HISTORY & PHYSICAL EXAMINATION

Evaluation Of The Infant COMPREHENSIVE HISTORY

or Child with ⋅Perinatal Period
Congenital Heart Disease ⋅Infancy
Maria Dolores B. Victor, MD., FPCC ⋅Older Child

Selected Aspects of History Taking: Selected Aspects of History Taking:

 Postnatal History
Heart murmur
 Gestational and Natal History Chest pain
Infections, medications, excessive smoking or alcohol Joint symptoms
intake during pregnancy Neurologic symptoms
Birth weight Medications

 Postnatal (or Past ) History  Family History

Hereditary diseases
Weight gain, development, and feeding pattern CHD
Cyanosis, cyanotic spells, and squatting Rheumatic fever
Tachypnea, dyspnea, puffy eyelids Sudden unexpected death
Frequency of respiratory infection DM, HPN, arteriosclerotic heart disease
Exercise intolerance

•HISTORY & PHYSICAL EXAMINATION

Physical Examination:

CLINICAL PRESENTATION Inspection:

a. Cyanosis General Appearance and nutritional state
b. Heart Failure
-feeding difficulties
-is the child in distress, well nourished or
-poor growth undernourished
-exercise intolerance
-respiratory distress
Chromosomal Abnormalities
c. Chest Pain
d. Extracardiac Congenital Malformation -obvious chromosomal abnormalities known to
e. Early Coronary Disease be associated with certain CHD should be
noted

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 Physical Examination: Physical Examination:

Color
- cyanotic, pale, or jaundiced
- arterial saturation is usually 85 % or lower
before cyanosis is detectable
- Central cyanosis
- peripheral cyanosis
- differential cyanosis manifested by blue
lower extremities and pink upper
extremities
Clubbing
- long-standing desaturation usually longer
than 6 months
- appears earliest and most noticeably in
the thumb
Respiratory rate, dyspnea, retraction
-tachypnea, along with tacchycardia is the
earliest sign of left-sided heart failure

Physical Examination Physical Examination

Palpation:
Peripheral pulses
- pulse rate and note any irregularities
- the right and left arm and an arm and a
leg should be compared.
• Weak leg pulses and strong arm pulses
suggests COA
• Bounding pulses are seen PDA, AR
• Weak pulses are found in cardiac failure
or circulatory shock
Chest
-Apical impulse
-Hyperactive precordium
-Substernal thrust: RV enlargement
-Apical heave:lv hypertrophy
-Thrills: palpable equivalent of murmurs

Physical Examination
AUSCULTATION
Heart Sounds
BP measurement
- The width of cuff should be 40 – 50% of the S1 S3
circumference of the arm
- Cuffs that are too narrow overestimate the S2 S4
true BP, cuffs that are too wide under
estimate the true pressure S2- physiologic split vs widely split
- The pressure in the leg is about 10 mmhg
higher than in the arms

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 AUSCULTATION CLINICAL PRESENTATION
 Ejection Clicks
 CYANOSIS
 Murmurs- described as to:
a. Intensity Grades I-VI (systolic)
Grades I-IV (diastolic)  MURMUR
b. Timing- Pansystolic
Diastolic  HEART FAILURE
Continuous
c. Location
d. Radiation
e. Pitch

 Friction Rubs

LABORATORY EVALUATION LABORATORY EXAMINATION

 Hematologic tests I. HEMATOLOGIC DATA

 Arterial Blood Gas/ Pulse Oximetry Hematocrit ⋅anemia
 Chest Roentgenogram ⋅Polycythemia (HCT >65 vol%)

 Electrocardiography Blood culture

 Echocardiography Serum electrolytes
Acute Phase Reactants
 Stress testing
ASO titer
 ± Cardiac Cath and Angiography
Arterial Blood Gases

LABORATORY EXAMINATION LABORATORY EXAMINATION

Il. RADIOLOGIC ASSESSMENT
Chest Roentgenogram
Postero-Anterior views
Lateral
⋅Cardiac size and shape and location of apex
⋅Pulmonary vascular markings
⋅Associated lung and thoracic anomalies
CXR:
1. Boot-shaped heart – tetralogy of fallot
2. Egg-shaped heart – transposition of
great arteries
3. Snowman sign – total anomalous
pulmonary venous return

