Pediatric Cardiology

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PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

CARDIAC ANATOMY

a membranous type because the membranous septum is the last to
close.

EMBRYOLOGY

AORTIC ARCH DEVELOPMENT

After fertilization there is the formation of your morula. And then on
the 18th day there is the appearance of a plate. They are two: a
tissue that becomes hollow to form a pair of endothelial tubes,
these will fuse to form your primitive cardiac tube.

Mechanism of aortic arch selection which is probably related to
redistribution of blood flow. The aortic blood vessels are devoid of
smooth muscles initially and therefore incapable of active
vasoconstriction. Initially you have 2 lines or2 arches: double aortic
arch as congenital anomaly. Aortich arches form new aorta.

It has 2 divisions/stages of cardiac development:

Early cardiogenesis

Morphologic stage
The morula and the plate will fuse to form your primitive heart. They
all came from angiogenic cell clusters. At 22 days of age, there are 2
pairs of tubes.
Divisions of primitive heart:
1. Truncus – becomes aorta
2. Bulbus cordis – divided into 2
3. Old ventricle
4. Atrium
Bulbus cordis becomes corus cordis and bulbus cordis; becoming
left and right ventricles. Atria will be divided into 2: the right and the
left atria, and then the sinus venosus.
Truncus forms aorta and pulmonary artery.
Bulbus – right ventricle
Conus cordis becomes outflow tract for both ventricles. The right
and the left ventricle will have its own outflow tract. However the
conus in the left ventricle will be absorbed unlike in the right
ventricle, it has a long outflow tract because the conus on the right
is not absorbed.
Primitive ventricle will become left ventricle.
CARDIAC LOOPING
Straight tube will loop prior to cardiac looping.
During cardiac looping: D loop or L loop

D loop – normal looping

L loop – dextrocardia or ventricular inversion
Atria will go up, rotate spirally. It will be placed at the back of your
truncus. Ventricle will be left on its place. Atria will be on upper part,
ventricle on lower part. Bulbus cordis would be placed on the
anterior, on the left side = normal looping
If you have an exposure to any authentic agent that may interrupt
the embryogenesis, it will produce congenital heart disease. It
depends on what stage the heart is being formed.
nd

If you have an infection on the 22 day where you do not have yet
the valves, you may have a patient with just a univentricular heart.
The earliest time that the mother has the infection, the worse is the
heart disease.
CARDIAC SEPTATION
You forming the interatrial septum. During the formation of the
interatrial septum,, initially you have a common atrium then you will
form a septum. The first one, upper one will outpouch. Septum
primum is the first to form. The hole between your endocaridal
cushion and your septum primum is your ostium primum. Primum
first to form after which, closure. Septum secundum grows to close
it fully. When they are closed fully, you are going to have a hole in
that septum coalesce to form you foramen ovale. Your foramen
ovale is important in the cardiac cycle because your fetus has a
parallel circulation.
Hole created is called foramen segundum/ostium segundum.
th

Atrial septation occurs at about the 28 day of fetal life.
th
Ventricular septation occurs on 25 day of fetal life.
2 ventricles – out pouching – will not meet endocardial cushion –
will wait until rotation of conus is complete: MC form of your VSD is

st

1 aortic arch (R and L) – maxillary artery
nd
2 aortic arch (R and L) – hyoid stapedial arteries
rd
3 aortic arch – common artery common carotids (internal and
external)
th
4 aortic arch – part of subclavian arteries
th
5 aortic arch – will not develop
th
6 arch – left pulmonary artery and ductus arteriosus
th

If there is an abnormality in the absorption of your 6 aortic arch
you are going to have your persistent/patent ductus arteriosus.
CARDIAC DIFFERENTIATION
Totipotential cells would tell what type of cell it will go into
differentiation. Signal a specific cell lineage. Induction regulates
formation of cell lineage.
Developmental changes in cardiac formation:

