SYMPOSIUM: NEONATOLOGY
Diagnosis and
management of critical
congenital heart defects
in infants
Yogen Singh
Abstract
Congenital heart defects (CHD) are the most common congenital
anomaly with an incidence of around 8e10 per 1000 live born infants.
Up to a third of all the CHDs are of a critical type. A critical CHD is fatal
within 28 days of birth unless there is cardiac surgery or catheter inter-
vention. Advances in diagnosis, pre-operative intensive care, cardiac
surgery techniques, catheter interventions and post-operative man-
agement, have made it possible to save most of these infants with
excellent long-term outcomes. Prompt recognition is essential as
delayed diagnosis of critical CHD is associated with higher mortality
and morbidity. Accurate, timely diagnosis allows healthcare profes-
sionals to institute timely and specific therapy. Although echocardiog-
raphy is required to precisely delineate the anatomical abnormality,
with a good understanding of the cardiovascular physiology it is usu-
ally possible to define the haemodynamic abnormality based upon the
clinical examination, pulse oximetry, blood gas and radiographic find-
ings. This short article outlines how these findings can be used to
guide clinicians in anticipation of diagnostic echocardiography and/
or cardiac catheterization. It outlines the principles for establishing a
diagnosis and offers guidance on how to initiate immediate manage-
ment and stabilization of affected babies.
Keywords cardiac emergencies; cardiovascular physiology;
congenital heart disease; critical congenital heart defect; diagnosis
and management; neonate
Introduction
The incidence of congenital heart defects (CHD) is around in 8
e10 per 1000 live births and 25e33% of all the CHDs are critical
congenital heart conditions. Critical CHD is defined as a
congenital heart condition needing surgery, intervention or
leading to death within 1 month of birth. 50e60% of these crit-
ical CHDs are detected on fetal anomaly screening. The routine
newborn physical examination alone, which is often conducted
during a period of transitional circulation, will fail to detect up to
half of undiagnosed critical CHDs.
Pulse oximetry screening 24e48 hours after birth may help in
detecting cyanotic heart conditions, however, non-cyanotic
Yogen Singh MD MA (Cantab) FRCPCH, Professor of Pediatrics, Loma
Linda University School of Medicine, California, USA, and
Department of Paediatrics - Paediatric Cardiology and Neonatal
Medicine, Cambridge University Hospitals NHS Foundation Trust,
Cambridge, UK. Conflicts of interest: none declared.
PAEDIATRICS AND CHILD HEALTH 32:9
critical CHDs such as coarctation of aorta may go undetected on
pulse oximetry screening in asymptomatic infants. A delay in
diagnosis of CHDs is associated with higher mortality and
morbidity, and hence in order to provide the best outcomes it is
essential to detect them in asymptomatic babies.
The clinical signs and symptoms of critical CHDs are non-
specific in neonates, especially during the transitional circula-
tion, and the routine bedside testing such pulse oximetry, blood
gas and chest X-ray often doesn’t greatly assist in making a
diagnosis. Urgent, 24/7 echocardiography by a skilled provider is
not available in all emergency settings. This poses a significant
challenge to the neonatologists, paediatricians or health care
professionals who may encounter infants with undiagnosed
critical CHDs. It is crucial for them to have a good understanding
of perinatal cardiovascular physiology, know how to recognize
critical CHDs and be able to initiate management.
Clinical presentation of infants with congenital heart
defects
The signs and symptoms of CHDs in neonates are often non-
specific e.g. poor feeding and may be absent during the transi-
tional period. The signs and symptoms suggestive of CHDs are
summarized in Box 1. Careful examination by experienced cli-
nicians is essential to detect some of the more specific signs e.g.
weak or absent femoral or brachial pulses. This is particularly
important if there are risk factors in the history such as abnor-
malities detected antenatal scans or dysmorphic features that
increase the risk of CHD.
Classification of congenital heart defects
CHDs can be classified according to postnatal adaptation
(Table 1). This broadly separates infants into two groups; duct-
dependent and non duct-dependent.
Cyanotic CHDs
Cyanotic CHDs constitute about 20% of congenital heart lesions.
Most of the cyanotic CHDs presenting in the neonatal period start
with ‘T’ (summarized in Box 2).
Signs and symptoms suggestive of underlying CHD
Signs and symptoms
C Unwell infant, poor feeding
C Weak or absent pulses (femoral and/or brachial pulses)
C Heart murmur maybe present but often absent in critical CHDs
C Presence of dysmorphic features or other congenital anomalies
C Persistent cyanosis in absence of respiratory distress or cyanotic
episodes
C Low oxygen saturation (<95% in air) or difference of >3% be-
tween pre- and post-ductal oxygen saturations
C Presence of arrhythmias
C Heart failure
C Collapse/sudden death
Other risk factors of CHD in neonates
C Suspicion of CHD on fetal anomaly screening (FAS)
C Positive family history of CHD
Box 1
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 SYMPOSIUM: NEONATOLOGY

