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Guideline management of congenital heart diseases

To know:

- Classification of congenital heart diseases.

- Presentations of congenital heart diseases.
- Evaluation of infant with suspected congenital heart diseases.
- Lesion-suggestive presentations & their outline management.
 Respiratory distress.
 Cyanosis.
o Approach to a cyanotic neonate.
o Differential diagnosis of a cyanotic neonate.
 Shock.
o Management of Coarctation of the Aorta
 Congestive Heart Failure.
 Heart murmurs.
- Duct dependent lesions.
- Patent ductus arteriosus.
Classification of congenital heart diseases (CHD):
Classification is based on

 The presence or absence of cyanosis.

 The status of pulmonary blood flow—whether normal, increased, or
 The presence of right ventricular hypertrophy (RVH), left ventricular
hypertrophy (LVH) or both to further narrow the possibilities.
n y
c a
r n
e o
a ti
s c
e d
d e
P e
B c
F t
Figure 7-1. Flow diagram of cyanotic congenital heart defects (Park, 2008c).
BVH, biventricular hypertrophy; HLHS, hypoplastic left heart syndrome; L-R, left-to-right; LVH, left
ventricular hypertrophy; PA, pulmonary artery; PBF, pulmonary blood flow; PS, pulmonary stenosis;
RBBB, right bundle branch block; RV, right ventricle; RVH, right ventricular hypertrophy; TAPVR, total
anomalous pulmonary venous return; TGA, transposition of the great arteries; TOF, tetralogy of Fallot;
VSD, ventricular septal defect. Only common entities are listed in the flow diagrams.
n c
c y
r a
e n
a o
s ti
e c
d d
e f
P e
BB cc tt
F s
Figure 7-2. Flow diagram of acyanotic c congenital heart defects (Park, 2008c).
AR, aortic regurgitation; AS, aortic stenosis; ASD, atrial septal defect; BVH, biventricular hypertrophy;
CoA, coarctation of the aorta; ECD, endocardial cushion defect; EFE, endocardial fibroelastosis; L-R, left-
to-right; LVH, left ventricular hypertrophy; MR, mitral regurgitation; MS, mitral stenosis; PAPVR, partial
anomalous pulmonary venous return; PBF, pulmonary blood flow; PDA, patent ductus arteriosus; PS,
pulmonary stenosis; RBBB, right bundle branch block; RVH, right ventricular hypertrophy; VSD,
ventricular septal defect.

Remember- Normal ECG and normal pulmonary vascular markings on chest x-ray
films do not rule out CHD .

Presentations of CHD:
Findings that should alert to the possibility of serious CHD in a newborn include:

1. Tachypnea of greater than 60 breaths/minute with or without retraction

suggests a cardiac abnormality.
2. Cyanosis, particularly when it does not improve with the administration of
3. Shock.
4. Metabolic acidosis (unexplained)
5. Difference in pulses (arm vs. leg), e.g. suggesting CoA.
6. Weak peripheral pulses, suggesting hypoplastic left heart syndrome (HLHS)
or circulatory shock.
7. Bounding peripheral pulses suggesting aortic runoff through the PDA .
8. Prominent heart murmur.
9. Single 2nd heart sound.
10.Abnormally loud 2nd heart sound.
11.Hyperactive precordium .
12.Hepatomegaly may suggest a heart defect. A midline liver suggests asplenia
or polysplenia syndrome.
13.Irregular cardiac rhythm and abnormal heart rate suggest a cardiac
Repeated examination is important because physical findings change rapidly in
normal infants as well as in infants with cardiac problems.

Evaluation of an infant with suspected CHD:


Family: hereditary diseases, previous sibling with CHD.

Pregnancy and perinatal: Viral infections, maternal medicines, and maternal


Postnatal: Poor feeding, cyanosis, tachypnea, tachycardia, edema.


Factors that ↑ risk for CHD include

 Maternal diabetes mellitus.

 Familial presence of genetic syndromes (e.g. Noonan syndrome).
 History of previous infant with CHD.
 Genetic factors.
Physical examination:

Auscultation: (Do this first, before infant starts crying).

 Heart rate, rhythm.

 Heart sounds (especially 2nd sound): intensity, split (presence,
 Murmur: quality (systolic, diastolic, continuous), intensity, timing,
 Other sounds: gallop, click.
Cardiovascular system (remainder): cyanosis, precordial activity, thrill, pulses
(e.g. femoral versus brachial), blood pressure (both arms vs. leg), peripheral
perfusion (capillary refill time).


 Respiratory distress (improving or worsening).

 Hepatosplenomegaly.
 Dysmorphic features.
 Urine output.

I. Chest radiograph:
1. Heart size
Determined using the cardiothoracic (CT) ratio.

a) A large heart indicates large shunt lesions, myocardial failure, or

pericardial effusion. An extremely large cardiac silhouette may be seen in
Ebstein's anomaly
b) A large heart usually rules out tetralogy of Fallot.
Figure 7-3. Diagram showing how to measure (CT) ratio from the posteroanterior
view of a chest x-ray film.

CT ratio is obtained by dividing the largest horizontal diameter of the heart (A + B)

by the longest internal diameter of the chest (C).

CT ratio = (A + B) ÷ C

Where A and B are maximal cardiac dimensions to the right and left of the
midline, respectively, and C is the widest internal diameter of the chest .


 With CT ratio exceeding 60%, the heart is considered enlarged.

 Cardiomegaly is a useful sign in detecting CHD, but highly unspecific .
 The CT ratio cannot be used with accuracy in newborns, in whom a good
inspiratory chest film is rarely obtained. In this situation, the degree of
inadequate inspiration should be taken into consideration .
2. Cardiac silhouette and pulmonary vascularity, edema and lung
a) D-transposition of the great arteries with an intact ventricular septum:
Cardiomegaly with increased pulmonary vascular markings. Egg-shaped
cardiac silhouette, with a narrow, superior mediastinum.