Hyperplasia – prenatal life

Hypertrophy – postnatal life

Fetal infant/heart – sensitive to Ca

Sensitive to preload and afterload
Na and Ca channel is only found in cardiac muscles.
Fetal myocardium very sensitive to Calcium which is mainly stored in
the endoplasmic reticulum. However the t tubule is open to
extracellular area. Calcium can easily go in. Ca channel blockers is
never given to 6 y/o and below.
SVT (supraventricular tachycardia) – Verapamil given in adults never
given in children 6 y/o and below(calcium antagonist)
During resuscitation, part of regimen given is Calcium gluconate,
because it can help in cardiac contractility of myocardium. patients
with low calcium may produce bradycardia. Any imbalance in
electrolyte may cause danger to fetal myocardium.
Pediatric patient with diarrhea – easily go into cardiac distress
FETAL CIRCULATION
In parallel
Umbilical and pulmonary vein = only 2 that carry oxygenated blood.
From the placenta which is high in pO2, it will go to umbilical vein,
then to ductus venosus 50% of which will go to the portal circulation
and the remaining goes to the right atrium with the help of a
structure directing blood to the foramen ovale, to the left atrium,
left ventricle then to the aorta. Blood from SVC which is less
oxygenated will go to the right atrium, right ventricle to pulmonary
artery, 30% will go to lungs not the whole of it because of high
pulmonary arterial pressure, the lung is collapsed. The blood will go
to the ductus arteriosus and then to the descending aorta, to the
circulation and then back to the placenta via the umbilical artery.
PRESSURES
In patients with Congenital Heart Disease, you always get the oxygen
saturation and intracardial pressures to know the flow of blood,
compute for pulmonary vascular resistance.
IVC – most of your blood will cross the foramen ovale and then to
the left side aorta.
From the SVC, 21% – actually O2 sat only about 15% goes to right
ventricle, then to the PDA.
After birth, your baby goes into Transitional circulation:

↓ PVR brought about by first breath; collapsed lung will
now open up, decreasing PVR; if you have a persistent
fetal circulation, you will have a persistent increase in PVR
Page 1 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra


SVR will gradually increase, heart adapts by increasing L
ventricular mass, LV will be bigger than RV but during fetal
life they are equal in size.

Hx – 80% you can diagnose the case
-

During fetal echocardiogram, sometimes there is difficulty in
distinguishing the right from the left because there are diseases
where the right atrium goes to the left ventricle. Ventricular
inversion = normally right atrium – tricuspid valve – RV; the right
atrium drains into tricuspid and then your left ventricle.
During fetal echocardiogram you try to distinguish whether the right
atrium or left ventricle are in their proper morphologic position.
There are structures that you have to identify inside the right
ventricle to know that it is really the right ventricle.



Closure of shunts: foramen ovale, ductus arteriosus
(becoming ligamentum arteriosum) and ductus venosus
(becoming ligamentum venosum)
Increase in catecholamines and myocardial receptors.
Catecholamines will help in the closure of your ductus
arteriosus. It will constrict your ductus to fully close.

NEONATAL CIRCULATION
1.

2.
3.
4.
5.

R→L vs L→R shunt via PFO; blood form RA will pass
through foramen ovale to LA = right to left shunt,
increased SVR, increased pressure on left side = left to
right shunt; right to left shunt after birth – think of
myocardial problem (probably high pulmonary arterial
pressure, persistent fetal circulation)
R→L vs L→R shunt via PDA; from RV to PA, ductus
arteriosus to descending aorta (right to left)
Pulmonary vasculature constrict more in response to
hypoxemia, hypercapnea, acidosis
Ventricular thickness RV = LV; more of LV as compared to
RV
High O2 consumption → high CO; baby very dependent in
blood volume
rd

Foramen ovale may still be patent up to 3 month of life.
If you have a baby with a high pulmonary arterial pressure, atretic
and cyanotic at birth do 2D echo – look at pulmonary arteries
(pulmonary arterial pressure) to see if there is a right to left shunt by
rd
the foramen ovale. Repaeat 2D echo on 3 month.
th

Ductus arteriosus closes on 10 – 15 hour of life; closure depends on
whether the baby is term or preterm; closes in term infant because
smooth muscles are more developed. Sometimes the baby is
dependent on the PDA if they have a congenital heart disease
especially those who are cyanotic
If your baby has Pulmonary valve atresia – blood from RV cannot go
to lungs because you have an atretic valve. To oxygenate your lungs
you need this PDA; or if you have a low or high O2 saturation. High
O2 sat – close, low O2 – open
Volume overload – open PDA (that’s why you give diuretics)
EVALUATION OF CARDIOVASCULAR SYSTEM
Hx and PE
1. Guide echocardiographic evaluation
e.g. 15 y/o patient in severe respiratory distress; RR = 30;
next question: what triggers the symptom, developed
dyspnea going up the stairs, 5 steps; when was it/did it
start? – a month ago; duration – for the last one month;
cough at night while lying down every night, when going
up stairs gasps for air; check up with doctor said patient
had pneumonia was given antibiotics; had fever for almost
2 weeks before check up; chest pain; 3 – 4 pillows give
relief; on and off sore throat since Jan. 2011; relieved at
rest = patient might be having Rheumatic fever, RHD or
CHD, CHF; birth history = congenital heart disease
2. Eliminating expensive laboratory exams
3. Activities e.g. dyspnea during activity
Doctor does echocardiogram in pediatric patients.