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 LABORATORY EXAMINATION LABORATORY EXAMINATION

IV. ECHOCARDIOGRAPHY
IIl. ELECTROCARDIOGRAPHY
⋅Rate and rhythm
⋅Chamber hypertrophy/ enlargement
⋅ST- T wave changes
⋅Cardiac structure
⋅Intracardiac pressures & gradients
⋅Cardiac contractile function
⋅Direction of flow
⋅Coronary arteries
⋅Vegetations, Pericardial fluid,
Tumors, Thrombi
⋅Pericardiocentesis, Balloon atrial septostomy
(BAS), Endocardial biopsy

Echo Techniques LABORATORY EXAMINATION

1. M-mode
V. EXERCISE TESTING
2. Trans-Esophageal Echo (TEE)
⋅Abnormalities in C-V performance
3. 2-dimensional Echo
⋅Symptoms
4. Fetal echo
⋅Severity of cardiac abnormalities
5. Doppler echo
⋅Assist in management

LABORATORY EXAMINATION LABORATORY EXAMINATION

VI. MRI, ELECTRON BEAM CT VII. CARDIAC CATHETERIZATION and

ANGIOCARDIOGRAPHY
and RADIONUCLIDE STUDIES
⋅Presurgical evaluation of cardiac anatomy and
shunt size
⋅Evaluate pulmonary vascular resistance
⋅Follow up after surgical repair or palliation of
complex CHD
⋅Myocardial biopsy
⋅Interventional cardiac catheterization
⋅electrophysiologic study or transcatheter ablation

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CONGENITAL HEART
DISEASE
Relative Frequency of Major CHDs
LESIONS
VSD
ASD 2º, PDA
Coarctation of Aorta, PS
Tetralogy of Fallot
d-Transposition of Great Arteries
Aortic Stenosis
TAPVR, Tricuspid Atresia,
Single Ventricle, Double Outlet RV
Etiology:
• The cause of most CHD is unknown
• Genetic factors- Marfans,Noonans,
conotruncal defects (
TOF,DORV,TA,PVA)
• Chrosomal abnormality- trisomy 18( 90%),
trisomy 21 ( 50 % )
• Environmental or adverse maternal
conditions- DM, SLE, congenital rubella
• Drugs- lithium, thalidomide,ethanol
• Gender differences
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EPIDEMIOLOGY & GENETIC BASIS
Prevalence: 0.5-0.8% of live births
2-3/1000 neonates : symptomatic with
heart diseases in 1st year of life
Diagnosis established in:
40-50% by 1 week old
50-60% by 1 month old
ETIOLOGY
% OF ALL UNKNOWN in most CHDs
LESIONS
25-30 Multifactorial (genetic & environmental)
6-8
Chromosomal abnormalities
Trisomy 21, 13 & 18
5-7 Turner Syndrome
3-5 2-4% - associated with known
4-7 environmental or adverse maternal
1-2 conditions and teratogenic influences
GENETIC COUNSELLING
• Probability of cardiac malformation
occurring in subsequent children
• A child with CHD ? 2-6% incidence of
CHD for a 2nd pregnancy
• 2 First-degree relatives with CHD, risk for
a subsequent child may reach 20-30%;
CHD similar to 1st child
 Fetal circulation
• Differs from adult circulation in several
ways:
1.In adults, gas exchange occurs in the
lungs.
In the fetus, the placenta provides the
exchange of gases and nutrients
2.Parallel circulation
3.3 unique features: ductus venosus,
foramen ovale, ductus arteriosus

Changes in Circulation after Birth

1. Interruption of the umbilical cords results in:
a) An increase in systemic vascular resistance
b) Closure of ductus venosus
2. Lung expansion results in:
a) Reduction of pulmonary vascular resistance
increase in pulmonary blood flow, fall in PA
pressure
b) Functional closure in foramen ovale
-closed by the 3rd mo of life
c) Closure of Patent Ductus Arteriosus

II. Chest Radiograph Findings

⋅Pulmonary vascular markings or
pulmonary blood flow
a. Increased
b. Normal
c. Decreased

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 ACYANOTIC CONGENITAL HEART
SYSTEMATIC APPROACH
LESIONS
III. Electrocardiogram
a. Right Ventricular A. Lesions Resulting in Increased
b. Left Ventricular Hypertrophy Volume Load
c. Biventricular
B. Lesions Resulting in Increased
IV. Echocardiography Pressure Work

A. LESIONS RESULTING IN INCREASED VOLUME LOAD A. LESIONS RESULTING IN INCREASED VOLUME LOAD

1. LEFT-to-RIGHT SHUNTING PATHOPHYSIOLOGY

⋅ASD Communication between the systemic or
⋅VSD pulmonary side of circulation? shunting
⋅AVSD of fully oxygenated blood back into the
lungs.
⋅PDA