A. B.
Figure 7- 4. A Posteroanterior view of the chest roentgenogram from a 2-month-
old infant with complete transposition of the great arteries. Note cardiomegaly
(cardiothoracic ratio of 0.7), “egg-shaped” heart with narrow waist, and increased
pulmonary vascular markings, which are characteristic of this condition . B. a
diagram of “Egg-shaped” cardiac silhouettes TGA.

a) Tetralogy of Fallot: Heart size normal or smaller than normal. Decreased

pulmonary vascular markings, concave main pulmonary artery segment
with an upturned apex (boot-shaped heart). May have a right aortic arch.

A. B.

Figure 7-5. A. Posteroanterior view of chest roentgenogram in tetralogy of Fallot.

The heart size is normal, and pulmonary vascular markings are decreased. A
hypoplastic main pulmonary artery segment contributes to the formation of the
“boot-shaped” heart, B. A diagram of“Boot-shaped” cardiac
tetralogy of Fallot.

a) Tricuspid atresia: Normal size or mild cardiomegaly with straight right heart
border with decreased pulmonary vascular markings

Occasionally, a concave PA segment may produce a boot-shaped heart, like

the x-ray findings of TOF.
Figure 7-6. Posteroanterior view of chest roentgenogram in an infant with
tricuspid atresia with normally related great arteries. The heart is minimally
enlarged. The pulmonary vascular markings are decreased, and the main
pulmonary artery segment is somewhat concave

a) Pulmonary atresia: Normal size or mild cardiomegaly with right atrial

enlargement and decreased pulmonary vascular markings.

b) Ebstein's anomaly of the tricuspid valve: In severe cases, the heart is

enormous, balloon-shaped, and occupies almost the entire cardiothoracic

Figure 7-7. Posteroanterior view (A) and diagram (B) of chest

roentgenogram from a 2-week-old infant with severe Ebstein's anomaly.
Note extreme cardiomegaly involving primarily the right atrium (RA) and
diminished pulmonary vascularity. AO, aorta; ARV, atrialized right ventricle;
LV, left ventricle; PA, pulmonary artery; RV, right ventricle.

Total anomalous pulmonary venous return: Cardiomegaly with right atrial and
ventricular enlargement and increased pulmonary vascular markings. "Snowman"
heart (or figure-of-8 configuration) can be seen with supracardiac, unobstructed

a) Usually seen after several months. With pulmonary venous obstruction,

there is radiographic evidence of pulmonary edema (diffuse reticular

Figure 7-8. (A) Posteroanterior view of plain chest film demonstrating the
“snowman” sign (B) Angiocardiogram demonstrating anatomic structures that
participate in the formation of the snowman sign; the vertical vein (left superior
vena cava), the dilated left innominate vein, and the right superior vena cava are
opacified. (C) A diagram of snowman” sign of cardiac silhouette).

b) Truncus arteriosus: Cardiomegaly with increased pulmonary vascular

markings. Right aortic arch may be seen.

c) Single ventricle: Depends on the presence or absence of pulmonary

stenosis. May have cardiomegaly with increased pulmonary vascular
markings or a relatively normal-sized heart with decreased pulmonary
vascular markings.

d) Right aortic arch is commonly seen in

o Truncus arteriosus: 36%

o Tetralogy of Fallot: 13-34%

o Double-outlet right ventricle: 20%

o Tricuspid atresia: 5-8%

o Transposition of the great vessels: 3%

o VSD: 2-6%.

b) Size of the great arteries often helps make a specific diagnosis

Figure 7-9. Abnormalities of the great arteries. (A) Prominent main

pulmonary artery (PA)segment. (B) Concave PA segment resulting from
hypoplasia. (C) Dilatation of the aorta may be seen as a bulge on the right
upper mediastinum by a dilated ascending aorta (AA) or as a prominence of
the aortic knob (AK) on the left upper cardiac border.

3. A midline liver strongly suggests complex cardiac defects associated with

asplenia or polysplenia syndrome.

Figure 7-10. An x-ray film of the chest and upper abdomen of a newborn infant
with polysplenia syndrome. Note a symmetrical liver (“midline liver”), a
stomach bubble in the midline, dextrocardia, and increased pulmonary

II. Electrocardiogram:
It is essential to refer to age-specific charts of normal values for most ECG
III. Monitoring:
O2 saturation by pulse oximetry, both pre-ductal (right hand) and post-ductal
(Right foot).

IV. Arterial pH and blood gas tensions:

Evaluate mainly for hypoxemia and metabolic acidosis.

In contrast to older children and adults, CO2 may be elevated in newborn infants
with congestive heart failure

Obtain Cardiology consultation as soon as the diagnosis of CHD is


Confirmation of the diagnosis

1. Echocardiography
Providing information about the structure and function of the heart and great
vessels in a timely fashion.

As the procedure may take an hour or more to perform, and may therefore not
be well tolerated by a sick and/or premature newborn, pay attention to

 Temperature instability due to exposure during this extended time of

examination may be a problem in the neonate.
 Extension of the neck for suprasternal notch views of the aortic arch may
be problematic, particularly in the neonate with respiratory distress or with
a tenuous airway .
Sick neonates should be closely monitored by a medical staff person
other than the one performing the echocardiogram, with attention to
vital signs, respiratory status, temperature, etc .

2. Multi-slice spiral Computed topography (C.T.):

Can be used in functional evaluations, including ventricular wall motion,
ventricular ejection fraction, and motion of cardiac valves, as well as
enabling the performance of high-quality coronary CT angiography .
3. Cardiac catheterization
Is rarely necessary for anatomic definition of intracardiac structures or for
physiologic assessment.

