination
 Presentation coincides with ductal closure.
 GPE- Vitals, Four limb SpO2 + BP, JVP
 Detailed Cardiovascular examination -Single S2,
murmur and crepts
 Hepatomegaly + edema
 Hyperoxia test – for all cases
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34. Pulse oxymetry (Pox)
 Universal screening with Pox and should be a part of
standard neonatal care.
 Measurement of oxygen saturation by pulse oxymetry
provides much easier, noninvasive and reliable
assessment of hypoxemia.
 Broadly SpO2 < 95% represent hypoxemia and at this
cut off it reaches high specificity of 99.9% and a
sensitivity of 75% for detecting cyanotic CHD.
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35. Pulse cont…
 All four limb pulse oxymetry is mandatory before they
are declared free of hypoxemia.
 Preferably POx screening should be done after 24hrs of
age or shortly before discharge if less than 24hrs of age
to decrease false positivity due to respiratory problmes.
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36. SpO2 in right hand & foot after
24hrs
>95% in all limbs
Pre & post duct diff
< 3%
POxS is negative
Critical CHD is
unlikely
Routine
neonatal care
90-94% in either of
the limbs
Pre & post ductal diff
> 3%
POxS is
intermediate
Repeat 3 times 1hr
apart
<90% in either of
limbs
POxS is positive
Critical CHD is
likely
Pediatric cardilogy
consultation
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37. Hyperoxia test
 Most sensitive and specific tool in the initial
evaluation of the all neonate with suspected critical
congenital heart disease, in sites with timely no access
to echocardiography.
 Determine Pao2 while the infant is on room air.
 Give 100% O2 for 10–20 min by mask/hood/ ET
 Obtain an arterial blood gas level while the infant is
breathing 100% oxygen.
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38. Hyper cont..
PaO2 Test result Interpretation
> 250mmHg Passed Eliminates critical structural cyanotic
heart disease
Possible respiratory/ CNS /normal
100-
250mmHg Intermediate
Structural heart disease with complete
intracardiac mixing
Hypoplastic left heart syndrome
< 100mmHg Failed Most likely due to intracardiac right-to-
left shunting and is virtually diagnostic
of cyanotic congenital heart disease in
absence of clear-cut lung disease
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39. Diagnosis cont..
 X ray chest and ECG
 ABG.
 Echocardiography as soon as possible.
 Septic workup- CBC, CRP and blood culture.
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40. Chest x ray
cardiomegaly
Pulmonary
plethora
TGA
Admixture
physiology
Pulmonary
oligemia
Ebstein
anamoly
No cardiomegaly
Normal
PBF
Single
atrium
Decrease
d PBF
TOF
physiology
Pulmonary
venous
hypertension
HLHS
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•Heart position
•Typical shapes of heart
•Heart contour
•abdominal situs
•Pulmonary blood
flow
•Pulmonary venous
hypertension
•Lung parenchyma

41. Echocardiography
 Gold standard investigation.
 To study anatomy of heart disease.
 Physiologic alterations.
 Size of duct, extent of constriction.
 Flow across various shunts and Doppler.
 Guide for further management.
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42. Differential Diagnosis
Cardivascular disease
 Acyanotic CHD with presure overload
 Acyanotic CHD with volume overload
 Duct dependent lesion with decreased systemic/
pulmonary/ both blood flow
 Cardiomyopathies
 Tachy and brady arrythmias
 Mycarditis and hypovolumia.
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43. Differ cont…
Pulmonary lesions (intrapulmonary right-to-left
shunt)
 Primary parenchymal lung disease
 Aspiration syndromes
 RDS
 Pneumonia
 Airway obstruction
Choanal stenosis or atresia
Pierre Robin syndrome
 Tracheal stenosis
 Pulmonary sling
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44. Differ cont…
Extrinsic compression of the lungs
 Pneumothorax
 Pulmonary interstitial or lobar emphysema
Chylothorax or other pleural effusions
Congenital diaphragmatic hernia
Thoracic dystrophies or dysplasia
Hypoventilation
Central nervous system lesions
Neuromuscular diseases
Sedation
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45. Differ cont…
 Sepsis
 Pulmonary arteriovenous malformations
 Persistent pulmonary hypertension
 Polycythemia
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46. Management
Initial resuscitation
 For the neonate who presents with evidence of
decreased cardiac output or shock, initial attention is
devoted to the basics of advanced life support.
 Stabilize airway & maintain adequate ventilation to
maintain SpO2 75-85%.
 Reliable vascular access is essential, including an
arterial line.
 In the neonate, this can most reliably be
accomplished through the umbilical vessels.
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47. Volume resuscitation and Ionotrpes
 Inotropic support-
 Dopamine can be expected to increase MAP,
improve ventricular function, and improve urine
output at doses of <10micro g/kg/min.
 Dobutamine- in few studies showed favourable
results compared to dopamine
 Combination of low dose dopamine +
dobutamine
 Upgrade ionotropes for desired improvement.
