Approach to Paediatric

Heart Diseases
dr. Liku Satriani, SpA(K)
Circulatory Changes at birth
The LA pressure is low
The RA pressure is higher than
LA ( receives all sys VR)
The flap valve of foramen ovale
is held open
Blood across the atrial septum to
LA
With the 1st breath, resistance
to pulm BF falls and volume of
blood returning to RA falls
Changes in pressure difference
cause flap valve of FO to be
closed.
The ductus arteriosus will
normally close within first
few hrs or days
Some babies with CH
lesion rely on BF through
the duct.
! Clinicalcondition
deteriorate dramatically
when duct closed (1-2
days)
 Epidemiology
Epidemiology
Congenital heart disease occurs in 0.50.8% of live births.
Incidence is higher in stillborns (34%), spontaneous abortuses
(1025%) and premature infants (about 2% excluding patent
ductus arteriosus [PDA])
About 23 in 1,000 newborn infants will be symptomatic with
heart disease in the 1st year of life.
The diagnosis is established by 1 week of age in 4050% of
patients with congenital heart disease and by 1 month of age in
5060% of patients.
Etiology
Etiology
Caused by developmental abnormalities
Genetic factors
chromosomal abnormalities
outflow tract defect may cause by abnormal development of neural crest-
derived cell (region of chromosome 22)
Environmental factors, such as congenital infection or teratogens
Multifactorial: genetic, environmental, and maternal factors
The risk of recurrence of congenital heart disease increases if a
1st-degree relative (parent or sibling) is affected.
 Chromosome
Abnormalities
Chromosome Abnormalities
Down Syndrome (40%) AVSD, VSD
Trisomy 13 (80%) VSD, PDA
Trisomy 18 (100%) VSD, PDA
Turners Syndrome (35%) Coarctation of aorta
Marfans Syndrome Aortic aneurysm, MVP
 Teratogen
Teratogen
Infection
Rubella PDA, peripheral PS
Drug
Dilantin PS, AS
Lithium Ebstein anomaly
Alcohol VSD, ASD
Retinoic acid Interrupted aortic arch, TGA, TOF
Maternal disease
Diabetes TGA, VSD, cardiomyopathy
SLE Complete AV block
 Frequency
Frequencies of CHD CHD
VSD 42 %
ASD 10 %
Pulmonary stenosis 8%
PDA 7%
TOF 5%
AVSD 5%
How to Approach Congenital Heart Disease
 History
History Taking Taking
Maternal and Perinatal History
History of drug used (Teratogen)
Birth weight
Family History
Usually prevalence 8 in 1,000
1st degree related increased in prevalence during 10 15 percent
 History
History Taking
Taking
History of cardiology related
Heart Failure
Usually present in first year old
Tachypnea, Prolong feeding, Poor weight gain, Diaphoresis, pale
(sympathetic activity increased), Exercise intolerance
Cyanosis
Hypoxic Spell
Cyanosis, Tachypnea, Flaccid or loss of consciousness
Arrhythmia
Most common -> Supraventricular tachycardia
Neurologic Symptoms
Cerebral thrombosis, Brain Abscess
 Physical Examination
General Appearance

Finding dysmorphic feature

Vital Signs

Blood pressure > Leg > Arm SBP 10-20 mmHg

Pulse

Weak Leg > Arm > Coartation of Aorta

Diminished pulse > Cariogenic shock or low cardiac output

Respiratory rate (Upper limit)

< 1 month 60 /min

1 month - 1 year 50 /min

1 year - 5 years 40 /min

> 5 years 30 /min

Physical Examination
Jugular Venous Pressure

Suspected right-side heart failure

Cardiac Exam

Inspection > Precordial bulging (RV overload)

Palpation

Apex : 4th intercostal space midclavicular line

Thrill for grading murmur

Auscultation
Physical Examination

Pulmonary Exam

Crepitation or Rales > Pulmonary congestion

Complication : Pneumonia > L-R shunt

Extremities

Edema > Systemic venous congestion >

Right-side heart failure

Clubbing of finger > Cyanotic heart

 Chest X-Ray
Cardiovascular Structure

Location of Heart, Stomach and Liver

Levocardia, Mesocardia, Dextrocardia (Left, Central, Right)