Classification of congenital heart defects based upon postnatal adaptation

Types of CHDs Examples of pathologies

Duct-dependent CHDs Duct-dependent pulmonary circulation Pulmonary atresia (PA)

(Cyanotic CHDs) Tricuspid atresia with intact IVS
Critical TOF
Critical pulmonary stenosis (PS)
Single ventricle with PS/PA
Severe Ebstein anomaly
Duct dependent systemic circulation Hypoplastic left heart syndrome (HLHS)
(Acyanotic CHDs) Critical aortic stenosis (AS)
Severe coarctation of aorta CoA
Interrupted aortic arch (IAA)
Single ventricle (SV) with severe AS or CoA
Poor central mixing (cyanotic CHD) TGA with intact ventricular septum
Non duct-dependent CHDs Mild cyanotic CHDs TAPVC
TOF
Truncus arteriosus communis (TAC) with mild
PS
TGA with VSD
Single ventricle
Left-to-right shunt CHDs Ventricular septal defect (VSD)
Patent ductus arteriosus (PDA)
Atrio-ventricular septal defect (AVSD)
Aorto-pulmonary window (APW)
Double outlet right ventricle (DORV) without
or with mild PS
Truncus arteriosus communis (TAC)
Single ventricle

Adapted from Singh Y, Tissot C. Echocardiographic Evaluation of Transitional Circulation for the Neonatologists. Front Pediatr 2018; 6:140.
AS - aortic stenosis; ASD - atrial septal defect; APW - aorto-pulmonary window; AVSD -atrio-ventricular septal defect; CHD - congenital heart disease; COA - coarctation of
aorta; DORV - double outlet right ventricle; HLHS - hypoplastic left heart disease; IAA - interrupted aortic arch; IVS - intact ventricular septum; PA - pulmonary atresia; PDA
- patent ductus arteriosus; PS - pulmonary stenosis; SV - single ventricle; TA - tricuspid atresia; TAC - truncus arteriosus communis; TAPVC - total anomalous pulmonary
venous connection; TGA - transposition of great arteries; TOF - Tetralogy of Fallot; VSD - ventricular septal defect.

