APPROACH TO CHD

INTRODUCTION
Congenital cardiovascular defects, also known as congenital heart
defects, are structural problems that arise from abnormal formation of

the heart or major blood vessels. Defects range in severity from tiny

pinholes between chambers that may resolve spontaneously to major

malformations that can require multiple surgical procedures

before school age and may result in death in utero, in infancy, or in

childhood.

AMERICAN HEART ASSOCIATION

 INCIDENCE
 Approximately 5 to 8 per 1000 live births .

 About 2 or 3 in 1000 infants will be symptomatic

during the first year of life with significant heart

diseases that will require treatment.

 CHD is the major cause of death in the first year of life.

 There are more than 35 well recognized cardiac defects

the most common heart anomaly is VSD.

Etiology
 Genetic 90%
 Environmental 10%
Predisposing factors radiation exposure,
infections,systemic maternal disease
The systematic approach to CHD includes:-
1.Detailed antenatal and postnatal history.
2.Systematic physical examination
3. Chest X-ray
4. Electrocardiography (ECG)
5. Echocardiography
6.Computed tomography (CT) angiogram and cardiac
magnetic resonance imaging (MRI) in selected cases
7.Invasive procedures like cardiac catheterization and
angiocardiography, if needed.
 History & Symptomatology
Mother’s marriage? Breathlessness?

Consanguinity? Exertional Syncope?

Age of Child Birth? Cough with sputum?

Antenatal History? Bluish discoloration?

Natal History? History of squatting?

Postnatal events? Cyanotic spells?

Physical development? Pedal edema?

6
 Family History
 If one parent has a congenital heart anomaly, the risk
of the child having one – 10 percent.
 When a first cousin has a congenital heart anomaly,
the risk of a sibling having one is approximately 2
percent.
 With no family history of CHD, if the firstborn has a
congenital heart lesion, the risk of a second child
having a congenital heart lesion is 2 to 3 percent.
Maternal History
 BIRTH WEIGHT
 HIGH BIRTH WEIGHT – TGA
 LOW BIRTH WEIGHT – Intra uterine
infections,drugs( alcohol)
 Does the child have Heart
Disease?
NADAS Criteria
MINOR
MAJOR
1. Systolic murmur Gr. II(soft all)
1. Systolic murmur Gr. III
2. Abnormal 2nd Sound
or > intensity
3. Abnormal ECG
2. Diastolic murmur
4. Abnormal Chest X Ray
3. Cyanosis 5. Abnormal Blood
4. CHF Pressure