A. LESIONS RESULTING IN INCREASED VOLUME LOAD A. LESIONS RESULTING IN INCREASED VOLUME LOAD

Direction and magnitude of shunt across the

communication depend on: COMPLICATIONS

a. Size of defect a. Heart Failure

b. Relative systemic and pulmonary b. Infective Endocarditis
pressure and vascular resistance
c. Eisenmenger Syndrome

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 Ventricular Septal Defect VSD
• Prevalence: most common form of CHD and accounts for 25% of all Clinical manifestations:
defects.
• Pathology: History:
A VSD may be classified into 4: 1. with small VSD, the patient is asymptomatic
1. Perimembranous- most common
2. Outlet defects- accoutn for 5-7%; also called supracristal, conal,
2. Moderate to large VSD, delayed growth and
subpulmonary or subarterial development, decreased exercise tolerance,
3. Inlet defects- account for 5-8% repeated pulmonary infections, and CHF are
4. Trabecular defects-accoutn for 5-20%; may be central, apical, relatively common
margin
3. With history of cyanosis and decreased level of
activity may be present

VSD VSD
Physical Examination: Laboratory Diagnosis
1. Infants with small VSDs are well developed. • Electrocardiogram
2. Infants and children with large VSDs may have 1.With small VSD, the ECG is normal
poor weight gain or show signs of CHF
2.With moderate VSD, LVH and occasional
3. A sytolic thrill may be present Precordial and
hyperactivity are present with arge VSD.
LAH may seen
4. murmur: regurgitant or pansystolic at LLSB 3.With large VSD, the ECG shows combined
ventricular hypertrophy

VSD VSD
X-ray studies: Natural History:
1. Spontaneous closure occurs in 30-50% of pxs with
• Small VSD: normal membranous and muscular VSDs during first 2 years of life.
2. CHF in infants with large VSD, but usually not until 6-8 weeks
of age
• Moderate-large VSD: 3. PVOD may begin to develop as early as 6-12 mos of age in
pxs with large VSDs.
1. Cardiomegaly of varying degrees and 4. Infundibular stenosis may develop in some infants with large
involves LV and RV. defects
5. Aortic insufficiency may develop in pxs with supracristal VSD
2. Pulmonary vascular marking !nereas
6. Infective endocarditis rarely occurs

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 VSD VSD
Management: Management:
1.Medical
-Treatment of CHF, if it develops, with digoxin and diuretics
2. surgical
-No .Exercise restriction is required in the absence of • If growth cannot be improved, the VSD
pulmonary hypertension should be operated on within the first 6
-Maintenance of .Blood oral hygiene and antibiotic months of life
prophylaxis against infective
Endocarditis • Surgery is NOT indicated for small VSD

Atrial Septal Defect ASD

Prevalence: occurs as an isolated anomaly in 6-8% more common in
females
3. ostium secundum defects occur in about
30% of all ASDs, including those that
Pathology: occur as part of AVSD. Isolated ostium
1. Three types exist- secundum defect, primum defect, sinus venosus
defect. A PFO does not ordinarily produce shunting and is not primum ASD occurs in 15% of all ASDs
considered an ASD.

2. Ostium secundum is the most common accounting for 50-70 % of all 4. Sinus Venosus Defect-occurs in about
asds . the delefect located at site of fossa ovalis allowing left-to-riflht
shunting of blood from left atrium to right atrium. Anomalous 10.5 of all ASDs, most commonly loacted
pulmonary venous return is present in 10% of cases. at the entry of superior vena cava into RA.

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 ASD ASD
Clinical Manifestations: Laboratory Diagnosis:
History: Infants and asymptomatic; subtle failure may be present in A. X-ray studies
younger children; in older children varying degrees of exercise
intolerance. 1. Cardiomegaly with enlargement of the RA and RV
PE: 2.A prominent pulmonary artery segment and increased
1. Relatively slender body built is typical pulmonary vascular markings
2. A widely split and fixed S2
3. A grade 2-3/6 systolic ejection murmur at LUSB secondary to relative
PS
B. Electrocardiogram
Right axis deviation and mild right ventricular hypertrophy
*no murmur, only if blood pass to pulmonic valve. or right bundle branch block

ASD ASD
C. Echocardiography Natural History:
- Diagnostic 1. Spontaneous closure in about 40 % in the first 4 years of life
- Indirect signs of left to right shunt RV 2. Most children remain active and asymptomatic.
enlargement and RA enlargement, dilated 3. If a large defect is untreated.CHF and pulmonary
PA, paradoxical motion of hypertension develop in their 20’s and 30’s
interventricutarseptum 4. With or without surgery, atrial arrhythmias may occur
D. Cardiac catheterization 5. Infective endocarditis does not occur in isolated ASDs.