Chamber and Heart Enlargement

Cardiothoracic Ratio 50-60%

Identified what chamber enlargement

Aorta

Right or Left Aortic Arch

Size of Aorta
 Chest X-Ray
Pulmonary Vasculature

Increased pulmonary vasculature (L-R shunt)

Decreased pulmonary vasculature (R-L shunt)

Normal pulmonary vasculature (PS,AS)

Extra cardiac Structure

Ribs and Vertebra

Diaphragm

Phrenic nerve paralysis, Diaphragmatic hernia

Lung parenchyma

Pneumonia or Atelectasis
Electrocardiography
Electrocardiography
Ventricular Hypertrophy (Right or Left)
Axis
Right Ventricular hypertrophy Left Ventricular Hypertrophy
R wave >98th percentile in lead V1 R wave >98th percentile in lead V6
S wave >98th percentile in lead V6 S wave >98th percentile in lead V1
R wave in V1 + S wave in V6 >98th percentile R wave in V6 + S wave in V1 >98th percentile
R/S ratio >98 percentile in lead V1 Q wave >98th percentile in lead lll or V6
Right axis deviation R/S ratio >98th percentile in lead V6
qR pattern in V1 LVH (by voltage criteria) with strain pattern
Upright T wave in V1 (1 week old to 8 years old)
RSR pattern in lead V1, where R >15 mm (<1
year old) or R >10 mm (>1 year old)
Pure R wave in V1 >10 mm (newborn)
RVH (by voltage criteria) with strain pattern
Congenital heart disease
Cyanotic / Acyanotic
Three major categories:
left-to-right shunt
right-to-left shunt
obstruction
A shunt is an abnormal communication between chambers or
blood vessels
Direction and magnitude of shunt depends on
Size of defect
Relative pulmonary and systemic pressures
Relative pulmonary and systemic resistance
 How to Approach Congenital Heart Disease
Congenital heart
disease

Cyanosis Acyanosis

Decrease pulmonary Increase pulmonary

blood flow blood flow

Increase pulmonary Normal pulmonary

blood flow blood flow
Acyanotic Congenital Heart
Disease
 Acyanosis

Increase pulmonary Normal pulmonary

blood flow blood flow

RVH LVH RVH LVH

ASD VSD PS AS
PAPVR PDA MS CoA
AVSD
 Acyanotic Heart
Disease
Acyanotic Heart Disease
Left to Right Shunt
Increased pulmonary blood flow
Clinical -> Heart Failure
VSD, ASD, PDA, AVSD
Obstructive Lesions
Normal pulmonary blood flow
Clinical -> Exercise intolerance
PS, AS, Coarctation of aorta
LR Shunts (Acyanotic CHD)
Defects
1. VSD
2. PDA
3. ASD
4. AVSD (or complete atrioventricular canal)
May not be apparent in neonate due to
high PVR (ie- bidirectional shunt)
LR Shunts General Points
PDA & VSD
Presents in infancy w/
heart failure, murmur,
and poor growth
Left heart enlargement
(LHE)
Transmits flow and
pressure
AVSD can present as either depending on size of ASD & VSD component
ASD
Presents in childhood w/
murmur or exercise
intolerance (AVSD or 1o ASD
presents earlier)
Right heart enlargement
(RHE)
Transmits flow only
 Increased PBF

Left Heart Right Heart

Overload Overload
 Pulm vasc
markings
equal in
upper and
lower zones

Cardiomegaly
Eisenmengers Syndrome
A long standing LR shunt will eventually
cause irreversible pulmonary vascular disease
This occurs sooner in unrepaired VSDs and
PDAs (vs an ASD) because of the high
pressure
Once the PVR gets very high the shunt
reverses (ie- now RL) and the patient
becomes cyanotic
Cyanotic Congenital Heart
Disease
 Cyanosis