Diagnostic indications of catheter:

A. Anatomic definition (not visualized by echocardiography) of
1. Coronary arteries
2. Aortic to pulmonary artery collateral vessels
3. Distal pulmonary artery anatomy
B. Hemodynamic measurements

Lesion-suggestive presentations and their outline

I. Respiratory distress:
Likely lesions include:

VSD, PDA, ASD: (left to right shunting lesions)

These rarely cause symptoms in first few days of life in term infants
unless more than one lesion is present.

AV canal, aortico-pulmonary window, total anomalous pulmonary venous

return (TAPVR) without obstruction.
TAPVR with obstruction:
Respiratory signs occur immediately after birth, may be very severe
and worsen progressively requiring emergency intervention.

Truncus arteriosus

 O2 is rarely helpful and may worsen shunting by decreasing pulmonary

vascular resistance.
 Correct anemia (if present).
 Limit sodium and fluid intake, administer diuretics.
 Optimize nutrition.
 Early surgery may be necessary for some
II. Cyanosis

The perception of cyanosis occurs when there is 5 gm of reduced hemoglobin

in the capillaries. In an infant with a normal hemoglobin level, perception of
cyanosis typically occurs at an oxygen saturation of 70%. An experienced
observer can sometimes detect cyanosis when the saturation falls to 80-85%.

Approach to the Cyanotic Infant:

1. Confirm central cyanosis with "Arterial" blood gas (ABG) in room air, if possible,
a sample from the right arm is the best site.
2. Assess the history and examination for cause, including four limbs Blood
 An upper to lower limb systolic difference of > 10mmHg is significant and
suggestive of Coarctation of the Aorta
 Hypotension in a cyanotic infant is a serious finding.
3. Correct metabolic acidosis and systemic hypoperfusion if present with fluid
boluses and bicarbonate.
4. Hyperoxia Test:
 100% FiO2 into headbox for > 10 minutes.
 Monitor SaO2
 Repeat ABG:
 PaO2 > 100mmHg or SaO2 increase by 15%: pulmonary disease likely
(Bowman and Fraser, 2006), except with V/Q mismatching.
 PaO2 < 100 mm Hg, and the rise is usually not more than 10 to 30 mm
Hg: cardiac cause likely, where there is a significant intracardiac right-to-
left shunt.
 Note, with PPHN PaO2
a- May either rise slightly or remain same.
b- With hyperventilation with 100%O2 for 10–15 min to decrease
PaCO2 to 25–30 mmHg & increase pH to >=7.50, a dramatic
increase in PaO2 suggests PPHN.
Limitations of Hyperoxia Test:
 Some critical cyanotic lesions (mixing lesions) may not be excluded if the
hyperoxia test yields a PO2 of >150 torr after 10 minutes
 Total anomalous pulmonary venous return (with a large pulmonary
blood flow).
 Tetralogy of Fallot with a predominant left-to-right shunt.
 Hypoplastic left heart syndrome.
 Conversely, infants with a massive intrapulmonary shunt from lung disease
(but with a normal heart) may not have a rise in arterial Po2 to 100 mm Hg.
 Therefore, the response of PO2 to 100% oxygen inhalation should be
interpreted in light of clinical pictures, especially the degree of pulmonary
pathology seen on chest x-ray films .
 Not as reliable as an echocardiogram and is not as important as resuscitation
and attendance to cardiorespiratory support, especially if acidosis or
respiratory distress is present.
 Limited value when only saturations are measured and not arterial PaO2
and should only be used in conjunction with a thorough clinical assessment.

 In some cases, the hyperoxia test must be done with the administration of
positive-pressure ventilation to expand atelectatic lung adequately to
exchange gas.
5. Assess right and left sided SaO2 for any ductal difference (Bowman and Fraser,
 Arterial PO2 from the right radial artery that is 10 to 15 mm Hg higher than
that from an umbilical artery catheter is significant.
 In severe cases of right-to-left ductal shunt, differential cyanosis may be
noticeable, with a pink upper and a cyanotic lower body. Such a right-to-left
ductal shunt is caused by
1. Persistent pulmonary hypertension of the newborn (PPHN)
2. Aortic obstructive lesions (such as interrupted aortic arch, coarctation of
the aorta [COA]).
6. CXR and ECG, if possible, are often useful in reducing the diagnostic possibilities
7. Intubation and paralysis if significant distress.
8. Two IV lines ideally or UVC.
9. Parental antibiotics (preferably after blood cultures taken).
10. Prostaglandin E1 (Prostin VR ®) infusion if a cyanotic congenital heart defect or
a ductus-dependent cardiac defect is suspected . (See below for duct
dependent lesions).


Measuring oxygen saturation at both preductal and postductal sites is part of

the initial evaluation in a patient with suspected heart disease

Differential cyanosis (i.e. pink upper body and blue lower body):

Preductal oxygen saturation is higher than the postductal oxygen saturation.

This pattern of cyanosis is seen with

1. Persistent pulmonary hypertension of the newborn

2. With left ventricular outflow obstruction (e.g., aortic arch hypoplasia,
interrupted aortic arch, critical coarctation, and critical aortic stenosis).
This sign occurs when the great arteries are normally related and deoxygenated
blood from the pulmonary circulation enters the descending aorta through a PDA
(right-to-left shunting)
Reversed differential cyanosis

When the postductal saturation is higher than the preductal saturation.

1. d-transposition of the great arteries with coarctation of the aorta or

interrupted aortic arch and

2. d-transposition of the great arteries with pulmonary hypertension.

In these situations, the descending aorta is filled with oxygenated blood from the
pulmonary system because of right-to-left shunting through the ductus arteriosus.

