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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 Mechanism of aortic arch selection which is probably related to
the 18th day there is the appearance of a plate. They are two: a redistribution of blood flow. The aortic blood vessels are devoid of
tissue that becomes hollow to form a pair of endothelial tubes, smooth muscles initially and therefore incapable of active
these will fuse to form your primitive cardiac tube. 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 st
1 aortic arch (R and L) maxillary artery
Morphologic stage nd
2 aortic arch (R and L) hyoid stapedial arteries
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3 aortic arch common artery common carotids (internal and
The morula and the plate will fuse to form your primitive heart. They external)
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all came from angiogenic cell clusters. At 22 days of age, there are 2 4 aortic arch part of subclavian arteries
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pairs of tubes. 5 aortic arch will not develop
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6 arch left pulmonary artery and ductus arteriosus
Divisions of primitive heart:
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1. Truncus becomes aorta If there is an abnormality in the absorption of your 6 aortic arch
2. Bulbus cordis divided into 2 you are going to have your persistent/patent ductus arteriosus.
3. Old ventricle
4. Atrium CARDIAC DIFFERENTIATION

Bulbus cordis becomes corus cordis and bulbus cordis; becoming Totipotential cells would tell what type of cell it will go into
left and right ventricles. Atria will be divided into 2: the right and the differentiation. Signal a specific cell lineage. Induction regulates
left atria, and then the sinus venosus. formation of cell lineage.
Truncus forms aorta and pulmonary artery.
Bulbus right ventricle Developmental changes in cardiac formation:
Conus cordis becomes outflow tract for both ventricles. The right Hyperplasia prenatal life
and the left ventricle will have its own outflow tract. However the Hypertrophy postnatal life
conus in the left ventricle will be absorbed unlike in the right Fetal infant/heart sensitive to Ca
ventricle, it has a long outflow tract because the conus on the right Sensitive to preload and afterload
is not absorbed.
Primitive ventricle will become left ventricle. Na and Ca channel is only found in cardiac muscles.
Fetal myocardium very sensitive to Calcium which is mainly stored in
CARDIAC LOOPING the endoplasmic reticulum. However the t tubule is open to
extracellular area. Calcium can easily go in. Ca channel blockers is
Straight tube will loop prior to cardiac looping. never given to 6 y/o and below.
During cardiac looping: D loop or L loop SVT (supraventricular tachycardia) Verapamil given in adults never
D loop normal looping given in children 6 y/o and below(calcium antagonist)
L loop dextrocardia or ventricular inversion During resuscitation, part of regimen given is Calcium gluconate,
because it can help in cardiac contractility of myocardium. patients
Atria will go up, rotate spirally. It will be placed at the back of your with low calcium may produce bradycardia. Any imbalance in
truncus. Ventricle will be left on its place. Atria will be on upper part, electrolyte may cause danger to fetal myocardium.
ventricle on lower part. Bulbus cordis would be placed on the Pediatric patient with diarrhea easily go into cardiac distress
anterior, on the left side = normal looping
FETAL CIRCULATION
If you have an exposure to any authentic agent that may interrupt
the embryogenesis, it will produce congenital heart disease. It In parallel
depends on what stage the heart is being formed.
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Umbilical and pulmonary vein = only 2 that carry oxygenated blood.
If you have an infection on the 22 day where you do not have yet From the placenta which is high in pO2, it will go to umbilical vein,
the valves, you may have a patient with just a univentricular heart. then to ductus venosus 50% of which will go to the portal circulation
The earliest time that the mother has the infection, the worse is the and the remaining goes to the right atrium with the help of a
heart disease. structure directing blood to the foramen ovale, to the left atrium,
left ventricle then to the aorta. Blood from SVC which is less
CARDIAC SEPTATION 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
You forming the interatrial septum. During the formation of the pulmonary arterial pressure, the lung is collapsed. The blood will go
interatrial septum,, initially you have a common atrium then you will to the ductus arteriosus and then to the descending aorta, to the
form a septum. The first one, upper one will outpouch. Septum circulation and then back to the placenta via the umbilical artery.
primum is the first to form. The hole between your endocaridal
cushion and your septum primum is your ostium primum. Primum PRESSURES
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 In patients with Congenital Heart Disease, you always get the oxygen
that septum coalesce to form you foramen ovale. Your foramen saturation and intracardial pressures to know the flow of blood,
ovale is important in the cardiac cycle because your fetus has a compute for pulmonary vascular resistance.
parallel circulation. IVC most of your blood will cross the foramen ovale and then to
the left side aorta.