Decrease pulmonary Increase pulmonary

blood flow blood flow

RVH LVH RVH CVH

TOF PA/IVS TGA

Truncus
PA/VSD Tricuspid TAPVR
arteriosus
Critical PS atresia DORV
DORV/PS HLHS
TGA/VSD/PS
 Cyanotic Heart
Disease
Acyanotic Heart Disease
Decreased pulmonary blood flow
Right ventricular outflow tract obstruction ->
righttoleft shunt
Increased pulmonary blood flow
Can be developed to congestive heart failure and cyanosis
RL Shunts (CCHD)
Blue blood bypasses the lungs
Degree of cyanosis varies
Classify based on pulmonary blood flow (PBF)

PBF PBF
Truncus arteriosus Tetralogy of Fallot
Total anomalous pulm. Tricuspid atresia
venous return (TAPVR) Ebsteins anomaly
Transposition of the great
arteries (TGA)

Please note: This is a generalization. In reality most of these defects can present with low or
high PBF (eg- ToF with little PS acts more like a VSD with high PBF)
 RL Shunts
PBF
Presents more often with
heart failure (except
TGA)
Pulmonary congestion
worsens as neonatal
PVR lowers
Sats can be 93-94% if
there is high PBF
There is unimpeded PBF; Equal
thus, extreme pulmonary pressures here
overcirculation. too
 RL Shunts
PBF
Presents more often
with cyanosis
See oligemic lung fields
Closure of PDA may
worsen cyanosis

Dynamic subvalvular
Why are
obstruction here causes
pressures equal?
Tet spells
 Pulmonary
overcirculation Too little
90%
70% PBF

70% 99% 60% 99%

99%

99%
70% 60%
Different amounts of PBF
(Truncus vs ToF)
Obstructive Lesions
Ductal Dependent Non-Ductal Dependent
1. Critical PS/AS 1. Mild-moderate AS
2. Critical CoA/IAA 2. Mild-moderate CoA
3. HLHS 3. Mild-moderate PS
Presents in CV shock at Presents in older child
2-3 days of age when w/ murmur, exercise
PDA closes intolerance, or HTN (in
+/- cyanosis CoA)
Needs PGE1 Not cyanotic
Ductal-Dependent
Lesion
Without a PDA there is no
blood flow to the abdomen
and lower extremities.

(Blue blood is better than no blood.)

Acquired Heart Disease
Kawasaki Disease (KD)
Now the #1 cause of acquired heart disease
A systemic vasculitis (etiology-unknown)
Tests CBC, CMP, CRP, ESR, EKG, ECHO
Rx IVIG at 2g/kg and high-dose ASA
Prognosis Coronary artery dilatation in
15-25% w/o IVIG and 4% w/ IVIG (if given
within 10 days of fever onset). Risk of
coronary thrombosis.
Kawasaki Clinical criteria
Fever for at least 5 days AND 4 of the following
5 criteria:
Eyes - conjunctival injection (ie- no exudate)
Lips & mouth - erythema, cracked lips, strawberry tongue
Hands & feet - edema and/or erythema
Skin - polymorphous exanthem (ie- any rash)
Unilateral, cervical lymphadenopathy
Rheumatic Fever
A post-infectious connective tissue disease
Follows GAS pharyngitis by 3 weeks (vs. nephritogenic
strains of GAS)
Injury by GAS antibodies cross-reacting with tissue
Dx JONES criteria (major and minor)
Tests Throat Cx, ASO titer, CRP, ESR, EKG, +/- ECHO
Rx PCN x10 days and high-dose ASA or steroids
2o Prophylaxis daily po PCN or monthly IM PCN
Rheumatic Fever organs
affected
1. Heart muscle & valves myocarditis &
endocarditis (pericarditis rare w/o the others)
2. Joints polyarthritis
3. Brain Sydenhams Chorea (milkmaids grip or
better yet, motor impersistance)
4. Skin erythema marginatum (serpiginous border) due
to vasculitis
5. Subcutaneous nodules non-tender, mobile and on
extensor surfaces
Thank You