Cyanosis could be practically subcategorized into

A. Cyanosis with normal or ↑ pulmonary blood flow:

Likely lesions include:
 Transposition of great arteries (TGA): the commonest form of CHD causing
cyanosis in the newborn period. (post-ductal saturation > pre-ductal).
 Truncus arteriosus (may present with respiratory distress)
 Double outlet right ventricle (DORV)
 Ebstein’s anomaly of the tricuspid valve (prominent RV impulse,

Differential diagnosis of cyanotic newborns with increased pulmonary


Table 7-1. Differential diagnosis of cyanotic newborns with increased

pulmonary vascularity.
BVH, biventricular hypertrophy; CHF, congestive heart failure; D-TGA, complete TGA; DORV, double-
outlet right ventricle; ECG, electrocardiogram; LSB, left sternal border; LVH, left ventricular hypertrophy;
PPHN, persistent pulmonary hypertension of the newborn; PS, pulmonary stenosis; RAH, right atrial
hypertrophy; RVH, right ventricular hypertrophy; TAPVR, total anomalous pulmonary venous return;
TGA, transposition of the great arteries; ULSB, upper left sternal border; VSD, ventricular septal defect.

Management of TGA without VSD:

 Start prostaglandin E1 (PGE1) to dilate the ductus arteriosus, in order to ↑
mixing between pulmonary and systemic circulations and ↑ systemic
oxygenation (See PGE1 use below).
 In patients who do not respond with an increased arterial oxygen saturation to
the opening of the ductus arteriosus with prostaglandin (usually these
neonates have very restrictive atrial defects or pulmonary hypertension),
further management include;
1. Maximizing Intercirculatory Mixing
a) Emergently enlarge the foramen ovale by balloon atrial septostomy.
This can be done in the catheterization laboratory. However, bedside
echocardiography is generally successful for catheter guidance.

b) Hyperventilation and treatment with sodium bicarbonate to promote

alkalosis, lower PVR, and increase PBF, which increases atrial mixing
following septostomy.
2. Maximizing Mixed Venous Oxygen Saturation
3. Decrease oxygen consumption (muscle relaxants, sedation, mechanical
ventilation, normothermia).
4. Improving oxygen delivery (increasing cardiac output with inotropic agents,
increasing oxygen carrying capacity by treating anemia).
5. Coexisting causes of pulmonary venous desaturation (e.g., pneumothorax )
should also be sought and treated.
Increasing the fraction of inspired oxygen to 100% will have little effect on the
arterial PO2, unless it serves to lower PVR and increase PBF.

If severe hypoxemia persists despite the above maneuvers, mechanical support

with extracorporeal membrane oxygenation or early arterial switch surgery may
be indicated ."Not yet available in our unit".

A. Cyanosis with ↓ pulmonary blood flow:

Likely lesions include:

 The most common causes are right-sided obstructive lesions that result in
inadequate blood flow to the lungs.
 Tetralogy of Fallot (TOF) (with or without pulmonic atresia). In mild cases,
cyanosis may not be present at birth (acyanotic Fallot, “pink tet”).
 Tricuspid atresia, pulmonic atresia with intact ventricular septum, severe
pulmonic stenosis.
 Ebstein’s anomaly of the tricuspid valve (Bartman and Teitel, 2003).
 Age of presentation varies depending on the lesion.
For example, Tricuspid atresia presents rapidly immediately after birth, mild
forms of Ebstein’s anomaly may not present until later in life.

 With some lesions, cyanosis decreases as pulmonary vascular resistance drops.

Differential diagnosis of cyanotic newborns with decreased pulmonary

Table 7-2. Differential diagnosis of cyanotic newborns with decreased pulmonary

AV, atrioventricular; BVH, biventricular hypertrophy; CHF, congestive heart failure; DORV, double-outlet
right ventricle; ECG, electrocardiogram; LSB, left sternal border; LVH, left ventricular hypertrophy; PA,
pulmonary artery; PDA, patent ductus arteriosus; PS, pulmonary stenosis; RAH, right atrial hypertrophy;
RBBB, right bundle branch block; RVH, right ventricular hypertrophy; TAPVR, total anomalous pulmonary
venous return; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; TR, tricuspid
regurgitation; ULSB, upper left sternal border; VSD, ventricular septal defect; WPW, Wolff-Parkinson-
Out line management of cyanosis with ↓ pulmonary blood flow:

For these infants, oxygen saturations in the 75-85% range are adequate.

Infants with severe right ventricular outflow tract (RVOT) obstruction can become
significantly cyanotic when PDA closes.

The following should be instituted immediately:

1. Prostaglandin E1 (alprostadil) infusion.

2. 100% oxygen.
3. Depending on the severity of the clinical condition, the patient likely needs
to be intubated and temporarily supported with mechanical ventilation.
4. Intravascular access (preferably a central venous access).
5. Appropriate sedation must be carefully planned.
6. If there is metabolic acidosis, administer sodium bicarbonate 1–2 meq/kg
7. If suspected hypovolemia, crystalloid fluid bolus administration 5–15 ml/kg
8. Optimize electrolytes, including ionized calcium levels.
“Cyanotic spells” of TOF
The following should be considered during an acute episode:

1. 100% Oxygen.
2. Positioning- knee-chest position.
3. Sedation with midazolam 0.05–0.1 mg/kg (IV, intranasal or intrarectal)
fentanyl 1–2 mg/kg (IV), or morphine 0.1 mg/kg (IM, IV, SC) .
4. Beta-blockers: Propranolol.
Propranolol is given by mouth for preventing cyanotic spells.

If a severe cyanotic spell in a child with congenital heart disease persists

despite optimal use of 100% oxygen, propranolol is given by intravenous
infusion .


By mouth; 0.25–1 mg/kg 2–3 times daily, max. 2 mg/kg 3 times daily.

By slow intravenous injection with ECG monitoring

Initially 15–20 micrograms/kg (max. 100micrograms/kg), repeated every

12 hours if necessary (BNFC, 2009a).