Table 1

Acyanotic congenital heart defects
Acyanotic CHDs form a spectrum of congenital heart lesions with
variable presentation depending upon the underlying anatomical
and physiological abnormality. Infants may be completely
asymptomatic presenting with a heart murmur alone or suffer
Cyanotic CHDs presenting in neonatal period
5 Ts in cyanotic CHDs
C Tetralogy of Fallot (TOF) and double outlet right ventricle (DORV) -
most common cyanotic CHD (Figure 1)
C Transposition of great arteries (TGA) - most common CHD pre-
senting in neonatal period (Figure 2)
C Truncus arteriosus
C Total anomalous pulmonary venous connection (TAPVC)
C Tricuspid atresia
C Pulmonary atresia with no VSD (severe spectrum of TOF )
C Ebstein anomaly (rare with variable presentation depending upon
the severity of the lesion)
Box 2
acute collapse or even death. The latter is more common in
critical CHD. Common critical acyanotic CHDs are left heart
obstructive duct-dependent lesions such as Hypoplastic left heart
syndrome (HLHS), Critical aortic stenosis (AS), Severe coarcta-
tion of aorta (CoA), Interrupted aortic arch (IAA), and Single
ventricle (SV) with severe AS or CoA, which may present with
acute deterioration when ductus arteriosus closes.
Clinical presentation of infant with critical CHD
The timing of presentation and severity of the presentation de-
pends upon:
1. The nature and severity of underlying defect.
2. The alteration in cardiovascular physiology secondary to the
effect of the transitional circulation, i.e.
A. Closure of ductus arteriosus (DA)
B. Restriction of patent foramen ovale (PFO)
C. Fall in pulmonary vascular resistance (PVR)
Critical cyanotic CHD
Cyanosis results from the presence of more than 50g/L of
deoxygenated haemoglobin. It follows that the ability to

PAEDIATRICS AND CHILD HEALTH 32:9 333 Ó 2022 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: NEONATOLOGY

Figure 1 Anatomy in tetralogy of Fallot tetralogy e the most common cyanotic congenital heart defect. Image a and b: show bigger right side of
the heart with caudally deviated interventricular septum, mal-aligned ventricular septal defect (VSD) leading to over-riding of aorta. Image c
showing VSD position at 10 O’clock with right ventricular outflow obstruction in parasternal short axis view. Image d shows similar anatomy but
with more than 50% over-riding of aorta, hence classified as double outlet right ventricle (DORV). The central image shows the flow pattern. RV e
right ventricle, LV - left ventricle, Ao e aorta (ascending aorta). Copyright Yogen Singh.

recognize cyanosis is related to the level of haemoglobin.
Cyanosis can be recognized more easily in babies with higher
haemoglobin concentrations.
Cyanosis in an infant may be due to one of four mechanisms:
(1) shunting of deoxygenated blood from the right side of the
heart to the left side systemic circulation leading to intracar-
diac shunt secondary to cyanotic CHD
(2) extrapulmonary right-to-left shunts at PDA or PFO in pulmo-
nary hypertension
(3) incomplete oxygenation process of blood passing through the
lung due to lung pathology e.g. intrapulmonary shunt or
(4) abnormal haemoglobin that cannot bind to oxygen.
diseases (for hyperoxia test details - see below in approach to
management).
Measuring preductal saturation (right arm) and post-ductal
saturation (right leg) may show a significant difference be-
tween pre- and post-ductal PaO2 indicating that the blood is
shunted at the level of PDA from pulmonary to systemic circu-
lation as it happens in cases of severe PPHN or CHDs with right
to left ductal shunt (such as in interrupted arch of aorta).
Critical acyanotic congenital heart diseases
Patients with acyanotic heart diseases may present acutely in a
critical condition either because of left-sided obstructive lesion or
due to heart failure.

The first two mechanisms occur in cyanotic infants due to CHDs
(central mixing such as in tetralogy of Fallot’s or across PDA in
pulmonary atresia without VSD). When deoxygenated blood is
mixed with oxygenated blood, the resultant blood has a lower
oxygen saturation than normal. This may lead to significant central
cyanosis. This type of shunt is called an intracardiac shunt.
One of the clinical bedside tests that can help in differenti-
ating intrapulmonary from intracardiac shunt is by giving 100%
O2 through oxygen hood to the affected neonate (the hyperoxia
test). The peripheral saturation usually improves in cases of lung
diseases associated with intrapulmonary shunt; while on the
other hand, the cyanosis, O2 saturation, and PaO2 do not
improve in cases of intracardiac shunt due to congenital heart
Critical acyanotic ductal-dependent heart diseases
presenting with shock
During the fetal and transitional circulation ductus arteriosus
(DA) maintains the blood flow to systemic circulation bypassing
the area of stenosis or interruption. When DA closes the flow to
descending aorta, in duct dependent left heart obstructive le-
sions, will be comprised leading to tissue hypoperfusion and
tissue hypoxia. This subsequently leads to lactic acidosis and
shock. The most common acyanotic critical CHD is neonatal
coarctation of the aorta (Figure 3). Other critical CHDs lesions in
this category include other left-sided obstructive cardiac lesions
such as interrupted aortic arch (IAA), critical aortic stenosis (AS),
aortic valve atresia, mitral atresia and HLHS.