Hole created is called foramen segundum/ostium segundum. From the SVC, 21% actually O2 sat only about 15% goes to right
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ventricle, then to the PDA.
Atrial septation occurs at about the 28 day of fetal life.
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Ventricular septation occurs on 25 day of fetal life. After birth, your baby goes into Transitional circulation:
PVR brought about by first breath; collapsed lung will
2 ventricles out pouching will not meet endocardial cushion now open up, decreasing PVR; if you have a persistent
will wait until rotation of conus is complete: MC form of your VSD is 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 Hx 80% you can diagnose the case
ventricular mass, LV will be bigger than RV but during fetal
life they are equal in size. - Prenatal
- Medications
During fetal echocardiogram, sometimes there is difficulty in - Exposures esp. congenital; term cyanotic delivery:
distinguishing the right from the left because there are diseases exposure to Rubella, PDA in congenital rubella syndrome;
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where the right atrium goes to the left ventricle. Ventricular when did the mother have a problem? Infection on 3
inversion = normally right atrium tricuspid valve RV; the right month of gestation cardiac anatomy is already formed,
atrium drains into tricuspid and then your left ventricle. probably pulmonary artery is stenosed or there is a lodged
shunt; exposure/illness at 1 month of gestation: TGA,TOF;
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During fetal echocardiogram you try to distinguish whether the right 7 to 9 month of gestation, most do not manifest with
atrium or left ventricle are in their proper morphologic position. cyanotic heart disease more of the infection
There are structures that you have to identify inside the right - Signs and symptoms depends on what age patient is:
ventricle to know that it is really the right ventricle. infant feeding history (sleeping during feeding), how
many ounces does the baby consume? Manifestation of
Closure of shunts: foramen ovale, ductus arteriosus congestion is with interrupted feeding. Upon feeding there
(becoming ligamentum arteriosum) and ductus venosus may be diaphoresis. Pattern of breathing e.g. fast
(becoming ligamentum venosum) breathing, fatigability; cyanosis e.g. 1 month when place
Increase in catecholamines and myocardial receptors. baby in air conditioned room (acrocyanosis)
Catecholamines will help in the closure of your ductus
arteriosus. It will constrict your ductus to fully close. 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
NEONATAL CIRCULATION relieved by rest, pain scale 5/10, does not hinder normal activities,
feeling of something on the chest, radiates to left and back
1. RL vs LR shunt via PFO; blood form RA will pass sometimes, sometimes stomach hurts, Ranitidine, Omeprazole,
through foramen ovale to LA = right to left shunt, Mallox; mother and sibling has RHD, cousin operated for valve
increased SVR, increased pressure on left side = left to replacement; Sumapen for the last 5 years but sometimes forgets to
right shunt; right to left shunt after birth think of take it (prophylaxis for RHD); having it for a year, pain with activities
myocardial problem (probably high pulmonary arterial = RHD
pressure, persistent fetal circulation)
2. RL vs LR shunt via PDA; from RV to PA, ductus Patients with aortic regurgitation may manifest
arteriosus to descending aorta (right to left) Heart failure feeding, diaphoresis, fatigue, breathing, respiratory
3. Pulmonary vasculature constrict more in response to distress, dyspnea, cyanosis (look at tongue)
hypoxemia, hypercapnea, acidosis
4. Ventricular thickness RV = LV; more of LV as compared to PE
RV
5. High O2 consumption high CO; baby very dependent in - General survey: aesthenic, hyperaesthenic, hypoaesthenic;
blood volume look for Harrisons groove
- Vital signs: CR, RR, Temp, BP, always get height and weight
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Foramen ovale may still be patent up to 3 month of life. - Cyanosis: mild, differential, acrocyanosis; may use pulse
If you have a baby with a high pulmonary arterial pressure, atretic oximeter to get O2 sat
and cyanotic at birth do 2D echo look at pulmonary arteries - Heart failure DOB, difficulty of feeding, easy fatigability
(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. 5y/o with HR of 50 = abnormal; normal is 70 and above up to 110 or
120
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Ductus arteriosus closes on 10 15 hour of life; closure depends on 1 month old with HR of 80 = abnormal; normal is 110 160
whether the baby is term or preterm; closes in term infant because 1 month old with HR of 180 = abnormal
smooth muscles are more developed. Sometimes the baby is
dependent on the PDA if they have a congenital heart disease Patients with shunt diseases are mostly wasted.