5. If cyanosis is still present after 10 minutes, sodium bicarbonate intravenous

infusion is given to correct acidosis
Dose: 1–2 meq/kg IV, or calculated according to arterial blood gas results ).

6. Phenylephrine: 2–5 mg/kg IV every 10–15 min, followed by a continuous

infusion 0.1–5 mg/kg/min as needed.
7. Alternatively is the use of vasopressin. Vasoconstrictors are indicated in
spells that appear refractory to sedation, volume expansion and beta-
8. Crystalloid or red blood cell administration 10–15 ml/kg IV.
Intubation with sedation and muscle relaxation if persistent or refractory

Differential diagnosis of a cyanotic neonate:

Table 7-3. Causes and clinical findings of central cyanosis

Table 7-4. Differential diagnosis of a cyanosed infant.

Breathing Right & PCO2 Severe Response

Pattern Left SaO2 metabolic to 100%
Difference acidosis O2
Primary Tachypnea No  No  PaO2
pulmonar , grunting difference
and SaO2
y Disease and

Cardiac Tachypnea +/- Normal or Present No

,  significant
(Usually 5-
slow/deep change

PPHN Tachypnea > 10-15% Normal or +/- +/-

, recession 
may be

Sepsis Respirator No Normal or +/- Moderate

y distress difference   PaO2
may be
and SaO2

PPHN; Persistent pulmonary hypertension in newborn

Differentiating the infant in severe septic shock from other causes of

cyanosis is very difficult and no safe clinical measure exist, thus generally all
infants with central cyanosis should be commenced on parentral antibiotics
early until further investigation is possible.

Differentiating PPHN from ductal dependent pulmonary cardiac lesions can

be very difficult, if uncertainty exists a PG infusion is generally the safest
Figure 7-11. Algorithm of managing cyanotic congenital heart disease (PaO2 < 100
mm Hg in 100% FiO2).
III. Shock:
Mechanism: Inadequate systemic blood flow.

The onset of signs relates to closure of the ductus arteriosus which may not occur
until a few days after birth.

Likely lesions include:

The most common types of CHD that cause shock are left-sided obstructive

 Mitral atresia or severe mitral stenosis (type of hypoplastic left heart

 Aortic atresia or severe aortic stenosis (type of hypoplastic left heart
 Interrupted aortic arch (IAA).
 Coarctation of aorta (CoA) (usually presents several days after birth)
 Total anomalous pulmonary venous return (TAPVR) with obstruction may
present with both respiratory distress and shock.

 PGE1 to allow right→left shunting through ductus arteriosus.

 Assisted ventilation to treat pulmonary edema (as well as apnea from
If PGE1 is begun in the absence of shock, ventilatory support is not
mandatory, provided that the infant is not apneic and the facilities for
intubation are immediately at hand.

 Be cautious with use of oxygen in setting of a single ventricle. Discuss use of

oxygen with senior cardiologist.
 Oxygen will accelerate the closure of the ductus arteriosus and worsen
infant’s condition by decreasing systemic output. Therefore, do not
increase environmental oxygen until after PGE1 has been started.
 In some cases of hypoplastic left heart, there is excessive pulmonary
blood flow and inadequate systemic output, even with PGE1. In such cases,
the following measures may be used in an attempt to decrease pulmonary
blood flow and increase systemic output. i.e., to decrease the pulmonary-
to-systemic blood flow ratio (Qp:Qs).
o Mechanical ventilation.
o Muscle relaxants are usually necessary (i.e., pancuronium bromide) to
prevent the infant from hyperventilating.
o ↑ PEEP to
 Try to impede pulmonary blood flow.
 To overcome pulmonary venous desaturation from pulmonary edema
secondary to left atrial hypertension.
o Decrease inspired oxygen to ~18% by adding N2 to inspired gas (to ↓
alveolar PO2) - Add CO2 to inspired gas (to ↓ arterial pH). These two
manipulations of inspired O2 and CO2 are not available in our unit.
o Target systemic O2 saturation of 80-85% and PCO2 of 40 mmHg.
 Correct metabolic acidosis.
 Inotropic agents to improve myocardial.
Note, IAA is commonly associated with DiGeorge's syndrome so

 Irradiate blood products given, to avoid Graft-versus-host disease in all

infants with IAA or CoA, unless Digeorge syndrome is specifically
 Hypocalcemia is a common finding in a patient with IAA, even those who
don't have DiGeorge's syndrome, requiring correction.

Specific management of CoA

Mechanical ventilation considerations:

 In newborns with isolated CoA, the expected decrease in the pulmonary

vascular resistance from ventilation and PGE1 does not affect the
hemodynamics. Conversely, in those with CoA and VSD, more blood will
be shunted left to right as the pulmonary vascular resistance falls.
 Ventilation must be carefully controlled to restore the pulmonary-to-
systemic blood flow ratio (Qp:Qs) to the range of 1:1 in patients with
arch obstruction and left-to-right intracardiac shunt.
 Ventilation strategy is designed to increase pulmonary vascular
resistance by
 Lowering the FiO2 to maintain O2 saturations of approximately 85%.
 Adjusting minute ventilation to achieve a pCO2 of 40 to 50 mm Hg.
This strategy for balanced Qp:Qs is necessary to maintain adequate
systemic blood flow.


Low-dose dopamine may be needed as inotropic support for ventricular

failure or to increase renal blood flow by dopaminergic effect when used in
doses of less than 5 mcg/kg/min.