PAEDIATRICS AND CHILD HEALTH 32:9 334 Ó 2022 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: NEONATOLOGY

Figure 2 Transposition of great arteries (TGA) e most common cyanotic congenital heart defect presenting in the neonatal period. Image a
showing anatomy in 2 D and image b with color Doppler - pulmonary artery originating from posterior ventricle (left ventricle) and dividing soon
after origin while ascending aorta originating from anterior (right) ventricle. Copyright Yogen Singh.

Without a timely and appropriate intervention, an infant with
critical coarctation or severe left-sided obstruction may deterio-
rate rapidly with possible demise. Clinicians should have a low
index of suspicion of critical acyanotic CHD in infants presenting
with shock during the first couple of weeks after birth. These
infants may mimic the clinical picture of septic or hypovolemic
shock e with early non-specific signs and symptoms and then
sudden deterioration leading to shock.
Management of infants with suspected critical acyanotic CHD
including ductal-dependent acyanotic heart lesion should include
hemodynamic stabilization and initiation of appropriate dose of
prostaglandin E1 infusion (see below) as soon as possible. When
there is a clinical suspicion of acyanotic critical CHD, there should
not be any delay in starting prostaglandin E1 infusion and clinicians
should not wait for a confirmatory echocardiography or cardiologist
opinion to start the drug, although they should be sought urgently.
Acyanotic heart diseases presenting with heart failure
This category of CHDs compromise infants with significant left to
right shunt resulting in increased pulmonary blood flow and thus

Figure 3 Critical coarctation of aorta (CoA). Image a and b show dilated right side of the heart with right ventricle (RV) being big and rounded in
image b. There is a muscular VSD seen in image b. Image c showing critical coarctation of aorta with severe narrowing at the isthmus, junction
between transverse arch and descending aorta. Image d shows classical diastolic decay seen in infants with coarctation of aorta e there is
increased velocity in systole and diastolic ‘tail’ which remains high during diastole. Copyright Yogen Singh.