especially those who are cyanotic
Cardiac PE
If your baby has Pulmonary valve atresia blood from RV cannot go - Inspection bulge; Harrisons groove (line that you see on
to lungs because you have an atretic valve. To oxygenate your lungs chest wall in patients with long standing heart failure
you need this PDA; or if you have a low or high O2 saturation. High mostly in patients with shunts particularly those with large
O2 sat close, low O2 open VSD, ASD or PDA secondary to prolonged congestion that
Volume overload open PDA (thats why you give diuretics) patient has been having)
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- Palpation apical impulse: normal location is 4 ICS;
EVALUATION OF CARDIOVASCULAR SYSTEM substernal thrust = Right Ventricular Enlargement; thrill
palpable murmur
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Hx and PE - Percussion very rarely done; dull sound in 6 ICS or
1. Guide echocardiographic evaluation axillary area in adults RVE
e.g. 15 y/o patient in severe respiratory distress; RR = 30; - Auscultation done immediately when patient is quiet
next question: what triggers the symptom, developed
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dyspnea going up the stairs, 5 steps; when was it/did it 1 month old baby with apical impulse on 5 ICS = abnormal; apex
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start? a month ago; duration for the last one month; beat goes to 5 ICS at around 4 to 5 months of age
cough at night while lying down every night, when going
up stairs gasps for air; check up with doctor said patient HEART SOUNDS
had pneumonia was given antibiotics; had fever for almost
2 weeks before check up; chest pain; 3 4 pillows give - S1 closure of AV valves
relief; on and off sore throat since Jan. 2011; relieved at - S2 closure of pulmonic and aortic valves
rest = patient might be having Rheumatic fever, RHD or A1
CHD, CHF; birth history = congenital heart disease P2
2. Eliminating expensive laboratory exams Split deep inhalation
3. Activities e.g. dyspnea during activity
When you inhale deeply there will be a more negative intrathoracic
Doctor does echocardiogram in pediatric patients. 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 right atrial enlargement; but if you have a tall P >0.25 mm normal
inspiration. 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
The QRS amplitude would tell you if you have a tall R it depends on
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MURMURS 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
- abnormal sounds heard on patients chest 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 3530
Sinus: followed by a QRS with a fixed PR interval
Atrial septal defect (secundum) 68
Patent ductus arteriosus 68
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 57
ventricular rate or in patients with complete heart block your atrial Tetralogy of Fallot 57
rate is slower than your ventricular rate or vice versa. Pulmonary valve stenosis 57
Aortic valve stenosis 47
You plot your QRS and T axis, measure QR interval; for any patient d-Transposition of great arteries 35
you always measure the PR, QRS and QT. Hypoplastic left ventricle 13
Hypoplastic right ventricle 13
Prolongation of your PR will tell you that you may have a block AV
Truncus arteriosus 12
block first degree, second degree, third degree, complete heart
block Total anomalous pulmonary venous return 12
Tricuspid atresia 12
QRS if you have PVCs; normal QRS is 0.08; for pediatric group the Single ventricle 12
normal PR is only 0.16 seconds, adults up to 0.2 Double-outlet right ventricle 12
Others 510
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 Bechets 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
rd
The cyanosis in TOF will manifest at 3 month of age Dilated Cardiomyopathy
Long QT syndrome
LATE DIAGNOSIS OF CHD
rd
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 Eisenmengers 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% 1. length and diameter of ductus
2. Outlet 5-7% 2. Relative resistances of Ao and PA
3. Inlet 5-8% Shunt flow occurs in systole and diastole continuous
4. Muscular 5-20% nurnur
a. central Laboratory Findings
b. apical CXR : PVM, cardiomegaly
c. marginal ECG : LAE, LVH