Fluid therapy with CoA & IAA

 While it is appropriate to correct dehydration, fluid volume expansion,

even in the hypotensive infant with critical Coarctation or IAA, may be
 Hypotension is usually not caused by hypovolemia in this setting but
rather by aortic obstruction, ductus closure, and ventricular failure.
 During the initial resuscitation, a small fluid bolus (normal saline, 5
mL/kg) may be useful as a therapeutic trial.
 An additional bolus is justified only if there is a favorable response to the
first bolus.
 Infants with prolonged shock and acidosis who develop fluid loss from
capillary leak syndrome may require repeated fluid boluses.
 Some infants develop systemic vasodilation after PGE1 and require
titrated isotonic fluid administration (5 mL/kg/dose).
 Conversely, the newborn with an established diagnosis of CoA and CHF
who is responding to PGE1 therapy should receive fluid restriction (70%
to 80% of maintenance requirements) to limit the salt and water load in
the face of heart failure.
 A second scenario involves the newborn who remains hypotensive
despite restoration of ductal patency with PGE1, where poor ventricular
function is the likely cause in this setting, and the infant may benefit
from inotropic support rather than volume expansion.
Indication and timing of surgery of CoA:

1. If CHF or circulatory shock develops early in life, surgery should be

performed on an urgent basis. A short period of medical treatment
improves the patient's condition before surgery.
2. If there is a large associated VSD, which occurs in 17% to 33% of patients
with CoA.

Postoperative management:

Post operative Complications:

1. Postoperative renal failure is the most common cause of death (Park,

2. Post-coarctectomy syndrome, which manifests as abdominal pain
and tenderness as well as hypertension, fever, and leukocytosis 2–3
days after the CoA repair.
Its etiology seems to involve a sudden increase in mesenteric
perfusion after the coarctectomy, resulting in mesenteric necrotizing

3. Spinal cord ischemia leading to paraplegia.

4. Chylothorax (due to thoracic duct injury).
5. Recurrent laryngeal nerve injury, phrenic nerve injury.
6. Recurrent coarctation (15%).
7. Sepsis.
8. Stroke (possibly owing to transiently increased intracranial pressure).
Management of postoperative hypertension:

It is crucial to control the systemic hypertension diligently in the first 24–48 h

postoperatively in order to prevent anastomotic leaks, the post-coarctectomy
syndrome and to minimize bleeding.

 Keep systolic blood pressure below 80–90 mmHg.

I. Pain control and sedation.

II. Continuous infusions of arterial vasodilators (e.g., sodium nitroprusside) are
important in the treatment of severe hypertension in the immediate
postoperative period.
III. Intravenous beta-blockers such as esmolol or labetalol may be necessary.
IV. For persistent hypertension, oral angiotensin-converting enzyme inhibitors
or beta-blockers may be necessary.
V. Once extubated and fed, the infants are transitioned to an angiotensin-
converting enzyme (ACE) inhibitor or beta-blocker for long-term treatment,
especially in the presence of aortic regurgitation .
Aggressive afterload reduction is contraindicated in the presence of
significant residual aortic stenosis.

Differential diagnosis of shock (Wernovsky and Gruber, 2007).

Hypovolemia Relative Vasodilatation.

Third space losses.
Absolute Blood loss.
Intracranial hemorrhage.
Insensible losses.
Cardiogenic Obstructive lesions Aortic stenosis.
Coarctation of Aorta.
Myocardial Hypoplastic left heart.
Hypoxic ischemic injury.
others Cardiac tamponade.
Tension pneumothorax.
Air embolism.
Septic Viral, Bacterial, Fungal

IV. Congestive Heart Failure:

Congestive heart failure (CHF) occurs when the heart can no longer meet
the metabolic demands of the body at normal physiologic venous pressures.

Lesions presenting with heart failure in the first week of life

I. Ductal-dependent abnormalities
1. Hypoplastic left heart syndrome with a large PDA.
2. Truncus arteriosus.
3. Interrupted aortic arch type B (i.e., interruption between the left common
carotid artery and the left subclavian artery).
4. Coarctation of the aorta with a VSD.
5. Critical aortic stenosis.
II. Non-ductal-dependent abnormalities
 Endocardial cushion defect.
 Single ventricle complex.
 Myocardial dysfunction (cardiomyopathy).
 Supraventricular tachycardia/arrhythmia.
CHF is a clinical diagnosis, made on the basis of the existence of certain signs and
symptoms rather than on radiographic or laboratory findings, although these may
be supportive evidence for the diagnosis


o Early:
 Tachypneia and tachycardia with an increased respiratory effort and rales.
 Cardiomegaly.
 Hepatomegaly.
 Delayed capillary refill.
 Weak peripheral pulses.
 Diaphoresis.
 Feeding difficulties.
 Edema is rarely seen.
 Growth failure (Wechsler and Wernovsky, 2008).
o Late: Cyanosis may also occur, making diagnosis more difficult.
o Finally, CHF may present acutely with cardiorespiratory collapse, particularly in
“left-sided” lesions.
o Hydrops fetalis is an extreme form of intrauterine CHF.
Investigations: (Only as a supportive evidence for the diagnosis).
Laboratory Studies

 Complete blood count

 Anemia may cause or aggravate heart failure.
 Electrolytes
 Hyponatremia reflects an expansion of extracellular fluid volume
 Hypokalemia and hypochloremia can be the result of prolonged
administration of diuretics.
 Hyperkalemia can be the result of impaired renal perfusion and marked
reductions in glomerular filtration rate (GFR) or from intracellular
potassium release due to impaired tissue perfusion.
 Renal function tests
 Elevated BUN and BUN/creatinine ratios, in decompensated heart
 Liver function tests (impaired hepatic function)
 Elevation of AST, ALT, lactic dehydrogenase (LDH), and other liver
 Hyperbilirubinemia (both direct and indirect) is related to acute hepatic
venous congestion and is common with severe right heart failure.
 Elevated alkaline phosphatase.
 Prolonged prothrombin time.
Chest Radiography

 Cardiomegaly and alveolar edema with pleural effusions and bilateral infiltrates
in a butterfly pattern.
 Other signs are haziness of hilar shadows, vascular redistribution and
thickening of interlobular septa

 To assess cardiac function and identify potential cardiovascular causes,

particularly anatomic lesions.
 Congestive heart failure itself is not an echocardiographic diagnosis.
The management of congestive heart failure (CHF) is difficult and sometimes
dangerous without knowledge of the underlying cause
Note -Oral Sedation for echocardiography can cause withdrawal of the
endogenous catecholamine drive, that is maintaining tissue perfusion, resulting in
cardiac decompensation
The goals of medical therapy for congestive heart failure include reducing
the preload, enhancing cardiac contractility, reducing the afterload, improving
oxygen delivery, and enhancing nutrition.