PAEDIATRICS AND CHILD HEALTH 32:9 335 Ó 2022 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: NEONATOLOGY
leading to heart failure. The common heart lesions include
ventricle septal defect (VSD), atrioventricular septal defect
(AVSD), PDA or combination of lesions. As the PVR drops after
birth, there is increased left (systemic) to right (pulmonary)
shunt leading to congestion of lungs from increased pulmonary
blood flow. They often present with signs and symptoms of heart
failure (breathlessness, tachypnoea, tachycardiac, poor feeding,
hepatomegaly and cardiomegaly) around 4e6 weeks after birth.
Any infant presenting with heart failure during the first 1
e2 weeks after should be suspected to have more serious critical
CHD such as left heart obstructive lesions.
An approach to understand cardiovascular physiology in
infants presenting as neonatal emergency
As discussed above, both acyanotic and cyanotic CHDs can
present as neonatal emergencies. These cardiac emergencies can
be broadly divided into 3 categories: duct-dependent CHDs, cir-
culation dependent upon mixing of blood centrally via foramen
ovale, and obstructed total anomalous pulmonary venous
connection (TAPVC).
Ductal dependent CHDs
In neonates with duct dependent critical CHDs, closure or
constriction of ductus arteriosus may be associated with pro-
found circulatory compromise. Duct dependent circulation can
be categorized in the following three categories:
1) To maintain adequate pulmonary blood flow in right sided
obstructive lesions (cyanotic CHDs)
 Pulmonary atresia with intact ventricular septum
 Pulmonary atresia with ventricular septal defect
 Critical pulmonary stenosis (PS)
 Tricuspid atresia with pulmonary atresia or critical (PS)
 Univentricular heart with pulmonary atresia
 Severe Ebstein’s anomaly
2) To maintain adequate systemic blood flow in left sided
obstructive lesions (Acyanotic CHDs)
 Hypoplastic left heart syndrome (HLHS)
 Critical aortic stenosis/aortic atresia
 Severe or critical coarctation of aorta
 Interrupted aortic arch (IAA)
 Single ventricle with severe aortic stenosis/coarctation
3) To ensure adequate central mixing in conditions with parallel
circulation
 Transposition of great vessels (TGA) without VSD
The circulation in infants with duct dependent CHDs require
patent ductus arteriosus to keep them stable until a definitive
procedure/intervention is performed. The dose and indications
vary depending upon timing of diagnosis and clinical pre-
sentations as discussed in the section below.
Circulation dependent upon central mixing at foramen
ovale
Certain congenital heart conditions require patent foramen ovale
to maintain hemodynamic stability in the newborn. The
congenital heart conditions requiring unrestricted intra-atrial
communication can be categorized in two sub-groups:
1) To maintain systemic blood flow
 Tricuspid atresia
 Pulmonary atresia with intact septum
PAEDIATRICS AND CHILD HEALTH 32:9
 Total anomalous pulmonary venous connection (TAPVC)
2) To relieve pulmonary congestion in
 Hypoplastic left heart syndrome/hypoplastic left ventricle
 Mitral stenosis/atresia
3) To permit adequate mixing of deoxygenated and oxygenated
blood as in TGA with intact ventricular septum.
These infants deteriorate when blood flow across the foramen
ovale is restricted, despite a patent ductus arteriosus. They need
urgent intervention (atrial septostomy or atrial septectomy) to
facilitate unrestricted blood flow via foramen ovale.
Obstructed total anomalous pulmonary venous
connection
Obstructed TAPVC, usually infracardiac type, presents as true
time critical emergency and clinical picture mimics severe PPHN.
However, these infants get worse with fall in PVR as compared to
persistent pulmonary hypertension of the newborn (PPHN), and
they often deteriorate after starting nitric oxide to manage PPHN
like clinical picture. Drop in PVR leads to increased pulmonary
blood flow but it increases pulmonary congestion because
drainage in obstructed TAPVC is blocked.
An urgent pediatric cardiology consultation is warranted
when a critical CHD is suspected, and a comprehensive echo-
cardiography would provide a definitive diagnosis.
A simplified approach to evaluation and initial
management of infants with suspected congenital heart
disease
The clinical presentation of CHD in the neonatal period can be
non-specific mimicking other more common conditions in in-
fancy such as sepsis, respiratory, hypovolemia or metabolic
condition. Therefore, having a high index of suspicion and a
systematic approach is vitally important for the timely diagnosis
and management. The initial evaluation of an infant with sus-
pected CHD should include a detailed history including obstetric
history and family history, meticulous physical examination
including palpation of peripheral pulses and measuring pre- and
post-ducal saturations, and hyperoxia test. The presence of
dysmorphic features and other congenital anomalies may help in
suspecting underlying CHD.
During the fetal period the shunt direction across ductus
arteriosus is right-to-left shunt (from pulmonary artery to
aorta) because of high PVR and low SVR. This leads to higher
pre-ductal saturation than post-ductal saturation, which is
often seen soon after birth during early transitional circulation
and infants with persistently high PVR (such in infants with
severe Persistent Pulmonary Hypertension of Newborn). Right
to left shunt can also be seen in certain critical CHDs such as
interrupted arch of aorta and critical aortic stenosis/aortic
atresia.
As discussed above, hyperoxia test can be useful in differ-
entiating between the cardiac and respiratory causes of
cyanosis in newborns infants. It works on the assumption that
in infants with cyanotic CHDs or those with right to left shunt,
regardless of the level of alveolar oxygenation, the desaturating
effect of the lesion/shunt will not alter. Oxygen is administered
through a plastic hood for at least 10 minutes in order to fill the
alveolar spaces completely with oxygen and arterial partial
336 Ó 2022 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: NEONATOLOGY