1 major or 2 minor
Acyanotic congenital heart diseases - present
with signs of congestive heart failure and/or heart
murmurs that are heard during physical
examination and can manifest any time during
infancy or early childhood.
Most of these patients present during the first 6
months of life.
PRESENTING COMPLAINTS
1.Feeding difficulties
2. Tachypnea
3. Sweating
4. Subcostal recession
5. Recurrent respiratory infections
6. Growth impairment in the infant
7. Exercise intolerance
8. Easy fatigability or murmur in the older child
The magnitude of the shunt or the severity of the
obstruction determines the clinical presentation and
symptoms.
 FEEDING DIFFICULTIES
 It is a common symptom in significant acyanotic
CHDs.
 In infants, feeding itself is a form of exercise or effort.
 An infant with heart failure has the inability to
complete feeds within 15 to 20 minutes, sucks less
volume of milk (< 3.5 ounces per feeding), gets tired
easily and takes frequent feeds (less than 2 hours).
 As the feeds are inadequate they become irritable
and cry excessively. This suck-rest-suck cycle
continues round the clock.
 RESPIRATORY DISTRESS
 It is the most prominent sign of heart failure caused by
significant left to right shunting in infancy.
 Symptoms of respiratory distress include tachypnea or
intercostal and subcostal chest retractions.
Easy Fatigability
 In an infant, it is seen as poor ability to suck and
feed.
 In older children, heart failure may be manifested
as exercise intolerance; difficulty in keeping up
with peers during sports or need for a nap after
coming home from school and poor growth.
Repeated Lower Respiratory
infections
 Recurrent pneumonia - two or more episodes of
RTI in one year or more than three in any time
frame requiring hospitalization or IV antibiotics.
 Frequent upper respiratory tract infection are
not related to CHD.
Growth Retardation or Failure
to Thrive
 Failure to thrive is defined as weight <3rd
percentile for age.
 A normal infant can grow while receiving 100
calories/kg/day; infants whose growth is impaired
by heart disease typically require 130 to 140
calories/kg/day
 GENERAL APPEARANCE
 A wide spectrum of
extracardiac malformations
occur in 15-45% of cases
with CHD.
 Extracardiac malformations
can give a clue towards
certain CHD.
 INSPECTION
 Precordial bulge – suggests chronic cardiac enlargement
 The upper sternum may bulge in children with a large left-to-
right shunt and pulmonary hypertension or with elevated
pulmonary venous pressure.
 Hyper active precordium – heart diseases with volume overload
– large left to right shunts , AV valve regurgitation.
 In patients with PDA,aortic stenosis and CoA, suprasternal
pulsations can be visible.
 SYMPTOMS
CCHD with ↓ PBF CCHD with ↑ PB Flow
 Exertional dyspnoea  Respiratory symptoms
 Cyanosis, spells, seizures predominate wDRecurrent
 CNS complications LRTI/Pneumonias
 No recurrent RTI/  Features of heart failure
no diaphoresis  Sick, dyspnoeic patient
 No breathlessness at rest  Diaphoresis/ breathlessness at
except in extremes rest
anaemia  lesser degrees ofcyanosis.
CYANOSIS
 Cyanosis abnormal bluish discoloration of the skin and
mucus membranes due to an absolute increased
concentration of deoxygenated hemoglobin in the
capillary bed.
 Christen Lundsgaard (1923)actually quantified the
amount of deoxy Hb (4-6 grams %or more )that was
required to produce that bluish discoloration that produces
the clinical finding of cyanosis.
 Appearence of Cyanosis - depends on dermal thickness,
cutaneous pigmentation, and state of the cutaneous
capillaries.

 Cyanosis is best appreciated in areas of the body where the

overlying epidermis is thin and the blood vessel supply
abundant - Lips, malar prominences (nose and cheeks),
ears, and oral mucous membranes (buccal, sublingual).
.
CENTRAL PERIPHERAL
CYANOSIS history????
 onset –at birth / several days after birth
 Severity of cyanosis
 Permanent /paroxysmal nature
 Parts of body that is cyanotic
 Cyanotic spells – time of appearance , duration of
spell, frequency of spell
 Should be differentiate from breath holding spell
(provocative>crying>cryng stops@ full
expirtion>apnic/cyanotic)
rare <6 months mc 2-5 ys abate after 5 yr
 History of squatting.
Causes of cyanosis
NEUROLOGICAL PULMONARY CARDIAC

Shallow irregular Tachypnea and Tachypnea

respiration respiratory distresswith
retraction and grunting
Poor muscle tone Crackles and decreased Doesnot responds to
breath sounds oxygen

Cyanosis disappear on Cyanosis disappear on Absent crackles unless

crying and with oxygen crying and with oxygen CHF supervenes

CXR- etiology
Pulse oximetry
 Standard of care for all infants with respiratory distress
& cyanosis

