PEDIATRIC CARDIOLOGY – Dr.
Marivic Montilla - Esguerra
CARDIAC ANATOMY
EMBRYOLOGY
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.
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.
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Atrial septation occurs at about the 28 day of fetal life.
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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
a membranous type because the membranous septum is the last to
close.
AORTIC ARCH DEVELOPMENT
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.
st
1 aortic arch (R and L) – maxillary artery
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2 aortic arch (R and L) – hyoid stapedial arteries
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3 aortic arch – common artery common carotids (internal and
external)
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4 aortic arch – part of subclavian arteries
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5 aortic arch – will not develop
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6 arch – left pulmonary artery and ductus arteriosus
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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
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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.
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. 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)
2. R→L vs L→R shunt via PDA; from RV to PA, ductus
arteriosus to descending aorta (right to left)
3. Pulmonary vasculature constrict more in response to
hypoxemia, hypercapnea, acidosis
4. Ventricular thickness RV = LV; more of LV as compared to
RV
5. High O2 consumption → high CO; baby very dependent in
blood volume
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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
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the foramen ovale. Repaeat 2D echo on 3 month.
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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.
Hx – 80% you can diagnose the case
- Prenatal
- Medications
- Exposures – esp. congenital; term cyanotic delivery:
exposure to Rubella, PDA in congenital rubella syndrome;
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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;
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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)
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- Palpation – apical impulse: normal location is 4 ICS;
substernal thrust = Right Ventricular Enlargement; thrill –
palpable murmur
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- Percussion – very rarely done; dull sound in 6 ICS or
axillary area in adults – RVE
- Auscultation – done immediately when patient is quiet
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1 month old baby with apical impulse on 5 ICS = abnormal; apex
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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
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PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

delay in the closure of your pulmonary valve. P2 delayed during deep
inspiration.
Physiologic splitting – delayed closure of pulmonic valve
MURMURS
- abnormal sounds heard on patients chest
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
The QRS amplitude would tell you if you have a tall R it depends on
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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

Grade I – barely audible P wave = normal axis is 90

Grade II – medium intensity
Grade III – loud but no thrill QRS complex – ventricular hypertrophy; also tells us of the systolic
Grade IV – loud murmur with a thrill overload pattern if you see tall PQR in right precordial leads (V1) or
Grade V – very loud diastolic overload pattern – RSR in primum ASD.
Grade VI – hear murmur even without placing stethoscope
Hematologic = request as needed
Look for timing: Patient severely cyanotic = request for CBC to know if the patient is
- Systolic Polycythemic. The normal Hct in a cyanotic patient is up to 0.6.
- Diastolic Above that we do phlebotomy.
- Pansystolic – murmur heard all throughout of systole;
most commonly produced by VSD 3D echo
- Continuous – murmur heard all throughout systole and TEE (transesophageal echocardiogram) – insert a probe through
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diastole; most commonly heard among patients with PDA your esophagus at the level of your heart, 4 intercostal; better
(machinery murmur) done in patients for operation because they are sedated
- Innocent – musical like sound heard in the heart secondary
to IDA, febrile patient Fetal echocardiography
Exercise testing – Stress test
Mitral regurgitation = heard during systole; AV valve not fully closed MRI/CT
Diastole = Mitral stenosis Radionucleide study
CT angiography – evaluate the structures not seen during echo, in
LAB EVALUATION patients with double aortic arch, long segment coarctation of the
aorta, evaluate peripheral stenosis; insert probe in inguinal area
- Good Hx towards the heart and get the pressures to know if patient is
- Good PE operable or not
- CXR – PA view or lateral view; right side: moguls – RV on
anterior; on right SVC, RA, RV; LV will not produce a Angiogram put a dye to see if there is stenosis, get pressures within
shadow on PA view because it is located posteriorly; heart
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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
CONGENITAL HEART DISEASE

Pure R in neonate – RVE PREVALENCE

- 0.5 – 0.8% of live births
Difference between pediatric and adult ECG: - Higher in stillborn 3 – 4%
1. HR – tachycardia accepted in newborn and gradually ↓ - Spontaneous abortions 10 – 25%; most of the patients that
with age; secondary to maturity of sympathetic nervous acquire congenital heart diseae in the first trimester of
system; HR dependent on ANS pregnancy spontaneously abort
2. RVH – in utero work of right ventricle is greater; at birth in - Premature 2%
↓ RVSP and ↑ in systemic resistance: LV becomes thicker
than RV VSD = most common; perimembranous VSD
3. Right axis deviation – may remain up to 1 year of age up to perimembranous septum – last to close in the formation of
135˚; patient 1 y/o with axis of 150 = abnormal ventricular septation