pressure of oxygen (PaO2) is measured before and after deliv-

ering 100% oxygen to assess the response to inhaled oxygen Dose of prostaglandin E1 infusion and desired response
(hyperoxia test). In infants with cyanotic CHD, the rise in PaO2 in infants with critical congenital heart defects
is usually no more than 10e30 mmHg and rarely exceeds Dose of prostaglandin E1 in different clinical scenarios of critical
100 mmHg. In comparison, in infants with pulmonary diseases, CHDs
PaO2 often rises to over 100 mmHg. However, infants with
massive intra-pulmonary shunt from a respiratory disease may Antenatal diagnosis of Duct- 5e10 ng/Kg/min or 0.005e0.01
not show a rise in PaO2 to more than 100 mmHg. Conversely, dependent CHD or Cyanotic mg/kg/min
some infants with cyanotic defects with a large pulmonary infant, well and not acidotic
blood flow, such as TAPVC, may demonstrate a rise in PaO2 of Infant with absent femoral 10e15 ng/Kg/min or 0.01
100 mmHg or higher. Therefore, hyperoxia test should be pulses, well and not acidotic e0.015 mg/kg/min
interpreted in the light of clinical picture and the degree of Unwell or acidotic infant with 50e100 ng/Kg/min or 0.05e0.1
pulmonary pathology seen on X-ray. In the modern NICU suspected duct-dependent CHD mg/kg/min
practice with easy access to echocardiography, hyperoxia test Desired response to prostaglandin E1 infusion in infants with critical
has been replaced by the early echocardiography which helps CHDs
in delineating anatomic abnormality and also provides addi- Suspected or known left-heart Palpable femorals, normal pH
tional hemodynamic information that is beneficial to manage- obstruction and lactate <2mmols/L
ment of an infant with critical CHD. Suspected or known right-heart Oxygen saturations 75e85%
Four limb blood pressures are of limited value in neonates but obstruction with normal lactate <2mmols/L
can be helpful in older children. If the systolic pressure in right Transposition of Great Arteries Oxygen saturations >75% with
arm exceeds 10 mmHg difference as measured in the leg, an (TGA) normal lactate <2mmols/L
aortic arch anomaly is likely. PDA may not allow this gradient to
manifest. Therefore, the absence of a systolic pressure gradient Table 2
alone does not rule out aortic arch anomaly in neonates.
Chest X-ray is important in excluding respiratory pathology, These infants should be discussed with pediatric cardiologists as
although its role is limited in diagnosing CHDs. The presence of soon as possible.
cardiomegaly or pulmonary vascular markings (plethoric or oli- In infants with suspected duct-dependent critical CHDs
gemic lung fields) may help in suspecting underlying CHD. ECG maintaining ductal patency can be lifesaving and there should
may be diagnostic in certain categories of CHD such as superior not be a delay in starting prostaglandin E1 while waiting for
axis deviation is seen in infants with tricuspid atresia, atrioven- echocardiography or specialist advice.
tricular septal defect (AVSD) and congenitally corrected TGA Infants on a low dose of prostaglandin E1 (less than 15 ng/kg/
(ccTGA). minutes) are unlikely to have adverse effects such as apnea and
Transthoracic echocardiography remains the gold standard in they do not routinely need mechanical ventilation. As prosta-
diagnosing CHDs and delineating the underlying anatomy. It may glandin infusion can cause apnea and hypotension, continuous
be available immediately in the emergency room or when acute cardiorespiratory monitoring is indicated. The adverse effects of
neonatologist/pediatrician encounters an undiagnosed CHD. As prostaglandin E1 are summarized in Table 3.
discussed above, management should not be delayed in infants Failure to respond to prostaglandin E1 infusion could be
with suspected duct-dependent CHDs while waiting for an because of incorrect diagnosis, insufficient central mixing
echocardiography or cardiology consultation. despite ductal patency (such as in transposition great arteries
Infants presenting with cardiorespiratory failure should first (TGA) with restrictive atrial septum, HLHS with restrictive
be stabilized as per Advanced Pediatric Life Support guidelines atrial septum, etc.) or a lack of ductal response to prostaglandin
and infants with cardiorespiratory failure will often need endo- E1. These infants may deteriorate rapidly without atrial sep-
tracheal intubation and mechanical ventilation, and infants with tostomy irrespective of maintaining ductal patency and this a
suspected duct-dependent CHD should be started on prosta-
glandin E1 as soon as possible.
Adverse effects of prostaglandin E1 infusion in neonates
Use of prostaglandin E1 infants with critical CHDs Common adverse Less common and Adverse effects due
effects rare adverse effects to prolonged use
Infants with an ‘open duct’ (those with antenatal diagnosis or
early presentation) need a small dose (0.005e0.01 mg/kg/minute Apnea Hypothermia Gastric outlet
or 5e10 ng/kg/minute) to maintain ductal patency while Hypotension Bradycardia obstruction
collapsed infants with weak or absent femoral pulses will need a Fever Convulsions syndrome
much higher dose, up to 0.1 mg/kg/minute or 100 ng/kg/minute. Tachycardia Diarrhea Cortical hyperostosis
The expected desired response would be an improvement in Flushing Disseminated
acidosis, serum lactate or femoral pulses in collapsed acyanotic intravascular
infant and an improvement in oxygen saturation in the cyanotic coagulation
CHD (Table 2). The dose can be doubled if response is inade-
quate, and progress should be reassessed every 20e30 minutes. Table 3