-

Prenatal
Medications
Exposures – esp. congenital; term cyanotic delivery:
exposure to Rubella, PDA in congenital rubella syndrome;
rd
when did the mother have a problem? Infection on 3
month of gestation – cardiac anatomy is already formed,
probably pulmonary artery is stenosed or there is a lodged
shunt; exposure/illness at 1 month of gestation: TGA,TOF;
th
th
7 to 9 month of gestation, most do not manifest with
cyanotic heart disease more of the infection
Signs and symptoms – depends on what age patient is:
infant – feeding history (sleeping during feeding), how
many ounces does the baby consume? Manifestation of
congestion is with interrupted feeding. Upon feeding there
may be diaphoresis. Pattern of breathing e.g. fast
breathing, fatigability; cyanosis – e.g. 1 month when place
baby in air conditioned room (acrocyanosis)

Chest pain: 15 y/o male, comes to you with chest pain – started a
year ago, even at rest, more svere pain during activity, sometimes
relieved by rest, pain scale 5/10, does not hinder normal activities,
feeling of something on the chest, radiates to left and back
sometimes, sometimes stomach hurts, Ranitidine, Omeprazole,
Mallox; mother and sibling has RHD, cousin operated for valve
replacement; Sumapen for the last 5 years but sometimes forgets to
take it (prophylaxis for RHD); having it for a year, pain with activities
= RHD
Patients with aortic regurgitation may manifest
Heart failure – feeding, diaphoresis, fatigue, breathing, respiratory
distress, dyspnea, cyanosis (look at tongue)
PE
-

General survey: aesthenic, hyperaesthenic, hypoaesthenic;
look for Harrison’s groove
Vital signs: CR, RR, Temp, BP, always get height and weight
Cyanosis: mild, differential, acrocyanosis; may use pulse
oximeter to get O2 sat
Heart failure – DOB, difficulty of feeding, easy fatigability

5y/o with HR of 50 = abnormal; normal is 70 and above up to 110 or
120
1 month old with HR of 80 = abnormal; normal is 110 – 160
1 month old with HR of 180 = abnormal
Patients with shunt diseases are mostly wasted.
Cardiac PE
Inspection – bulge; Harrison’s groove (line that you see on
chest wall in patients with long standing heart failure
mostly in patients with shunts particularly those with large
VSD, ASD or PDA secondary to prolonged congestion that
patient has been having)
th
Palpation – apical impulse: normal location is 4 ICS;
substernal thrust = Right Ventricular Enlargement; thrill –
palpable murmur
th
Percussion – very rarely done; dull sound in 6 ICS or
axillary area in adults – RVE
Auscultation – done immediately when patient is quiet
th

1 month old baby with apical impulse on 5 ICS = abnormal; apex
th
beat goes to 5 ICS at around 4 to 5 months of age
HEART SOUNDS
-

S1 – closure of AV valves
S2 – closure of pulmonic and aortic valves
A1
P2
Split – deep inhalation

When you inhale deeply there will be a more negative intrathoracic
pressure, more blood will go to the right ventricle so there will be a

Page 2 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra
delay in the closure of your pulmonary valve. P2 delayed during deep
inspiration.

right atrial enlargement; but if you have a tall P >0.25 mm normal
height of P wave is 2.5 (2 small squares and a half), the duration is
up to 0.25

Physiologic splitting – delayed closure of pulmonic valve
MURMURS
-

abnormal sounds heard on patients chest

Grade I – barely audible
Grade II – medium intensity
Grade III – loud but no thrill
Grade IV – loud murmur with a thrill
Grade V – very loud
Grade VI – hear murmur even without placing stethoscope
Look for timing:
Systolic
Diastolic
Pansystolic – murmur heard all throughout of systole;
most commonly produced by VSD
Continuous – murmur heard all throughout systole and
diastole; most commonly heard among patients with PDA
(machinery murmur)
Innocent – musical like sound heard in the heart secondary
to IDA, febrile patient
Mitral regurgitation = heard during systole; AV valve not fully closed
Diastole = Mitral stenosis
LAB EVALUATION
-

-

Good Hx
Good PE
CXR – PA view or lateral view; right side: moguls – RV on
anterior; on right SVC, RA, RV; LV will not produce a
shadow on PA view because it is located posteriorly;
nd
enlargement of 2 mogul on the left think of PDA or VS
ECG – demonstrate anatomic and hemodynamic system;
13 lead – 15 lead – 18 lead
Echocardiogram

Pure R in neonate – RVE
Difference between pediatric and adult ECG:
1. HR – tachycardia accepted in newborn and gradually ↓
with age; secondary to maturity of sympathetic nervous
system; HR dependent on ANS
2. RVH – in utero work of right ventricle is greater; at birth in
↓ RVSP and ↑ in systemic resistance: LV becomes thicker
than RV
3. Right axis deviation – may remain up to 1 year of age up to
135˚; patient 1 y/o with axis of 150 = abnormal
Routine ECG interpretation:
Rhythm – sinus or non-sinus
Sinus: followed by a QRS with a fixed PR interval
HR: compute for atrial and ventricular rate; in patients with atrial
fibrillation or atrial flutter, your atrial rate is faster than your
ventricular rate or in patients with complete heart block your atrial
rate is slower than your ventricular rate or vice versa.
You plot your QRS and T axis, measure QR interval; for any patient
you always measure the PR, QRS and QT.
Prolongation of your PR will tell you that you may have a block – AV
block first degree, second degree, third degree, complete heart
block
QRS if you have PVCs; normal QRS is 0.08; for pediatric group the
normal PR is only 0.16 seconds, adults up to 0.2
QT for pediatric group is up to 0.44. We compute for the QTc
(corrected QT) using the Bechet’s formula.The P wave amplitude
and the duration will tell you if you have left atrial enlargement or