ASD
Treatment:
1. Non-surgical closure
Devices that can be delivered through cardiac
catheterization

2. Surgical closure -indications: QpQs of > 1.5:1

-age: 3 to 4 years of age
-procedure: open heart surgery
-mortality: < 1%
-complications: CVA, arrhythmias

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 Patent Ductus Arteriosus
Prevalence:
-Occurs in 6-8. % Of all CHD, excluding premature
infants; more common in females
Pathology: there, is persistent patency of a
normal fetal structure between the left and the
descending aorta, about 5-10 mm, distal to the
origin of left subclavian artery
PDA
Clinical Manifestations:
History;
1. Patients are asymptomatic when the
ductus is small.
2. A large-shunt PDA may cause lower,
respiratory tract infection, atelectasis, and
CHF (tacchypnea and poor weight gain)

PDA PDA: Laboratory Diagnosis

Physical Examination: Electrocardiogram:
1. Tachycardia and exertional dyspnea in children 1. A normal ECG or LVH is seen with small to moderate
with large PDA. PDA
2. With PVOD a right-left shunt results in cyanosis 2. Combined ventricular hypertrophy is seen with large
only in lower half of the body (differential PDA
cyanosis)
CXR Studies:
2. Bounding peripheral pulses and wide pulse
pressure are characteristic. 1. Normal with small PDA
2. Cardiomegaly of varying degrees with enlargement of
4. Murmur continuous murmur heard best at ULSB LA, LV and ascending aorta pulmonary vascular
or infraclavicular area. markings are increased

PDA
Treatment:
Surgery:
Indications: anatomic existence, regardless of size
Timing: anytime between 6 mos to 2 years of
age or when the diagnosis is made in an older
child
Procedure: ligation or transection
Catheter closure
Mortality: <1%

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 Complete Atrio-Ventricular Canal Defect
A. LESIONS RESULTING IN INCREASED VOLUME LOAD

2. REGURGITANT LESIONS
4 ⋅Mitral Regurgitation
1
3 ⋅Ebstein Anomaly
2
⋅Pulmonary Regurgitation
⋅Aortic Regurgitation
1. Atrial septal defect, primum type.
2. Inlet (type III) ventricular septal defect.
± VSD
3. Cleft and deformity of tricuspid valve.
4. Cleft and deformity of mitral valve.

Ebstein's Anomaly of the Tricuspid Valve from birth to 7 years of age

A. LESIONS RESULTING IN INCREASED VOLUME LOAD
2 days old

6 months old
3. CARDIOMYOPATHIES
⋅Viral
⋅Metabolic
1 ⋅Genetic Defects
2

7 years old

B. LESIONS RESULTING IN INCREASED PRESSURE

WORK Pulmonary Stenosis
Prevalence:
PATHOPHYSIOLOGY: Pathology
Obstruction to normal blood flow 1. PS may be valvular, subvalvular (infundibular )
supravalvular
2. In valvular PS the valve is thickened, with fused or
1. Obstruction to Ventricular Outflow absent commissures.
3. Dysplastic valves are frequently seen with Noonan’s
⋅Valvular PS syndrome isolated infundibular PS is rare, usually seen
⋅Valvular AS in TOF
⋅Coarctation of Aorta 4. Supravalvular PS is occasionally seen with rubella
Williams syndrome