PAEDIATRICS AND CHILD HEALTH 32:9 337 Ó 2022 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: NEONATOLOGY
time critical emergency needing urgent discussion with a pe-
diatric cardiologist.
Establishing a definitive diagnosis of CHD
The definitive diagnosis of CHD is made by diagnostic echocar-
diography which should be performed by a pediatric cardiologist
or clinician trained in performing echocardiography for congen-
ital heart defects such Pediatrician (or Neonatologist) with
Expertize in Cardiology (PEC). If a CHD is suspected on neona-
tologist performed echocardiography (NPE) or targeted neonatal
echocardiography (TNE), even when NPE/TNE trained clinician,
the infant should be referred to the specialist pediatric cardiolo-
gist. All infants with suspected critical CHDs should be discussed
urgently with the pediatric cardiologist.
Conclusion
Infants with CHDs, even those with critical CHDs, may remain
completely asymptomatic during the transitional period when
fetal shunts are open and often present with non-specific signs
and symptoms. Clinicians should keep a low index of suspicion
of critical CHDs in infants presenting with shock or cyanosis
during the neonatal period. Neonatologists and acute pediatri-
cians need to have a good understanding of cardiovascular
physiology and the varied presentations of undiagnosed critical
CHDs. Timely and the appropriate dose of prostaglandin E1
infusion can be lifesaving in ductal-dependent CHDs. There
should not be any delay in starting prostaglandin E1 infusion in
infants with suspected critical CHD while waiting for the echo-
cardiography or cardiology consultation. A
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Key practice points in infective endocarditis in
children
C The incidence of CHD is around 8e10 per 1000 live births, and
around 25e33% of these lesions are critical lesions needing ur-
gent surgery or intervention.
C The signs and symptoms of critical congenital heart defects are
often non-specific soon after birth.
C A delay in diagnosis of critical CHDs leads to poor outcome with
higher mortality and morbidities.
C A high degree of suspicion is warranted in critical CHDs in infants
presenting with shock or hypoxia.
C Understanding perinatal cardiovascular physiology is essential for
timely diagnosis and initiate appropriate management for infants
with critical CHDs.
C Timely recognition and therapy with prostaglandin E1 infusion
can be lifesaving in duct dependent neonatal cardiac
emergencies.
338 Ó 2022 Elsevier Ltd. All rights reserved.