The QRS amplitude would tell you if you have a tall R it depends on
th
the right or left leads. V1 tall R; V1 and V2 at level of 4 ICS; RV – look
at V1, LV – look at V5, V6 and V7. If you see a tall R in V6 you have left
ventricular enlargement
P wave = normal axis is 90
QRS complex – ventricular hypertrophy; also tells us of the systolic
overload pattern if you see tall PQR in right precordial leads (V1) or
diastolic overload pattern – RSR in primum ASD.
Hematologic = request as needed
Patient severely cyanotic = request for CBC to know if the patient is
Polycythemic. The normal Hct in a cyanotic patient is up to 0.6.
Above that we do phlebotomy.
3D echo
TEE (transesophageal echocardiogram) – insert a probe through
th
your esophagus at the level of your heart, 4 intercostal; better
done in patients for operation because they are sedated
Fetal echocardiography
Exercise testing – Stress test
MRI/CT
Radionucleide study
CT angiography – evaluate the structures not seen during echo, in
patients with double aortic arch, long segment coarctation of the
aorta, evaluate peripheral stenosis; insert probe in inguinal area
towards the heart and get the pressures to know if patient is
operable or not
Angiogram put a dye to see if there is stenosis, get pressures within
heart

CONGENITAL HEART DISEASE
PREVALENCE
0.5 – 0.8% of live births
Higher in stillborn 3 – 4%
Spontaneous abortions 10 – 25%; most of the patients that
acquire congenital heart diseae in the first trimester of
pregnancy spontaneously abort
Premature 2%
VSD = most common; perimembranous VSD
perimembranous septum – last to close in the formation of
ventricular septation
Relative Frequency of Major Congenital Heart Lesions
LESION
% OF ALL LESIONS
Ventricular septal defect
35–30
Atrial septal defect (secundum)
6–8
Patent ductus arteriosus
6–8
Coarctation of aorta
5–7
Tetralogy of Fallot
5–7
Pulmonary valve stenosis
Aortic valve stenosis
d-Transposition of great arteries
Hypoplastic left ventricle
Hypoplastic right ventricle
Truncus arteriosus
Total anomalous pulmonary venous return
Tricuspid atresia
Single ventricle
Double-outlet right ventricle
Others
*

5–7
4–7
3–5
1–3
1–3
1–2
1–2
1–2
1–2
1–2
5–10

Excluding patent ductus arteriosus in preterm neonates, bicuspid
aortic valve, physiologic peripheral pulmonic stenosis, and mitral
valve prolapse.
Page 3 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra
VSD > ASD > PDA etc.; most complex are least common
Sometimes CHD is diagnosed even at birth; sometimes diagnosed
late
There are certain diseases that do not manifest early. If you hear a
murmur at birth, you should be alarmed because most of the
murmurs heard at birth are those that are PDA dependent.
Sometimes there is the late manifestation of the symtptoms just like
your Tetralogy of Fallot. Your baby will not get cyanotic immediately.
rd
The cyanosis in TOF will manifest at 3 month of age
LATE DIAGNOSIS OF CHD
rd

Late manifestation of symptoms e.g. TOF – cyanosis 3
month of age because during this time the pulmonary
vascular resistance is going down or the PDA is closing

Hemodynamic effects of transitional circulation – there is a
drop in PVR after the baby breathes, SVR gradually
increases after first breath; if you have a high PAP, high
PVR if patient has PDA your murmur will not be heard. But
once the systemic vascular resitance overcomes your
pulmonary vascular resistance, the continuous murmur of
your PDA can now be appreciated.

Shunt anomalies size of defects → very important:

VSD - smaller the size, more audible is the murmur
because there is a great difference in the pressue of
the right and the left ventricle. Your LV is ~ 80 – 120,
that is your systolic pressure. Your RV has about a
pressure of 10 – 15so there is a great difference in
the pressure.buta s your VSD or ASD increases in size,
there will be an equalization of pressure. That is why
in patients with large VSD you do not really
appreciate the murmur because of the equalization of
pressure of the right and left ventricle. The same goes
for patients with AVSD or common atrium/ventricle,
you do not appreciate the murmur early in life

Common atrium/ventricle – it will take you ~ 1 – 2
months before you can appreciate the murmur
because it is during that time that your pressure in
the pulmonary artery decreases.