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 PS
History:
1. Children with mild PS are completely
asymptomatic. Exertional dyspnea and
easy fatigability may be present in pts with
moderately severe cases.
2. Newborns with critical PS feed poorly, are
tachypneic, and may be cyanotic
PS
ECG:
1. Normal in mild cases
2. Right axis deviation and RVH are present In
moderate. The degree of RVH on ECG
correlates with severity of PS
3. Right atrial hypertrophy and RVH with strain
may be seen with severe PS
4. Neonates with critical PS may show LVH
because of a hypoplastic RV
PS
Treatment
A. Medical:
1. Restriction of activity not necessary except in
cases of severe PS
2. Antibiotic prophylaxis against SBE
3. Balloon valvuloplasty is the procedure of choice
4. Newborns with critical PS: prostaglandin infusion;
balloon valvuloplasty
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PS
Physical Examination:
1. Most patients are acyanotic and well developed.
Newborns with critical PS are cyanotic and techypneic.
2. A right ventricular tap and systolic thrill may be present
3. A systolic ejection click is present with valvular PS. The
S2 may split widely, and the P2 may be diminish
intensity. An ejection-type systolic murmur is best
audible at ULSB. The louder and longer the murmur, the
more severe the stenosis is.
PS
Studies:
1. Heart size is usually normal, but the main PA
segment is prominent
2. Pulmonary vascular markings are usually
normal but may decrease with severe PS.
3. In neonates with critical PS, lung fields are
oligemic with varying degree of cardiomegaly.
Echocardiography
PS
B. Surgical:
1. Children with valvular PS in whom balloon
valvuloplasty is unsuccessful.
2. Other types of obstruction (infundibular,
anomalous RV muscle) with significant pressure
gradient also require surgery
3. If balloon valvuloplasty is unsuccessful or
unavailable, infants with critical PS require
surgery on an urgent basis

AS
History:
1. Most children with mild to moderate AS are
asymptomatic. Occasionally, exercise
intolerance may be present
2. Exertional chest pain, easy fatigability, or
syncope may occur with severe obstruction
3. Infants with critical AS may develop CHF within
first few weeks to months of life.
AS
Natural history:
1. Chest pain, syncope, and even sudden death may occur
with severe AS
2. Heart failure occurs with severe AS during the newborn
period or later in adult life
3. A significant increase in PG frequently occurs with
growth
4. The stenosis may worsen with aging as a result of
calcification
5. SBE occurs in approximately 4%
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Aortic Stenosis
Prevalence: accounts for 5%; M > F
Pathology:
1.Stenosis may be valvular, subvalvular. supravalvular
2. Valvular AS may be caused by bicuspid AV, unicuspid
AV, or stenosis of tricuspid AV. (Bicuspid AV is the most
common form)
3. Supravalvular AS is an annular constriction above AV;
often associated with Williams syndrome
4. Subvalvular, AS may result from simple diaphragm or
from long tunnel-like fibromuscular narrowing of LV
outflow tract
AS
ECG: normal in mild cases. LVH with or without strain
pattern may be present in severe cases.
X-ray studies:
1. Heart size is usually normal, but a dilated ascending
aorta or prominent aortic knob may be seen with valvular
AS.
2. Significant cardiomegaly does not develop unles CHF
occurs.
3. Newborns with critical AS show generalized
cardiomegaly with pulmonary venous congestion.
AS
Management:
Medical:
1. Maintenance of good oral hygiene and antibiotic
prophylaxis
2. Children with mod-severe AS should not
perform sustained, strenuous physical activities
3. For critically ill-newboms with CHF, inotropics,
diuretics and prostaglandin should be started
 Severe Aortic Stenosis in an infant
AS
Balloon valvuloplasty- indicated for children RV

with mod-severe AS, and in symptomatic RA

neonates LA
1
Surgical treatment- reserved for valves that
are not amenable to balloon therapy
1. Surgical valvotomy
2. Aortic valve replacement
1) Valvular Stenosis
with a bicuspid
aortic valve

Coarctation of Aorta COA

Prevalence: occurs in 5-7 % of all lesions; M>F
-May occur at any point; but most common just
below the origin of left subclavian artery (98%)
- May be a feature of Turner's (30%)
- Associated with bicuspid AV, mitral valve
abnormalities, and subaortic stenosis (Shone
complex)
Asymptomatic children:
1. Normal growth and development
2. Arterial pulses in the leg are either absent or
weak and delayed. There is hypertension in the
arm
3. A systolic thrill present at parasternal notch,
ejection click is frequently audible which
originates from bicuspid AV. A short systolic
murmur can be heard at URSB and mid or lower
left sternal border

COA COA
X-ray studies: ECG:
-infants with severe COA, marked cardiomegaly -Normal or rightward QRS axis and RVH in most infants
with severe COA
and pulmonary edema are present -LVH is seen in older children; maybe normal in 20% of
-in children, heart size maybe normal or slightly patients
enlarged, dilatation of ascending aorta, rib Echocardiography:
notching in older children - Will show the site and extent of COA
- Will demonstrate associated abnormalities like bicuspid
AV, MV abnormalities, PDA

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Coarctation of the Aorta with severe aortic stenosis in an infant
B. LESIONS RESULTING IN INCREASED PRESSURE
WORK
1