Routine ECG interpretation: Relative Frequency of Major Congenital Heart Lesions

LESION % OF ALL LESIONS
Rhythm – sinus or non-sinus Ventricular septal defect 35–30
Sinus: followed by a QRS with a fixed PR interval
Atrial septal defect (secundum) 6–8
Patent ductus arteriosus 6–8
HR: compute for atrial and ventricular rate; in patients with atrial
fibrillation or atrial flutter, your atrial rate is faster than your Coarctation of aorta 5–7
ventricular rate or in patients with complete heart block your atrial Tetralogy of Fallot 5–7
rate is slower than your ventricular rate or vice versa. Pulmonary valve stenosis 5–7
Aortic valve stenosis 4–7
You plot your QRS and T axis, measure QR interval; for any patient d-Transposition of great arteries 3–5
you always measure the PR, QRS and QT. Hypoplastic left ventricle 1–3
Hypoplastic right ventricle 1–3
Prolongation of your PR will tell you that you may have a block – AV
Truncus arteriosus 1–2
block first degree, second degree, third degree, complete heart
block Total anomalous pulmonary venous return 1–2
Tricuspid atresia 1–2
QRS if you have PVCs; normal QRS is 0.08; for pediatric group the Single ventricle 1–2
normal PR is only 0.16 seconds, adults up to 0.2 Double-outlet right ventricle 1–2
Others 5–10
QT for pediatric group is up to 0.44. We compute for the QTc *
Excluding patent ductus arteriosus in preterm neonates, bicuspid
(corrected QT) using the Bechet’s formula.The P wave amplitude
aortic valve, physiologic peripheral pulmonic stenosis, and mitral
and the duration will tell you if you have left atrial enlargement or
valve prolapse.

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PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

VSD > ASD > PDA etc.; most complex are least common ETIOLOGY
Sometimes CHD is diagnosed even at birth; sometimes diagnosed
late Chromosomal abnormalities
There are certain diseases that do not manifest early. If you hear a  Trisomy – VSD most common
murmur at birth, you should be alarmed because most of the  Di George – abnormalities in trunk e.g. TGA, truncus
murmurs heard at birth are those that are PDA dependent. arteriosus; Shprintzen (velocardiofacial) syndrome
Sometimes there is the late manifestation of the symtptoms just like  Conotruncal lesions – involves your truncus arteriosus;
your Tetralogy of Fallot. Your baby will not get cyanotic immediately. VSD, branchial arch defect
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The cyanosis in TOF will manifest at 3 month of age  Dilated Cardiomyopathy
 Long QT syndrome
LATE DIAGNOSIS OF CHD
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 Late manifestation of symptoms e.g. TOF – cyanosis 3 Get thorough family history once you have a patient with CHD;
month of age because during this time the pulmonary parents should go into genetic counseling. Most of the time if you
vascular resistance is going down or the PDA is closing have a patient with ASD, ask the siblings, they may have an
 Hemodynamic effects of transitional circulation – there is a increased incidence of another patient/sibling with congenital heart
drop in PVR after the baby breathes, SVR gradually disease.
increases after first breath; if you have a high PAP, high
PVR if patient has PDA your murmur will not be heard. But BASIC TOOLS IN CARDIOLOGIC EVALUATION OF INFANTS AND
once the systemic vascular resitance overcomes your CHILDREN
pulmonary vascular resistance, the continuous murmur of  History
your PDA can now be appreciated.  PE
 Shunt anomalies size of defects → very important:  CXR
 VSD - smaller the size, more audible is the murmur  15 lead ECG
because there is a great difference in the pressue of  2DED
the right and the left ventricle. Your LV is ~ 80 – 120,  3D, TEE, Fetal echo
that is your systolic pressure. Your RV has about a  Others: CBC, Exercise testing, MRI, CT Angio/Angiography
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.