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48. Manage cont..
 Supportive measures
 Detection and Management of hypoglycemia,
hypocalcemia, hypothermia and metaboilic
acidosis
 CCF and its management
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49. Recommendation
 Continuous infusion of prostaglandin-E1 in low
dosage of 0.005–0.01 micro g/kg/min is never
wrong.
 Treatment should immediately be started in any
newborn, whose condition worsens clinically in
the first days of life; in particular, if the
cardiovascular system cannot be immediately
analyzed by echocardiography.
 This recommendation is also true, when sepsis is
suspected or even confirmed by laboratory data.
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50. Key points in the treatment of suspected or
confirmed CHD with duct-dependent SBF:
 Reopening of the arterial duct
(initially high PGE1 dosage, rapid reduction)
 Reduction of diastolic left to right shunt across a non-
obstructive right-left-shunting DA;
 Avoidance of inadequate measures resulting in the
reduction of the Rp;
 Reduction of Rs without jeopardizing adequate
perfusion pressures;
 Rapid transfer of the patient to a pediatric cardiac
center.
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51. Prostaglandin E1
 PGE1 has been used since the late 1970s to
pharmacologically maintain patency of the ductus
arteriosus in patients with duct-dependent systemic or
pulmonary blood flow.
 PGE1 must be administered as a continuous parenteral
infusion.
 The usual starting dose is
0.05 to 0.1 μg/kg/minute.
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52. Prosta….
 The response to PGE1 is often immediate if patency of
the ductus arteriosus is important for the hemodynamic
state of the infant.
 Failure to respond to PGE1 may mean that the initial
diagnosis was incorrect, the ductus arteriosus is
unresponsive to PGE1 or the ductus is absent.
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53. Prosta….
 In most infants, the ductus will reopen within 30
minutes to 2 hours after starting PGE1 evidenced by
PO2 values typically rise 20-30 mm Hg.
 Once the ductus has opened, the dose can usually be
reduced to 0.002-0.05 mcg/kg/min.
 Therapy is continued until balloon atrial septostomy
or cardiac surgery is performed.
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54. Preparation of Prostaglandin E1
Add 1 Ampule
(500 μg/1 mL)
to
Concentratio
n
(μg/mL)
mL/hour × Weight (kg)
Needed to Infuse 0.1
μg/kg/minute
200 mL D10% 2.5 2.4
100ml D10% 5 1.2
50ml D10% 10 0.6
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55. PGE1 side effects
 Apnea (10% to 12%)- dose dependent
 Fever (14%)
 Cutaneous flushing (10%)
 Bradycardia (7%),
 Seizures (4%)
 Tachycardia (3%)
 Cardiac arrest (1%)
 Edema (1%).
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56. Urgent cardiac intervention
 Balloon atrial- septostomy (Rashkind procedure)
 Live-threating hypoxemia can be effectively treated by
atrial septum manipulation.
 Compromised systemic blood flow due to duct
obstruction by transcatheter stenting, pulmonary run
off by bilateral surgical pulmonary banding.
 Critical aortic coarctation by balloon angioplasty as
bridging procedure to surgical repair.
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57. Balloon atrial- septostomy
 This procedure may be performed either in an
intensive care unit under echocardiographic guidance
or in the cardiac catheterization laboratory under
fluoroscopic guidance.
 Similarly, either the femoral vein or the umbilical
vein can be used for the venous approach.
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58. Balloon cont..
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59. Refferal
 A well-prepared rapid transfer of the patient to a
pediatric cardiac center is the most important measure
for appropriate disease specific cardiac intervention by
expert.
 Continuous monitoring of all vitals.
 Monitoring of hypoglycemia, hypothermia and
ongoing medication.
 Well organized cardiac care ambulance with trained
health care professional.
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60. Preventive measures
 Antenatal diagnosis of cardiac defects by USG, fetal
Echo and doppler in suspected newborn demise in
past.
 Immunization with rubella vaccine.
 A newborn with critical CHD should be delivered in a
pediatric heart center (PHC), if possible.
 In case of a postnatal diagnosis, transfer to a PHC
should be immediately prepared.
 Pulse oxymetry.
 Educate front line health workers.
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61. Take home message
 Careful postnatal examination.
 Universal screening for CHD
 Mandatory use of pulse oxymetry, no new-born
should be discharged without POx.
 POx screening preferably after 24hrs & if
abnormal 2D Echo and doppler by expert.
 Any neonate who is detoriating in 1st few weeks of
life, should be started with PGE1 even in case of
proved sepsis.
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62. Take cont…
 Remember left sided obstructive lesions presents
with shock, where as right sided obstructive
lesions presents with cyanosis & features of CCF.
 Any newborn who comes to ED with shock/CCF in
1st few weeks, should be resuscitated first,
followed by PGE1 and transfer of pateint to
pediatric cardiac centre.
 All newborn with sepsis/ respiratory distress
should be evaluated for cCHD and 2D ech.
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63. Thank you
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