2. Obstruction to Ventricular Inflow

⋅Tricuscupid Stenosis
2

⋅Mitral Stenosis
⋅Cor Triatriatum
1. Coarctation of the aorta,
distal to the left
subclavian artery

2. Severe aortic stenosis

B. LESIONS RESULTING IN INCREASED PRESSURE

WORK CYANOTIC CONGENITAL HEART LESIONS

COMPLICATIONS
⋅Heart Failure Divided according to Pathophysiology:
PULMONARY BLOOD FLOW (PBF)
⋅Total Circulatory Collapse
⋅± Cyanosis a. Decreased PBF
⋅Systemic Hypertension b. Increased PBF

A. DECREASED PBF A. DECREASED PBF

TOF Included in these LESIONS are:

Pulmonary Atresia with Intact Ventricular 1. Obstruction to PBF

Septum 2. Pathway by which VENOUS BLOOD

can shunt from R? L and enter
Tricuspid Atresia systemic circulation (PFO, ASD,VSD)

TAPVC with Obstruction

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 Tetralogy Of Fallot
Prevalence: occurs in 5 – 7 % of all CHD
- Most common cyanotic heart defect seen in
children beyond infancy
Pathology:
1. Consists of 4 abnormalities: large VSD,
right ventricular outflow tract obstruction,
RVH, overriding of the aorta.
2. The pulmonary annulus and MPA is often
small.
3. The aortic arch is right-sided in 20 %
TOF
• The degree of right ventricular outflow
obstruction determines the timing of
onset of symptoms, the severity of
cyanosis, and the degree of RVH
• When the obstruction is mild, the
patient may not be visibly cyanotic
( pink TOF )

TOF TOF
History: ECG: RAD and RVH
1. A heart murmur is usually audible at birth
CXR: heart size is normal or smaller than
2. Most patients are symptomatic with normal, the pulmonary vascular
cyanosis at birth or shortly thereafter.
Dyspnea on exertion,squatting, or hypoxic
markings are decreased
spells develop later, even in mildly cyanotic -a concave MPA segment with
infants upturned apex (boot-shaped or coeur
3. Infants with acyanotic TOF maybe en sabot)
asymptomatic or may show signs of CHF -RAE and right aortic arch

TOF TOF
Natural History:
Echo: establishes the diagnosis, extent 1. Infants with acyanotic TOF gradually
become cyanotic
of aortic override, location and degree
2. Polycythemia develops
of pulmonary obstruction, size of
3. Development of relative iron-deficiency
proximal branch pulmonary arteries state
Angiography: best demonstrates the 4. Hypoxic spells
anatomy of pulmonary arteries 5. Growth retardation
6. Brain abscess and cerebrovascular
accidents
7. SBE

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 Pulmonary Atresia with Intact PA with Intact Septum
Septum
- The pulmonary valve is atretic, ventricular CM:
septum is intact -severe cyanosis and tachypnea are seen
- No egress of blood from the RV in distressed infants
- RA pressures increases and blood shunts via -S2 is single, heart murmur is usually
the foramen ovale into the LA absent, but soft murmur of PDA or TR
- These pts may have sinusoidal channels maybe audible
within the RV
-inadequate interatrial communication
- An interatrial communication and PDA
are necessary for the pt to survive causes hepatomagaly

PA with Intact Septum PA with Intact Septum

ECG: QRS axis 0 – 90 degrees, RAE, Treatment:

LVH
CXR: heart size maybe normal or large,
resulting from RAE. Pulmonary
vascular markings are decreased
Echo: useful in estimating th RV
dimensions, size of TV, sinusoidal
channels which are of prognostic value
-infusion of Prostaglandin
-aortopulmonary shunt
-radiofrequency ablation catheter
followed by balloon valvuloplasty
-Fontan procedure

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 Tricuspid Valve Atresia
Prevalence: accounts for 1 – 2 % of CHD
Pathology:
1. TV is absent, and the RV is hypoplastic.
Associated defects such as ASD, VSD an
PDA are necessary for survival
2. TVA is usually classified according to the
presence or absence of PS and TGA. The
great arteries are normally related in about
70% and transposed in 30%

TVA
CM:
1. Cyanosis usually evident at birth
2. A systolic thrill is rarely when
associated with PS
3. S2 is single, a ger 2-3/6 regurgitant
systolic murmur of VSD is noted at
LLSB
4. Hepatomegaly may indicate an
inadequate interatrial communication
or CHF
TVA
ECG: LAD , LVH
CXR: heart size maybe normal or slightly
increased. Pulmonary vascularity
decreases in most patients, although it
may increase with TVA/TGA
Echo: establishes the diagnosis;
absence of tricuspid orifice, small RV,
large LV, associated abnormalities