Medical management should be tailored to the specific details of each case

Table 7-6. Pharmaceutical agents used in the treatment of congestive heart failure .

Agent Dose Comment

Preload reduction
Furosemide 1 mg/kg/dose PO or IV May increase to qid
Hydrochlorothiazide 2 mg/kg/d PO divided bid May increase to qid
Dopamine See below for dosage.
After load reduction
Captopril 0.1-0.5 mg/kg/d PO divided q8h
Nitroprusside 0.5-10 mcg/kg/min IV May need to monitor
cyanide level
Nitroglycerin 0.1-0.5 mcg/kg/min IV
Alprostadil (PGE-1) 0.03-0.1 mcg/kg/min IV

Table 7-7. Doses and effect of inotrops (Wechsler and Wernovsky, 2008).

Drug Usual dose (µg/kg/min) Effect

Dopamine 1-5 ↑ urine output, ↑ HR (slightly), ↑ contractility

6-10 ↑ HR, ↑ contractility, ↑ BP

11-20 ↑ HR, ↑ contractility, ↑ SVR, ↑ BP

Dobutamine 1-20 ↑ HR (slightly), ↑ contractility, ↓ SVR

Epinephrine 0.05-0.50 ↑ HR, ↑ contractility, ↑ SVR, ↑ BP

HR = heart rate; BP = blood pressure; SVR = systemic vascular resistance; PVR = pulmonary vascular
 A combination of low-dose dopamine (up to 5 µg/kg/minute) and dobutamine
may be used to minimize the potential peripheral vasoconstriction induced by
high doses of dopamine while maximizing the dopaminergic effects on the
renal circulation.
 Care should be taken to repletion of intravascular volume before institution
of vasoactive agents (Wechsler and Wernovsky, 2008).
 Inotropic support must be used judiciously and with caution in the setting of
patients with heart failure (Satou and Halnon, 2009).

Note, Although increased inotropy results in improved cardiac output and blood
pressure, it comes at the expense of increased myocardial oxygen consumption
and demand.

Early and elective use of mechanical circulatory support should be considered

in all patients with severe myocardial dysfunction that is refractory to medical


Loading: "Digitalization" used for acute congestive heart failure.

Table 7-8.DIGOXIN- Total loading and maintenance doses .

Maintenance doses
Loading doses
PMA IV PO Intervals
PMA IV PO weeks mcg/kg mcg/kg (hours)
weeks mcg/kg mcg/kg
< 29 4 5 24
< 29 15 20
30-36 5 6 24
30-36 20 25
37-48 4 5 12
37-48 30 40
> 49 5 6 12
> 49 40 50
Titrate based on clinical response
Divide into 3 doses over 24 hours

Loading: 75 mcg/kg IV over 60 min, immediately followed by

Maintenance infusion: 0.5 to 0.75 mcg/kg per minute.

For pre term< 30 weeks:

Loading: 0.75 mcg/kg per minute for 3 hours, immediately followed by

Maintenance infusion: 0.2 mcg/kg per minute.

Adjunctive therapy:
 Correct acidosis with the administration of fluid and/or bicarbonate.
 Calcium should be administered when hypocalcemia is documented

V.Heart murmurs:
Not all newborns with murmurs have CHD.

Not all newborns with CHD have murmurs.

 A heart murmur may be a presenting sign of a congenital heart defect,

although innocent murmurs are much more frequent than pathologic
 Most pathologic murmurs should be audible during the first month of life, with
the exception of an ASD .
 The time of appearance of a heart murmur depends on the nature of the
 Heart murmurs of stenotic lesions (e.g., AS, PS) and those due to AV
valve regurgitation are audible immediately after birth and persist
because these murmurs are not affected by the level of pulmonary
vascular resistance.
 Heart murmurs of large VSD may not be audible until 1 to 2 weeks of
age, when the pulmonary vascular resistance becomes sufficiently low to
allow shunt to occur.
A newborn with a large ASD may not have a heart murmur.

 The murmur of an ASD appears after a year or two, when the

compliance of the RV improves to allow a significant atrial shunt .
Infants who are in severe CHF may not have a loud murmur until the
myocardial function is improved through anticongestive measures .

Duct dependent lesions: (Where ductus arteriosus should be kept


Why ‘keep duct patent’?

I. To maintain pulmonary blood flow.
II. To maintain systemic blood flow.
III. To obtain a mixture between systemic and pulmonary blood flow if in
parallel circulations.

When to keep duct patent?

(Indications for prostaglandin (PG) initiation)

 In cyanotic infant whom cardiac disease is suspected a low threshold for
starting PG is needed.
 When critical heart disease is present and echocardiography is not immediately
available, prostaglandin infusion can be lifesaving.
If the diagnosis is uncertain a trial of PG for 30-60 minutes with repeated ABG may
be warranted and will usually outweigh the risk of delaying treatment
Figure 7-12. Clinical presentations suggestive of duct dependent lesions,
necessitating starting prosraglandin.

I.Ductal Dependent Systemic Circulation

Presentions: (See "Shock")

 Cardiac failure with systemic hypoperfusion.