 Accurate& reliable method of monitoring o2 saturation

in infants noninvasively

 Pulse oximeter probes on R hand & lower extremity

 HYPEROXIA TEST
 To Determine Intrapulmonary vs. Intracardiac Shunt.
 On room air (if tolerated) -measure pO2 by blood gas
 On 100% FIO2 - blow-by mask, ETT-repeat measurement of Po2
 Oxygen should be administered for at least 10 min to fill the
alveolar space completely with O2
 pO 2 < 100; cyanotic CHD likely
 pO 2 100-150; cyanotic CHD possible
 pO 2 > 150; cyanotic CHD unlikely
 DANGERS- Pulmonary overcirculation Closing the PDA
 Apex Beat
 In infants and children below 4 years, the apex beat is
located in the fourth intercostal space (ICS).
 Between 4 and 7 years it can be either in the fourth or
fifth ICS and thereafter in the fifth ICS.
 The displacement of the apex beat laterally or inferiorly
indicates cardiac enlargement.
 A hyperdynamic apical impulse is seen in volume
overload conditions like post-tricuspid shunts
 A sustained heaving apical impulse is seen in pressure
overload conditions like in LVOTO.
 Thrill
 Thrills at lower sternal border are more likely to be
associated wd VSD than mitral or tricuspid regurgitation
 Thrills at the right upper sternal border (RUSB) or
suprasternal notch are most likely to be due to severe
aortic stenosis
 Auscultation
 loud S1 can occur with increased flow across the
AV valves from large left to right shunts, such as
ASD,VSD,PDA.
 Normal splitting: - small VSD, mild aortic or
pulmonic stenosis
 Wide splitting-
 Volume overload (e.g., ASD, PAPVR)
 Pressure overload (e.g., PS)
 Electrical delay (e.g., RBBB)
 Early aortic closure (e.g., MR)
Paradoxical splitting or reversed splitting – PDA,
severe aortic stenosis, complete left bundle branch
block
Single S2
 Pulmonary hypertension
 One semilunar valve (e.g., pulmonary atresia, aortic
atresia, persistent truncus arteriosus)
 Severe AS
Abdominal Examination-
 Normally liver is palpable (2–3 cm below costal margin) at
midclavicular line up to 4 to 5 years of the age, after that it
remains palpable up to 1 cm till late childhood. If liver is
palpable further downwards and the infant is irritable it
probably indicates presence of congestive heart failure.
 If the liver is in midline and palpable symmetrically, it indicates
cardiac malpositions and underlying complex heart disease.
 Spleen is not normally palpable;if palpable it indicates
possibility of infective endocarditis.
Respiratory System Examination
 Unlike adults, where crepitations is commonly basal, in neonates
and infants it is more diffusely heard
 PULSE
Character of the pulse –
 Bounding pulses – PDA , large systemic AV Fistulas ,
persistent truncus arteriosus.
 Pulsus parvus et tardus- severe AS.
 Weak ,thready pulse – cardiac failure /circulatory shock
,myocarditis, cardiomyopathy, legs in patients with CO A.
•Difference between upper and lower limb pulses – CO A.
•Discrepancy in both radial pulses - supravalvular aortic
stenosis, aortic isthmus stenosis or aortoarteritis with
obstruction to left subclavian artery.
 BLOOD PRESSURE
 Upper extremity hypertension – first sign of CoA.
 A narrow pulse pressure - low cardiac output or
severe aortic stenosis.
 Pulse pressure widens in conditions with an elevated
cardiac output (anemia and anxiety) or with
abnormal runoff of blood from the aorta during
diastole (PDA or aortic insufficiency).
 CXRay
Feature of increased PBF
 Visibility of pulmonary vascularity in lateral 1/3rd
 Hilar haze in lateral views.
 Ratio between The end on vessel diameter to that of
accompanying Bronchus Normally it is 1.2:1 and in plethora it
is ≥1.5:1.
PULMONARY VENOUS CONGESTION
 Pulmonary venous congestion is characterized by
a hazy and indistinct margin of the pulmonary
vasculature.
 This is caused by pulmonary venous
hypertension secondary to left ventricular failure
or obstruction to pulmonary venous drainage
(e.g., mitral stenosis, TAPVR, cor triatriatum).
NORMAL PULMONARY VASCULATURE
 Pulmonary vascularity is normal in patients with
obstructive lesions such as pulmonary stenosis or
aortic stenosis.
 Patients with small left-to-right shunt lesions also
show normal pulmonary vascular markings.
 VSD
 SMALL VSD - N , mild dilatation of pulmonary trunk
 MOD VSD - cardiomegaly , enlarged LA – SPLAYING OF
CARINA., increased PBF features
 LARGE VSD – cradiomegaly , globular silhoutte- LA,LV,RV
,occ RA dilatation