↑ PVR → ↓ congestion – during fetal life you have PVR
because of the collapsed lung, after first breath PAP
gradually goes down; increase in flow of blood to the
lungs; ↓ PVR → congestion. In patients with large VSD, the
symptoms of congestion are manifested at 1 month of
age.

ETIOLOGY
Chromosomal abnormalities

Trisomy – VSD most common

Di George – abnormalities in trunk e.g. TGA, truncus
arteriosus; Shprintzen (velocardiofacial) syndrome

Conotruncal lesions – involves your truncus arteriosus;
VSD, branchial arch defect

Dilated Cardiomyopathy

Long QT syndrome
Get thorough family history once you have a patient with CHD;
parents should go into genetic counseling. Most of the time if you
have a patient with ASD, ask the siblings, they may have an
increased incidence of another patient/sibling with congenital heart
disease.
BASIC TOOLS IN CARDIOLOGIC EVALUATION OF INFANTS AND
CHILDREN

History

PE

CXR

15 lead ECG

2DED

3D, TEE, Fetal echo

Others: CBC, Exercise testing, MRI, CT Angio/Angiography

CATEGORIES OF CHD:
1. Acyanotic

Shunts – most common is PDA, ASD, VSD and AVSD

Obstructive lesions

Regurgitant lesions
2.

Cyanotic e.g. DORV (double outlet right ventricle) – the
aorta and pulmonary artery arises from the right ventricle,
the blood in your right ventricle is deoxygenated, your
aorta will also receive the deoxygenated blood
ACYANOTIC HEART DISEASE

Volume overload

Pressure overload

L → R Shunts
Qp:QS 2:1
Heart Failure
Aorta
↑ Catecholamines

Pulmonary stenosis
Aortic stenosis
Coarctation of

Qp:Qs > 2:1 = manifest congestion; quantity of pulmonary flow
against the quantity of systemic flow; Qp of 2 – twice of your blood
goes to your pulmonaries compared to the systemic. Supposedly the
normal value is 1:1. There is an equal amount of blood that goes to
your pulmonary and systemic circulation but if your Qp:Qs is > 2:1,
you have greater blood volume going to the lungs and you have
more chances of pulmonary congestion. You will also manifest heart
failure and also in these patients there is an increase in the
catecholamines. That is why they are tachycardic just like the VSD;

Page 4 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra
most of them are diaphoretic and have a fast heart beat because of
the release of your catecholamines secondary to the sympathetic
stimulation.

Valvular regurgitation
MR
TR
AI
PR
Insufficiency is the same with regurgitation (backflow) e.g. mitral
valve should close during systole for unidirectional flow of blood.
During systole, if your mitral valve is open, some of your blood will
go back to the LA.

DCM = ↓ heart/myocardial function, there will now be atrial and
ventricular overloading producing pulmonary edema
↑ atrial and ventricular filling pressure → pulmonary
edema
Under pressure overload:
Stenosis – inadequate opening of your heart valves
↑ pulmonary blood flow
flow
LVH

↓ pulmonary blood

RVH

VSD
PDA
AVSD

ASD
PAPVR
Eisenmenger’s Phenomenon

You may diagnose the patient by just looking at the x-ray.
LEFT TO RIGHT SHUNTS

Atrial Septal Defect

Ventricular Septal Defect

Patent Ductus Arteriosus

Atrioventricular Septal Defect
ATRIAL SEPTAL DEFECT
Interruption between your interatrial septum. They are
divided into 3: primum (bottom), segundum (middle) and
sinus venosum (top)




Persistent communication between the RA and LA
7% of CHD
Can occur anywhere in the atrial septum



Physiologic consequences depend on:
 location
 size
 association with other anomalies
Determinants of L  R shunt
 size of defect – very important to have
a manifestation. The bigger the defect,
the more manifestations.
 relative compliance of RV /LV
Shunt flow occurs in systole and diastole
RA and RV overload – the blood from RA, RV, lungs, LA and
then back to RA. You will have more blood going to the






right side of your heart leading to RA and RV enlargement.
On ECG: p wave produced by atrial contraction; enlarged
P/wide P think of RAE.
Laboratory Findings
 CXR :  PVM; the normal vascularity should
only up to the middle, if your lung is divided
into three: inner zone, middle zone and
outer zone. If in the outer zome you can see
blood vessels think of increase in the
pulmonary blood flow; patient has RVE; ASD
looks like an elephant trunk; cardiomegaly
RVH: more than 2/3 of retrosternal area;
the space is occupied by your right
ventricle.
 ECG : RAE, RVH
RBBB pattern in V1
 ECHO : size and location of defect
size of RA and RV
amount and direction of shunt

PAPVR (partial anomalous pulmonary venous return): normally
your pulmonary vein drains to the LA, draining elsewhere would lead
to PAPVR. Most of the time you have an ASD. If you have a sinus
venosus, look for PAPVR, 90% of the time they have PAPVR
Papvr vs tapvr
Associated lesion: ASD (sinus venosus)
Scimitar syndrome – see it like a Turkish sword; enlarged left
pulmonary artery
VENTRICULAR SEPTAL DEFECT

Most common CHD =25%

Defect can occur anywhere along the interventricular
septum

Enlargement of LV because most of the VSD, the hole is
very near your pulmonary artery. So when the LV
contracts, the blood immediately goes to the pulmonary
artery. It does not stay in your RV unlike in ASD where
blood stays in your RV. There is more effort on the left side
of the heart to pump more blood. So there is ventricular
hypertrophy and ventricular dilation in VSD.