TVA
Treatment:
- Prostaglandin infusion
- Atrial septostomy
- Aortopulmonary shunt procedure
- Bidirectional Glenn Shunt
- Fontan procedure

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 A. DECREASED PBF A. DECREASED PBF

Degree of cyanosis depend on:

COMPLICATIONS
a. Degree of obstruction to PBF:  Hypercyanotic, TET, Spells
MILD vs SEVERE  Shock
 Infective Endocarditis
b. When the ductus arteriosus closes
 Cerebrovascular accidents
 Brain abscess

ABNORMAL VENTRICULO-ARTERIAL
B. INCREASED PBF CONNECTIONS

• Not associated with obstruction to PBF • Transposition of Great Arteries (TGA)

• Cyanosis caused by either
a. Abnormal ventricular-arterial connections
b. Total mixing of systemic venous and
pulmonary venous blood within the heart
Systemic blood? RA? RV? Aorta? Body
Oxygenated blood? pulmonary veins?
LA? LV? pulmonary artery? lungs
Survival depends on fetal pathways
(PFO, PDA and VSD)

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 TGA
-Defects that permit mixing of the two
circulations ( ASD/PFO, VSD, PDA )
are necessary for survival.
-Half of patients with tga do not have
associated defect other than pfo or
small pda simple tga
-VSD is present in about half of these
patients
-The clinical presentation varies in
relation to presence or absence of
associated defects
TGA with Intact Septum
PE:
1. Moderate to severe cyanosis, in large,
male newborns
2. Infant is tachypneic but without
retractions unless CHF supervenes
3. S2 is single and loud. Murmurs maybe
absent, or a soft systolic ejection
murmur
21 | K e n n e t h D . E s p i n o
TGA with Intact Septum
CM:
1. Cyanosis and tachypnea are always
present
2. Hypoxemia is usually severe
-the condition is a medical emergency; early
diagnosis and appropriate treatment can
avert the development of hypoxemia of
severe hypoxemia and acidosis, which lead
to death
TGA with Intact Septum
Laboratory Studies:
-severe arterial hypoxemia with or without
acidosis
-hypoglycemia and hypocalcemia
ECG: RAD, RVH
CXR: cardiomegaly with increased
pulmonary vascularity
“egg-shaped” cardiac silhoutte
Echo: diagnostic
 TGA with Intact Septum
Treatment:
1. Prostaglandin – to improve
oxygenation
2. Maintain normothermia, correction of
acidosis and hypoglycemia
3. Atrial septostomy- performed in all
patients in whom delay in operation is
necessary
TGA with Intact Septum
Arterial switch operation ( Jatene )
- Surgical treatment of choice
- Usually performed within the first 2
weeks of life
- Restores the normal physiologic
relationships of systemic and
pulmonary blood flow
- Survival rate is 90 – 95 %

TOTAL MIXING LESIONS

• Common Atrium or Ventricle

• Total Abnomalous Pulmonary Venous
Return (TAPVR)
• Truncus Arteriosus

Total Anomalous Pulmonary Venous TAPVR

Return
Prevalence: accounts for 1-2 % of CHD
Pathology:
1. No direct communication exists between
pulmonary veins and the LA
2. Defect maybe divided into 4 types:
a. Supracardiac: accounts for 50 %; pulmonary
veins drain into right SVC via the left
vertical vein and innominate vein
b. Cardiac: accounts for 20 %; common
pulmonary vein drains into the coronary
sinus or directly into the RA
c. Infracardiac: common pulmonary vein
into the portal vein, hepatic vein, or
IVC; most commonly associated with
obstruction
d. Mixed type

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 Total Anomalous Pulmonary Venous Return to the Innominate Vein

Innominate
Vein

Vertical
2 Vein

Right
Pulmonary Veins

Vertical
AO RPA AO
Vein
RV
RPA

LV LA Venous Venous
Confluence Confluence

TAPVR TAPVR
CM: ECG: RAE, RVH
A.Without pulmonary venous obstruction
1. CHF with growth retardation and frequent CXR:
respiratory infections
2. History of mild cyanosis from birth 1. Moderate to marked cardiomegaly
3. Precordial bulge with hyperactive RV with increased pulmonary vascular
4. S2 is widely split and fixed, P2 is markings
accentuated 2. “snowman sign” or figure of eight
5. A gr 2-3/6 systolic ejection murmur at maybe seen in supracardiac type
ULSB