.
 PATENT DUCTUS ARTERIOSUS
 Enlargement of the left heart
chambers.
 Enlargement of the ascending
aorta or aortic arch.
 Pulmonary plethora with enlarged
central and peripheral artery.
 Filling up of the angle between the
aortic arch and the pulmonary
artery : Most specific sign.
 CO A
 In a heavily exposed film, the precoarctationand postcoarctation
dilatation of the aorta may be seen as a “figure of 3.”
 Rib notching is a specific finding of COA in an older child (usually
older than 5 years) and is usually found between the fourth
and eighth ribs.
DDS FOR RIB NOTCHING
 Chronic superior venacava obstruction
 Chronic inferior venacava obstruction
 Aortoarteritis
 Intercostal AV malformations
TGA
TAPVC
REFRENCES
 GHAI TEXTBOOK OF PEADIATRICS
 PARK’S CARDIOLOGY
 MOSS ADAM’S CARDIOLOGY
 Nelson textbook of pediatrics
THANK YOU
 SCIMITAR SIGN

• The scimitar sign is produced by

an anomalous pulmonary vein
that drains any or all of the lobes
of the right lung.
 Third Heart Sound

 The S3 - low-frequency sound in early diastole and

is related to rapid filling of the ventricle .
 It is best heard at the apex or lower left sternal
border. It is commonly heard in normal children and
young adults.
 A loud S3 is abnormal and is audible in conditions
with dilated ventricles and decreased ventricular
compliance (e.g., large-shunt VSD or CHF).
 When tachycardia is present, it forms a “Kentucky”
gallop.
Fourth Heart Sound or Atrial Sound
 The S4 is a relatively low-frequency sound of late
diastole (i.e., presystole) and is rare in infants and
children.
 When present, it is always pathologic and is seen
in conditions with decreased ventricular
compliance or CHF.
 With tachycardia, it forms a “Tennessee” gallop.
Murmur
ECG
 APPROACH
ACYANOTIC congenital heart
disease>

Step -1-see which chamber is enlarged

Step -2-suppose it is RV

Step-3-is it volume overload(rsr’/rsR’)or

pressure overload(monophasic R/qR)

Step-4-
• Volume overload-ASD
• Pressure overload-PS, Infantile
coarctation,cor triatriatum & congenital MS-
Step-2-suppose it is LV

Step-3-is it LVH alone/BVH

Step-4-LVH alone-

Step-5-volume/pressure

Step-6-
• LV Volume overload-Moderately restrictive VSD,
PDA
• LV Pressure overload-coarctation of
aorta/congenital AS/interrupted aortic
arch/critical PS of infancy
Step-4-BVH-nonrestrictive VSD,
large PDA ,L-TGA

Step -5- q in lateral leads/v1

Step -6- q in v1,absent in lateral

leads-L TGA

q in lateral leads-simple VSD,PDA

 CROCHETAGE
In inferior leads, a notch near the apex of the R
wave,coined “crochetage,” has been correlated with ASD
Seen in 73.1%, specificity - 92% (all 3 inf
leads),86.1%(atleast 1 inf lead)
 ASD
 increased PBF
 right atrail enlargement
 LAE – rare , seen in older adults with AF , associated
MS
 RVH – acute angle at apex
 LVH – if MR is present in OP- ASD.
 SV –ASD – localized dilatation of SVC at junction with
RA.
There is bilateral increased pulmonary vascularity and prominence of
the atrial appendage. The heart size is within normal limits. No rib or soft
tissue abnormality.
Differential cyanosis - Pink fingers and blue toes
 PDA with reversal of shunt
 Coarctation of aorta with PDA and reversal of shunt
 Interruption of Aortic arch with PDA and reversal of shunt

Reverse differential cyanosis

 Upper part of the body remains cyanotic while the
lower part of the body remains pink.
 TGAwith PAH and shunt through PDA