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 Pulmonary stenosis

Qp:QS 2:1 Aortic stenosis
Heart Failure Coarctation of
Aorta
↑ Catecholamines

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;

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PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

most of them are diaphoretic and have a fast heart beat because of right side of your heart leading to RA and RV enlargement.
the release of your catecholamines secondary to the sympathetic On ECG: p wave produced by atrial contraction; enlarged
stimulation. P/wide P think of RAE.
 Laboratory Findings
 CXR :  PVM; the normal vascularity should
Valvular regurgitation only up to the middle, if your lung is divided
MR TR into three: inner zone, middle zone and
AI PR outer zone. If in the outer zome you can see
blood vessels think of increase in the
Insufficiency is the same with regurgitation (backflow) e.g. mitral pulmonary blood flow; patient has RVE; ASD
valve should close during systole for unidirectional flow of blood. looks like an elephant trunk; cardiomegaly
During systole, if your mitral valve is open, some of your blood will RVH: more than 2/3 of retrosternal area;
go back to the LA. the space is occupied by your right
ventricle.
 ECG : RAE, RVH
DCM = ↓ heart/myocardial function, there will now be atrial and RBBB pattern in V1
ventricular overloading producing pulmonary edema  ECHO : size and location of defect
↑ atrial and ventricular filling pressure → pulmonary size of RA and RV
edema amount and direction of shunt

Under pressure overload: PAPVR (partial anomalous pulmonary venous return): normally
Stenosis – inadequate opening of your heart valves 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
↑ pulmonary blood flow ↓ pulmonary blood venosus, look for PAPVR, 90% of the time they have PAPVR
flow Papvr vs tapvr
Associated lesion: ASD (sinus venosus)
LVH RVH
Scimitar syndrome – see it like a Turkish sword; enlarged left
pulmonary artery
VSD ASD
PDA PAPVR VENTRICULAR SEPTAL DEFECT
AVSD Eisenmenger’s Phenomenon  Most common CHD =25%
 Defect can occur anywhere along the interventricular
You may diagnose the patient by just looking at the x-ray.
septum
 Enlargement of LV because most of the VSD, the hole is
LEFT TO RIGHT SHUNTS
very near your pulmonary artery. So when the LV
 Atrial Septal Defect
contracts, the blood immediately goes to the pulmonary
 Ventricular Septal Defect
artery. It does not stay in your RV unlike in ASD where
 Patent Ductus Arteriosus
blood stays in your RV. There is more effort on the left side
 Atrioventricular Septal Defect
of the heart to pump more blood. So there is ventricular
hypertrophy and ventricular dilation in VSD.
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

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PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

 CLASSIFICATION OF VSD  Determinants of L  R shunt

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
VSD
 Determinants of L  R shunt
1. Size of VSD
2. Difference in resistance between pulmonary
and 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
 ↑ 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
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 ASD AS VSD W/
PS
VSD PAPVR COA PERIPHERAL
PS
AVSD 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

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PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