Page 5 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra


CLASSIFICATION OF VSD
1. Perimembranous – most common, 80%
2. Outlet – 5-7%
3. Inlet – 5-8%
4. Muscular – 5-20%
a. central
b. apical
c. marginal
d. “swiss cheese” septum – multiple muscular



Determinants of L  R shunt
1. Size of VSD
2. Difference in resistance between pulmonary
systemic circulation
3. Difference in pressure between RV and LV
Shunt flow occurs in systole – from beginning of systole to
end of systole you will hear the sound. That is why it is
called a pansystolic murmur.
Biventricular – will occur late because in the long run if
there is continuous increase in pulmonary blood flow your
pulmonary artery is sclerosed so there will be thickening
producing an increase in your PVR so there will be an
increase in your PAP – RV has to compensate by increasing
in size.
Laboratory Findings
CXR :  PVN, cardiomegaly
ECG : LAE, LVH
ECHO : location/size of VSD
LA and LV size
Amount and direction of shunt
Estimate PA pressure
UNTREATED VSD
 irreversible changes take place in the
pulmonary arterioles
= Pulmonary vascular obstructive
disease (Eisenmenger’s disease) late stage of VSD






VSD


and













↑ PVR -- ↓ L → R shunt
Signs/Sx: cyanosis- if patient develops
cyanosis
and
pulmonary
arterial
hypertension before they would only last
for about 2 years more after the onset of
pulmonary hypertension; but with the
outbreak of medicine there is Sildenafil –
very effective in decreasing pulmonary
arterial pressure (0.25 mg/kg/day; paper
tab 100 mg divided into 1 paper tabs to
make 10 mg/paper tab); Milrnone increases
both cardiac contractility and decreases
pulmonary arterial pressure.
loud, single S2
CXR : RVH

PATENT DUCTUS ARTERIOSUS

5-10% of CHD

Communication between the aorta and pulmonary artery

Risk factors : prematurity, rubella : congenital rubella
syndrome – ccongenital cataract with PDA, with mental
retardation/delay, sometimes Down syndrome

Left enlarges: RA, RV, pulmonary artery (after birth it
should be left to right) goes to pulmonary vein then to the
left, LA to LV back to PDA, back to lungs , pulmonary veins,
LA, LV and so on – more volume on the left, never goes to
the right

Determinants of L  R shunt
1. length and diameter of ductus
2. Relative resistances of Ao and PA
Shunt flow occurs in systole and diastole – continuous
nurnur
Laboratory Findings
CXR :  PVM, cardiomegaly
ECG : LAE, LVH
ECHO : size of ductus and gradient
estimate PA pressure
UNTREATED PDA
 PVOD
 bidirectional shunting : differential
cyanosis – increase PAP, increase PVR –
Eisenmenger phenomenon, if not treated
you will develop pulmonary arterial
hypertension or Esienmenger phenomenon
(↑ PAP, ↑PVR and cyanosis)
 PE : loud single S2
 CXR : normal heart size;vdilated central and
main
PA
 ECG : RVH
ACYANOTIC HEART DISEASE
↑ PBF

↓ PBF

LVH

RVH

LVH

RVH

PDA
PS
VSD
PS
AVSD

ASD

AS

VSD

PAPVR

COA

W/

PERIPHERAL

EISSEMENGER

Congenital MS – produces LAE, you have a small MV, the blood from
the LA cannot pass through, it has increased pressure, as pressure
increases it will be reflected to the lungs = pulmonary edema, RVH in
the long run. When you develop heart faiure, the right side of the
heart enlarges and you are going to have hepatomegaly.
Aortic stenosis – small aortic valve or there is an obstruction before
your aortic valve; during systole LV will contract, the blood will not
be able to go or ass through your aortic valve because it is small or is
obstructed, LV has to increase in pressure, LV enlarges.
Coarctation of the Aorta – stenosis in your aorta; the most common
COA is type 1; after third branch is the first (type 1), after second
branch is second (type 2), after the first branch the third (type 3); if
ypu have a constriction, the blood from the aorta, the pressure is
reflected to the LV, has to increase in pressure, in size to push the
blood and pass through the constriction
VSD with PS – the patient would have developed cyanosis unless the
patient is having right sided heart failure. Since you have increased
pressure on the left side, even if you have stenotic valve still the
blood will be pushed to the pulmonary artery. But if you have
pulmonary valve atresia, the blood from the right will go to the
aorta, no cyanosis; TOF has cyanosis because there is malposition of
your great vessels
Peripheral pulmonary stenosis – the pulmonary arteries which ais
very near your lungs are stenosed