d. swiss cheese septum multiple muscular ECHO : size of ductus and gradient
VSD estimate PA pressure
Determinants of L R shunt UNTREATED PDA
1. Size of VSD PVOD
2. Difference in resistance between pulmonary bidirectional shunting : differential
and systemic circulation cyanosis increase PAP, increase PVR
3. Difference in pressure between RV and LV Eisenmenger phenomenon, if not treated
Shunt flow occurs in systole from beginning of systole to you will develop pulmonary arterial
end of systole you will hear the sound. That is why it is hypertension or Esienmenger phenomenon
called a pansystolic murmur. ( PAP, PVR and cyanosis)
Biventricular will occur late because in the long run if PE : loud single S2
there is continuous increase in pulmonary blood flow your CXR : normal heart size;vdilated central and
pulmonary artery is sclerosed so there will be thickening main PA
producing an increase in your PVR so there will be an ECG : RVH
increase in your PAP RV has to compensate by increasing
in size. ACYANOTIC HEART DISEASE
Laboratory Findings
CXR : PVN, cardiomegaly PBF PBF
ECG : LAE, LVH
ECHO : location/size of VSD
LA and LV size LVH RVH LVH RVH
Amount and direction of shunt
Estimate PA pressure PDA ASD AS VSD W/
UNTREATED VSD PS
irreversible changes take place in the VSD PAPVR COA PERIPHERAL
pulmonary arterioles PS
= Pulmonary vascular obstructive AVSD EISSEMENGER
disease (Eisenmengers disease) late stage of VSD
PVR -- L R shunt Congenital MS produces LAE, you have a small MV, the blood from
Signs/Sx: cyanosis- if patient develops the LA cannot pass through, it has increased pressure, as pressure
cyanosis and pulmonary arterial increases it will be reflected to the lungs = pulmonary edema, RVH in
hypertension before they would only last the long run. When you develop heart faiure, the right side of the
for about 2 years more after the onset of heart enlarges and you are going to have hepatomegaly.
pulmonary hypertension; but with the
outbreak of medicine there is Sildenafil Aortic stenosis small aortic valve or there is an obstruction before
very effective in decreasing pulmonary your aortic valve; during systole LV will contract, the blood will not
arterial pressure (0.25 mg/kg/day; paper be able to go or ass through your aortic valve because it is small or is
tab 100 mg divided into 1 paper tabs to obstructed, LV has to increase in pressure, LV enlarges.
make 10 mg/paper tab); Milrnone increases
both cardiac contractility and decreases Coarctation of the Aorta stenosis in your aorta; the most common
pulmonary arterial pressure. COA is type 1; after third branch is the first (type 1), after second
loud, single S2 branch is second (type 2), after the first branch the third (type 3); if
CXR : RVH 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
PATENT DUCTUS ARTERIOSUS blood and pass through the constriction
5-10% of CHD
Communication between the aorta and pulmonary artery VSD with PS the patient would have developed cyanosis unless the
Risk factors : prematurity, rubella : congenital rubella patient is having right sided heart failure. Since you have increased
syndrome ccongenital cataract with PDA, with mental pressure on the left side, even if you have stenotic valve still the
retardation/delay, sometimes Down syndrome blood will be pushed to the pulmonary artery. But if you have
Left enlarges: RA, RV, pulmonary artery (after birth it pulmonary valve atresia, the blood from the right will go to the
should be left to right) goes to pulmonary vein then to the aorta, no cyanosis; TOF has cyanosis because there is malposition of
left, LA to LV back to PDA, back to lungs , pulmonary veins, your great vessels
LA, LV and so on more volume on the left, never goes to
the right 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 90s
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 70s
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 PDAs 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 + RL 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
resistancemore 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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PEDIATRIC CARDIOLOGY Dr. Marivic Montilla - Esguerra

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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