PEDIATRICS II

Lecture 2: Pediatric Cardiology II

Dr. Marivic Motilla-Esguerra November 21, 2013

**Text inside dashed boxes are from Nelson Textbook of Pediatrics
OUTLINE
I. Cyanotic Congenial Heart Lesions
A. Transposition of the Great Arteries (TOGA)
B. Tetralogy of Fallot (TOF)
C. Tricuspid Atresia
D. Truncus Arteriosus
E. Total Anomalous Pulmonary Venous Return (TAPVR)
II. Obstructive Lesions
A. Pulmonary Stenosis
B. Aortic Stenosis
C. Coarctation of the Aorta
CYANOTIC CONGENITAL HEART LESIONS
1. Decreased pulmonary blood flow
Obstruction (TV, RV or pulmonary valve level) & a pathway by
systemic venous blood can shunt from R->L & enter the
systemic circulation
Tricuspid atresia
Tetralogy of Fallot
Single ventricle with PS
Degree of cyanosis depends on the degree of obstruction to
pulmonary blood flow
2. Increased pulmonary blood flow
Not associated with obstruction to pulmonary blood flow
Cyanosis due to either abnormal ventricular-arterial
connections or total mixing of systemic venous & pulmonary
venous blood within the heart
Transposition of the great vessels
Total anomalous pulmonary venous return
Truncus arteriosus
Deoxygenated systemic venous blood and oxygenated
pulmonary venous blood mix completely in the heart 02
saturation is equal in the pulmonary artery & aorta
TRANSPOSITION OF THE GREAT ARTERIES (TOGA)
Occurs in about 5% of all CHDs (M>F)
Most common cyanotic heart lesion among newborns
The aorta arises from the RV carrying desaturated blood to the
body; the PA arises posteriorly from the LV carrying oxygenated
blood to the lungs
Result: complete separation of pulmonary & systemic
circulations hypoxemic blood circulating throughout the body
& hyperoxemic blood circulating in the pulmonary circuit
(umiikot sa katawan, deoxygenated kasi may switch ng great
arteries)
Defects that permit mixing of the 2 circulations (ASD, VSD, PDA)
are needed for survival (shunts needed for mixing! Mortality in 6
months if not adressed)
About 50% have PFO or a small PDA
History & Physical Exam
VSD in 30-40%
History of cyanosis from birth
Signs of CHF with dyspnea & feeding difficulties develop during
the newborn period
Moderate to severe cyanosis esp.in large, male newborns
Single & loud S2; no heart murmur is heard in infants with an
intact ventricular septum (no heart murmur if ventricle intact)
Severe arterial hypoxemia with or without acidosis (hypoxemia
unresponsive to oxygen administration)
Diagnostics
ECG: RVH, LVH, CVH
X-ray: egg-shaped cardiac silhouette with a narrow, superior
mediastinum
Progressive hypoxia & acidosis result in death unless the mixing
of the systemic & pulmonary blood improves
Egg on its
side - TGA
Management
Without surgery, death in 90% of patients before they reach
6 months old
Infants with an intact ventricular septum are the sickest but have
the most dramatic improvement after surgery
Infants with VSD most likely to develop CHF & PVOD (surgery
before 3-4 months old)
With VSD & PS longer survival without surgery because
pulmonary vascular bed is protected from developing pulmonary
hypertension
If with CH: digoxin and diuretics
Surgeons do it by stage
Definitive repair: switch right- & left-sided blood at 3 levels: atrial
(Senning or Mustard), ventricular (Rastelli), great artery level
(Jatene)
Follow-up every 6-12 months: to detect arrhythmia, TR,
depressed RV function