SHUNTS ( Left to Right )
OBSTRUCTIVE VALVULAR LESIONS – may have LVH or RVH
REGURGITANT VALVULAR LESIONS
ACYANOTIC HEART DISEASE

Volume overload

Pressure overload

Valvular regurgitation
MR
TR
AI
PR
Page 6 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

Mitral Regurgitation – LA and LV enlarge
Aortic Insufficiency – LV alone enlarges
Tricuspid Regurgitation – RV and RA
Pulmonary Regurgitation – RV alone
CONGENITAL HEART DISEASE

Categories of CHD
1. ACYANOTIC
Shunts
Obstructive Lesions
Regurgitant Lesions
2. CYANOTIC
CYANOTIC CHD
↓Blood flow

↓PBF + RL shunt
e.g. TOF – most common
common





↑ Blood flow

Abnormal VA connections
e.g TGA –

most

Total mixing
TETRALOGY OF FALLOT

Large VSD

Overriding of the aorta over the septal defect – your aorta
is directly above your VSD

RVOTO (right ventricular outflow tract obstruction); conus
cordis becomes ventricular outflow tract; conus on the left
absorbed; long outflow tract on right because conus on
right is not absorbed; sometimes outflow tract
hypertrophies producing stenosis in the right ventricular
outflow tract

RVH



PHYSIOLOGY OF TOF

Normal O2 sat on the right is about 60% (60 – 65); same amount of
O2 sat will go to RV and then mixes with the high O2 in the left.
Patients with TOF are desaturated.


Clinical Manifestation – symptoms will depend on severity
of outflow tract obstruction.

Mild RVOTO (pink tetralogy)
 (+)signs of pulmonary overcirculation due to
L-R shunt at VSD
 Normal sPO2 (pink tetralogy)
 CHF occurs with normal decrease in PA
pressure during first week of life

Moderate RVOTO
 Near-normal Qp:Qs, sPO2 low 90’s
 Patient are asymptomatic

Severe RVOTO
 Significant R-L shunting across the VSD
 sPO2 at 70’s
Newborns/infants- diagnosed when they present with
cyanosis or systolic ejection murmur; cyanosis usually
presents at 1 month of age in TOF but if you have
pulmonary atresia you will have cyanosis at birth
Mortality rates:

PS- 30% at 6 mos, 50% by 2 yrs

PA- 50% by 1 yr, 85% by 5 yrs

CHF- seen in large PDA’s or aortopulmonary collateral
arteries

In TOF with PA, decreasing O2 sats in neonate
indicate a closing PDA

(+)MAPCAs (major aorto pulmonary arterial collateral
e.g. severe pulmonary stenosis, the body will
compensate, if you do not have a PDA the body has
to find a way to oxygenate its lungs, there will be an
angiogenesis from the aorta in the whole descending
aorta which will go to your lungs to oxygenate lungs;
very dangerous prone to rupture, may die of
bleeding) - alleviates cyanosis to a variable degree,
may tend to develop stenosis or PVOD in
unobstructive MAPCAs

Clinical Manifestation
 Hypercyanotic episodes (Tet Spell) –
secondary to pulmonary vasospasm –
pulmonary artery, more cyanosis, acidosis,
may die of hypoxemia; have to break cycle
of cyanosis and vasospasm
 Characterized by severe and prolonged
decrease in arterial saturation cause by
abrupt changes in Qp:Qs (Inc R-L shunting)
 Caused by sudden decrease in systemic
vascular resistance or dynamic changes in
the degree of subpulmonic obstruction
 Irritability, hyperpnea, marked cyanosis,
pallor, lethargy of unconsciousness
 (+)severe hypoxia secondary metabolic
acidosis  hyperpnea/ inc in pulmonary
resistancemore R->L shunting  brain
damage
 Breaking the cycle – IMPORTANT

Physical Examination
 Reflect combination of PS, right ventricular
hypertension, and any R-L shunt
 Infants are generally full-sized although
growth failure may occur overtime; most
infants are chubby as compared to your
shunt anomalies they are cachectic.
 Cyanosis of varying degree – depends on
degree of RVOTO
 Accentuated RV impulse
 S2 single and loud
 Widened pulse pressure if with PDA,
collaterals or shunt
 Murmur- harsh systolic – secondary to
pulmonary stenosis