 Poor or absent peripheral pulses.
 Increasing metabolic acidosis.
 Cyanosis may not develop until the latter stages of the clinical course.
Lesions: (Critically obstructed systemic circulation)

 Hypoplastic left heart syndrome.

 Critical aortic stenosis.
 Coarctation of the aorta.

1. Maintaining ductal patency using PGE1. See below.

2. Maintaining pulmonary to systemic blood flow through the PDA, by
increasing pulmonary vascular resistance (PVR) and reducing pulmonary to
systemic flow ratio (Qp/Qs).
3. Maintaining adequate tissue perfusion rather than correcting the cyanosis.
Increasing pulmonary vascular resistance (PVR) to reduce pulmonary to systemic
flow ratio (Qp/Qs) is achieved by

 Controlled positive-pressure ventilation with care taken to avoid

hyperventilation can limit pulmonary blood flow.
 Using positive end-expiratory pressure (PEEP); allowing delivery of lung
volumes that exceed functional residual capacity and subsequently
compressing pulmonary vasculature with a resultant increase in PVR.
 Aim for a CO2 of 37 - 45 mmHg.
 SaO2 75 - 85%.
 Supplemental oxygen, a potent pulmonary vasodilator, is avoided
Ventilate in air if possible.
Although patients with respiratory distress syndrome, pneumonia,
atelectesis or other primary lung pathology might benefit from the use of
supplemental oxygen for severe hypoxia.

 Another method is "Medical gas management", supplying hypoxic gas

mixtures, by blending nitrogen and oxygen to achieve inspired sub
atmospheric fiO2 of 0.14 to 0.20. (This method is not yet available at our
Adjunctive Therapies
 Increase the hematocrit to 50%.
 Attenuate sympathetic vascular tone to improve systemic perfusion,
specifically for the stressed neonate with increased SVR, using low-dose
morphine to reduce agitation and work of breathing.
 Avoid enteral feeding during the preoperative period, because of the
potential for systemic hypoperfusion.
 Parenteral nutrition is provided with volume administration that is generally
10% to 20% higher, to account for the capillary leak that is expected with
PGE1 therapy.
 Diuretic therapy is reserved for those patients who demonstrate respiratory
insufficiency from increased interstitial lung edema related to either
pulmonary over circulation or restrictive pulmonary venous return.
Note, The response is judged by peripheral pulsation, urine out put and the
improvement of metabolic acidosis.

The use of vasoactive medications with "duct dependent systemic

blood flow"
 The need for preoperative inotropic support is variable and directed by
presentation and echocardiographic features.
 Patients who present in cardiogenic shock most commonly benefit from
inotropic support.
 Caution should be taken to avoid a dose escalation that results in an
undesired increase in SVR that subsequently raises Qp/Qs.
 For patients with elevated Qp/Qs and systemic perfusion is compromised,
use inodilator therapy with milrinone.
Note, Milrinone, however, also may reduce PVR and again carries the undesired
risk of increasing Qp/Qs and on the other hand, could result in significant
hypotension in patients already at risk for decreased perfusion secondary to
aortopulmonary runoff

II. Ductal Dependent Pulmonary Circulation


 Cyanosis.
 Tachypnea without respiratory distress.
 Adequate perfusion initially.

 Critical pulmonary stenosis (Bowman and Fraser, 2006).

 Pulmonary atresia with intact interventricular septum.
The aim is to maintain systemic to pulmonary blood flow through a patent

This depends on three factors:

1. Ductal resistance (overcomed by prostaglandin). See below.

2. Decreasing PVR to increase pulmonary blood flow.
3. Increasing systemic vascular resistance.
For management details refer to "Cyanosis with decreased pulmonary flow".

III. To obtain a mixture between systemic and pulmonary blood flow

if in parallel circulations
 TGA with intact septum.
For management refer to TGA "Cyanosis with incresed pulmonary flow".

Protaglandin E1 (PGE1) (Alprostadil)

Prostin VR® 500 μg /ml,

Prostavasin® 20 μg /ampoule.

Use:Temporary maintenance of patent ductus arteriosis (PDA), in neonates with

ductal-dependent congenital heart disease.


 The starting dose is 0.05 to 0.1 μg/kg per minute, administered in a

continuous intravenous drip.
 When the desired effects (increased PO2, increased systemic blood
pressure, improved pH) are achieved, the dose should be reduced step by
step to 0.01 μg/kg per minute.
 When the initial starting dose has no effect, it may be increased up to 0.4
μg/kg per minute.
 Dilute with dextrose or normal saline (NS).
 Recommended concentration is 10 µg/mL.
 Maximal drug effect usually seen within 30 minutes in cyanotic lesion and
may take several hours in acyanotic lesions .
Special considerations:

 Dilute before administration to a concentration less than 20 μg /ml.

 Fresh infusion every 24 hours
Complications of PGE1:

 Apnea (12%) is the most common complication and is due to a direct effect
of PGE1 on the CNS.
The vast majority of infants who require PGE1 will also need assisted
ventilation, either for the severity of their CHD or because of apnea.

Do not start PGE1 unless:

(a)Infant is intubated and receiving assisted ventilation or

(b)You are prepared to intubate the infant and start assisted ventilation
immediately after PGE1 has been started.
 Hypotension, due to vasodilatation of peripheral circulation and decreased
cardiac output, occurs most commonly at higher doses.
 Fever (14%) which makes evaluation for infection difficult.
 Irritability, abnormal EEG and seizures due to a direct CNS effect and more
common at higher doses.
 Diarrhea. Although this may occur, infants who require PGE1 for several
days may be tried on enteral feedings, if their condition otherwise permits.
 Extravasation may cause tissue sloughing ad necrosis.
 Longer term effects include hypertrophy of gastric mucosa mimicking
congenital pyloric stenosis and periosteitis