Truncus Arteriosus
- Single arterial trunk arises from the
heart and supplies the systemic,
pulmonary and coronary circulations
- A VSD is always present and is directly
below the truncus
- A right aortic arch is present in 30 %
- Di George syndrome present in 33 %
- 4 types

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 TA TA
- Clinical picture varies with age, ECG: CVH is present in 70 %
depending on level of pulmonary CXR: cardiomegaly with increased
vascular resistance pulmonary vascularity, right sided arch
is seen in 30%
- In newborn period: signs of heart failure
are usually absent; minimal cyanosis Echo: demonstrates a large truncal artery
overriding a large VSD
- In older infants: pulmonary blood flow is
increased and the clinical picture is
dominated by heart failure

Truncus Arteriosus TOTAL MIXING LESIONS

Deoxygenated systemic venous blood and

1 oxygenated blood mix completely in
heart? oxygen saturation equal in
2 pulmonary artery and aorta
3

COMPLICATIONS
1. Pulmonary arteries arise from aorta.
Truncus
⋅Cyanosis
⋅Heart failure
2. Truncal valve, occasionally quadracuspid,
LV
stenotic and or insufficient.
Overrides the ventricular septal defect.
3. Ventricular septal defect, large

OTHER CONGENITAL HEART & VASCULAR

MALFORMATIONS PULMONARY HYPERTENSION (PH)

1. Primary PH
• Anomalies of aortic arch

2. Pulmonary Vascular Disease

• Anomalous origin of coronary arteries
(Eisenmenger Syndrome)

24 | K e n n e t h D . E s p i n o
 EISENMENGER SYNDROME GENERAL PRINCIPLES OF TREATMENT OF CHD

• Patients with VSD, blood shunted R? L
2º pulmonary vascular disease
• Also seen in patients with ASD, AVSD,
PDA
• Present with cyanosis, right heart failure
• MILD CHD-
Require NO treatment in most patients,
NO restrictions in physical activity
• MODERATE TO SEVERE CHD-
⋅PE modified appropriate to child’s physical activity
determined by EXERCISE TESTING
⋅Prophylaxis vs Bacterial endocarditis
ex. Dental procedure
Instrumentation of urinary tract
Before lower GI manipulation

PAROXYSMAL HYPERCYANOTIC ATTACKS

GENERAL PRINCIPLES OF TREATMENT OF CHD (Hypoxic, “blue” or “tet” spells)

• MONITOR CYANOTIC PATIENTS • A problem during first 2 years of life

⋅Iron deficiency anemia/ polycythemia • Characterized by:
⋅Dehydration avoided Hyperpnea and restlessness
Increased cyanosis
⋅High altitudes and sudden change in thermal
Gasping respirations
environment be avoided Syncope/ seizures (severe spells)
• PHLEBOTOMY IN SYMPTOMATIC PATIENT WITH Generalized weakness→ sleep
POLYCYTHEMIA (Hct >65%) • Occur frequently in the morning on awakening
or after vigorous crying
• COUNSEL WOMEN ON RISKS ASSOCIATED WITH
• Disappearance of systolic murmur
CHILDBEARING

*
Congenital Heart Disease
Procedures To Be Instituted During Spells:
• ACYANOTIC CHD • CYANOTIC CHD
1. Lesions Resulting In Increased 1. Increased Pulmonary Blood
Volume Load Flow:
1. ASD 1. Transposition of the great vessels
2. VSD 2. Defects with a common atrium or
1. Place infant on the abdomen in a 3. AVSD ventricle,
4. PDA 3. Total Anomalous Pulmonary Venous
knee- chest position. 2. Lesions Resulting In Increased
Return
4. Truncus Arteriosus
Pressure Load
2. Administer oxygen A. Obstructions To Ventricular Outflow:
2. Decresed Pulmonary Blood Flow:
1) Valvular Pulmonic Stenosis
1. Tetralogy of Fallot
2) Valvular Aortic Stenosis,
3. Inject Morphine SO4 0.2 mg/k 3) Coarctation Of The Aorta
2. Tricuspid Atresia
3. Various forms of single ventricle with
B. Obstruction To Ventricular Inflow:
Pulmonary Stenosis
1) Tricuspid Stenosis
2) Mitral Stenosis
3) Cor Triatriatum THE END!

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