 Moderate RVOTO
Mitral Regurgitation – LA and LV enlarge  Near-normal Qp:Qs, sPO2 low 90’s
Aortic Insufficiency – LV alone enlarges  Patient are asymptomatic
Tricuspid Regurgitation – RV and RA  Severe RVOTO
Pulmonary Regurgitation – RV alone  Significant R-L shunting across the VSD
 sPO2 at 70’s
CONGENITAL HEART DISEASE  Newborns/infants- diagnosed when they present with
 Categories of CHD cyanosis or systolic ejection murmur; cyanosis usually
1. ACYANOTIC presents at 1 month of age in TOF but if you have
Shunts pulmonary atresia you will have cyanosis at birth
Obstructive Lesions  Mortality rates:
Regurgitant Lesions  PS- 30% at 6 mos, 50% by 2 yrs
2. CYANOTIC  PA- 50% by 1 yr, 85% by 5 yrs
 CHF- seen in large PDA’s or aortopulmonary collateral
CYANOTIC CHD arteries
↓Blood flow ↑ Blood flow  In TOF with PA, decreasing O2 sats in neonate
indicate a closing PDA
 (+)MAPCAs (major aorto pulmonary arterial collateral
↓PBF + RL shunt Abnormal VA connections e.g. severe pulmonary stenosis, the body will
e.g. TOF – most common e.g TGA – most compensate, if you do not have a PDA the body has
common to find a way to oxygenate its lungs, there will be an
Total mixing angiogenesis from the aorta in the whole descending
aorta which will go to your lungs to oxygenate lungs;
TETRALOGY OF FALLOT very dangerous prone to rupture, may die of
 Large VSD bleeding) - alleviates cyanosis to a variable degree,
 Overriding of the aorta over the septal defect – your aorta may tend to develop stenosis or PVOD in
is directly above your VSD unobstructive MAPCAs
 RVOTO (right ventricular outflow tract obstruction); conus  Clinical Manifestation
cordis becomes ventricular outflow tract; conus on the left  Hypercyanotic episodes (Tet Spell) –
absorbed; long outflow tract on right because conus on secondary to pulmonary vasospasm –
right is not absorbed; sometimes outflow tract pulmonary artery, more cyanosis, acidosis,
hypertrophies producing stenosis in the right ventricular may die of hypoxemia; have to break cycle
outflow tract of cyanosis and vasospasm
 RVH  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
 PHYSIOLOGY OF TOF
 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
Normal O2 sat on the right is about 60% (60 – 65); same amount of of pulmonic obstruction
O2 sat will go to RV and then mixes with the high O2 in the left.
 occasional AR murmur
Patients with TOF are desaturated.
 continuous murmur- PDA
 TOF with PA- no murmur
 Clinical Manifestation – symptoms will depend on severity
 Diagnostics
of outflow tract obstruction.
 Parasternal Long-Axis
 Mild RVOTO (pink tetralogy)
 Large VSD and overriding great
 (+)signs of pulmonary overcirculation due to
vessel
L-R shunt at VSD
 Associated AI is appreciated
 Normal sPO2 (pink tetralogy)
 Short-axis view
 CHF occurs with normal decrease in PA
pressure during first week of life

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 Info regarding infundibulum and must have a patent foramen ovale so that blood can go to LV to LA,
proximal pulmonary artery aorta then the lungs; PDA is very important in this patient, if it
 Color flow- antegrade flow into closes, the patient dies; do emergency BTS, if interatrial
Pas communication is not present we cut through it (Blalock Hanlon
 Coronary artery anatomy technique) or balloon atrialseptostomy; diagnose with 2 D echo
 Apical four-chamber
 Clear image of perimembranous VSD and TRICUSPID VALVE ATRESIA – no tricuspid valve, blood goes from
relationship to TV and AV IVCto RA to LA; do balloon atrialseptostomy
 Cardiac catheterization – not done
routinely; only done for intervention; ECG Pathology
can diagnose well  Atretic TV
 Done if echo has been unable to  Dimple floor of the RA
identify coronary anatomy clearly  Membrane
 alternate sources of PBF, provide  Usually muscular; may be fibrous
thorough description of central  Interatrial Communication
PA, ductus, MAPCA and stenosis  Usually wide PFO
of MAPCA  May be ASD secundum; rarely ostium
 Assess degree of distortion of PAs primum
caused by the shunt  VSD
 Measure McGoon ratio  Usually perimembranous
 Interventional:
1) balloon valvuloplasty Classification of TVA
2) coil embolization of  Type I (70%)
MAPCAs  normally related great arteries
 CXR  occurs in 70% to 80% of patients with TVA
 Overall heart size is normal  Type II (28%)
 Boot shaped heart – secondary to  d-transposition of the great arteries
RVE; RVH – upliftment of cardiac  occurs in 12% to 25% of patients
silhouette  Type III (3%)
 Vascularity is diminished in  uncommon form of TVA (3% to 6% of
proportion to the degree of patients)
cyanosis  used by some authors for those patients
 Hematologic born with more complex associated lesions
 Polycythemia such as l-transposition or malposition of the
 Clotting factors, platelet count great arteries
and fibrinogen levels are reduced
– deranged clotting system, Normally Related Great Arteries
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 Transposition of the Great Arteries
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

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

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PEDIATRIC CARDIOLOGY – Dr. Marivic Montilla - Esguerra

DORV PS(double outlet right ventricle) – pulmonsry artery and TRUNCUS ARTERIOSUS – truncus does not septate and spiral, you
aorta arise to the RV only have one outflow tract, a single trunk arises from your RV and
LV VSD is important.; Management depends on what anatomy the
TGA PS 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 GOD BLESS!
I. Supracardiac (40-50%) 
II. Cardiac (18-31%)
III.Infracardiac (13-24%)
IV.Mixed (5-10%)

 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

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