LUSB in location

crescendo-decrescendo

intensity of the murmur
inversely parallels the degree
of pulmonic obstruction

occasional AR murmur

continuous murmur- PDA
 TOF with PA- no murmur

Diagnostics
 Parasternal Long-Axis

Large VSD and overriding great
vessel

Associated AI is appreciated
 Short-axis view

Page 7 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra






Info regarding infundibulum and
proximal pulmonary artery

Color flow- antegrade flow into
Pas

Coronary artery anatomy
 Apical four-chamber
 Clear image of perimembranous VSD and
relationship to TV and AV
 Cardiac catheterization – not done
routinely; only done for intervention; ECG
can diagnose well

Done if echo has been unable to
identify coronary anatomy clearly

alternate sources of PBF, provide
thorough description of central
PA, ductus, MAPCA and stenosis
of MAPCA

Assess degree of distortion of PAs
caused by the shunt

Measure McGoon ratio

Interventional:
1) balloon valvuloplasty
2) coil embolization of
MAPCAs
 CXR

Overall heart size is normal

Boot shaped heart – secondary to
RVE; RVH – upliftment of cardiac
silhouette

Vascularity is diminished in
proportion to the degree of
cyanosis
 Hematologic

Polycythemia

Clotting factors, platelet count
and fibrinogen levels are reduced
– deranged clotting system,
proloned PT and PTT and low
platelet count
 Echocardiography

Mainstay of diagnosis for
intracardiac anatomy
Palliative Procedure – done in very small patient with
very small pulmonary artery
 Modified BTS (Blalock-Taussig shunt) and
Central Shunts

Encourage further arterial growth
in severe pulmonary hypoplasia
 Drawbacks:

Pulmonary artery distortion

Additional ventricular overloading

Surgical risk with attendant
Post-Operative Care
 Causes of Significant residual VSD :

RV dysfunction sec to RV volume
loading

PR after relief of outflow tract
obstruction

LA dysfnction sec to LV volume
loading
 Low cardiac output

Related to size of ventricular
incision

Poor compliance of RV after vent
incision

Sinus tachycardia- hallmark
 Dysrhythmias

CHB

RBBB- seen in nearly all patients
with a ventriculotomy

JET- related to degree pof RV
dysfunction

must have a patent foramen ovale so that blood can go to LV to LA,
aorta then the lungs; PDA is very important in this patient, if it
closes, the patient dies; do emergency BTS, if interatrial
communication is not present we cut through it (Blalock Hanlon
technique) or balloon atrialseptostomy; diagnose with 2 D echo
TRICUSPID VALVE ATRESIA – no tricuspid valve, blood goes from
IVCto RA to LA; do balloon atrialseptostomy
Pathology






Atretic TV
 Dimple floor of the RA
 Membrane

Usually muscular; may be fibrous
Interatrial Communication
 Usually wide PFO
 May be ASD secundum; rarely ostium
primum
VSD
 Usually perimembranous

Classification of TVA

Type I (70%)
 normally related great arteries
 occurs in 70% to 80% of patients with TVA

Type II (28%)
 d-transposition of the great arteries
 occurs in 12% to 25% of patients

Type III (3%)
 uncommon form of TVA (3% to 6% of
patients)
 used by some authors for those patients
born with more complex associated lesions
such as l-transposition or malposition of the
great arteries
Normally Related Great Arteries

Transposition of the Great Arteries

PVA IVS (pulmonary valve atresia with intact septum) – you do not
have a flow from your RV to the pulmonary artery plus you have
pulmonary valve atresia; IVS means you do not have an intact
ventricular septum blood from RA to RV will go back to your RV, you
Page 8 of 9

PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra
DORV PS(double outlet right ventricle) – pulmonsry artery and
aorta arise to the RV
TGA PS

TRUNCUS ARTERIOSUS – truncus does not septate and spiral, you
only have one outflow tract, a single trunk arises from your RV and
LV VSD is important.; Management depends on what anatomy the
patient has.

EBSTEINS ANOMALY
CYANOTIC CHD W/↓PBF

TGA W/O PS

HLHS (hypoplastic left heart syndrome) – you do not have left side
of the heart, you have a very small aorta, only have large pulmonary
artery


DORV W/O PS



TAPVR

TAPVR- Classification
A. Based on the anatomic site of the abnormal connection
I. Supracardiac (40-50%)
II. Cardiac (18-31%)
III.Infracardiac (13-24%)
IV.Mixed (5-10%)







GOD BLESS!


Supracardiac TAPVC to LIV
 Individual PV form a horizontal venous
confluence (HVC) that connects to LIV via
the vertical vein
Infradiaphragmatic TAPVC to the portal vein
 The PV forms a vertical confluence that
descends below the diaphragm and
typically joins the portal vein
 PV blood enters the IVC via the ductus
venosus or hepatic sinusoids
TAPVC to the coronary sinus (CS)
Mixed type TAPVC
 Left PV connect to the LIV and the right PV
connect with the coronary sinus, in this
example

Page 9 of 9

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