TETRALOGY OF FALLOT

Occurs in 10% of all CHDs

Most common cyanotic heart defect beyond infancy

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 4 abnormalities: Maintain good dental hygiene & antibiotic prophylaxis against
o large VSD, SBE
o RVOT obstruction (can be pulmonic stenosis) Surgery esp.for neonates with TOF & pulmonary atresia, severely
o RVH
cyanotic infants <3 months old, infants <3 months old who have
o overriding of the aorta *(VSD and ROT more important
RVOT obstruction is most frequently in the form of medically unmanageable hypoxic spells
infundibular stenosis (45%) Blalock (ang teacher)-TAUSSIG (ang student na talagang
If with severe PS: normal heart size in CXR with decreased nakadiscover) (>3 mos.old) and Gore-Tex shunt (<3 mos.old)
pulmonary vascularity (reduced pulmonary venous return to the Total repair of the defect (patch closure of VSD & widening of
left side of the heart) the RVOT by resection)
Natural history: polycythemia develops, growth retardation if
cyanosis is severe, brain abscess & CVA rarely occur,
Prognosis & Complications
coagulopathy is a late complication
Despite the use of antibiotic agents, mortality remains at 20- 25%
Physical Exam Serious morbidity occurs in 50-60% of children with documented
Varying degrees of cyanosis, tachypnea, clubbing IE (heart failure due to aortic or mitral valve vegetations)
RV tap along the left sternal border and a systolic thrill at the Myocardial abscesses
upper and mid-left sternal borders Systemic or pulmonary emboli
Ejection click; single S2; gr 3-5/6 systolic ejection murmur at the Mycotic aneurysms
mid and ULSB (from PS)
Heart block
Acyanotic form: systolic murmur from VSD at LSB but the ECG
shows RVH
Treatment
Diagnostics Several weeks are required for a vegetation to organize
ECG: RAD, RVH completely therapy must be continued through this period to
CXR: small heart size, decreased pulmonary vascular markings; avoid recrudescence
conca Total of 4-6 weeks is recommended
Nonstaphylococcal disease: fever resolves in 5-6 days with
antibiotics
Surgery for severe aortic or mitral valve involvement with
intractable heart failure, myocardial abscess, recurrent emboli,
Boot-
new heart block, increasing size of vegetations while receiving
shaped:
therapy
TOF
Staphylococcus: Oxacillin with optional addition of Gentamicin; if
resistant Vancomycin
Streptococcus: Penicillin G Na or Ceftriaxone plus Gentamicin
Prosthetic valve endocarditis due to Staph: Oxacillin + Rifampicin
+ Gentamicin

TRICUSPID ATRESIA
1. Atretic (Missing) Tricuspid Valve
2. Hypoplastic Right Ventricle
HYPOXIC SPELL in TOF (important in TOF) 3. Ventricular Septal Defect
paroxysm of hyperpnea, irritability, prolonged crying, 4. Atrial Septal Defect
increasing cyanosis, decreasing intensity of the murmur 5. Pulmonic Stenosis
After crying, feeding or defecation (valsalva maneuver)
Activities that suddenly lower the SVR initiate the spell --
increases L->R shunt decreases PBF increase in
cyanosis
Sudden onset of tachycardia or hypovolemia can cause the
spell
Hypoxemia increase in p02, fall in pH stimulates the
respiratory center hyperpnea inc.systemic venous return
to the RV with fixed resistance at RVOT, the inc.SVR to the
aorta must go out the aorta- further decrease in arterial 02
saturation
Hypoxic Spell (break the cycle!)

Management
KNEE-CHEST POSITION (traps the systemic venous blood in the
legs, decreasing the systemic venous return and helps calm the
baby; may increase SVR by reducing arterial blood flow through
the femoral arteries)
PATHOPHYSIOLOGY
MORPHINE SULFATE suppresses the respiratory center &
There is no outlet from the RA to the RV present
abolishes hyperpnea (0.2 mg/kg/dose SQ) The entire systemic venous return enters the left side of the
NaHCO3 (1meq/kg/dose) dilute with same amt of D5W heart by means of the foramen ovale or an associated ASD
Make sure the IV is in = causes 2nd 3rd deg burns Left ventricular blood usually flows into the RV via a VSD.
02 may improve arterial oxygen saturation Pulmonary blood flow (and thus the degree of cyanosis)
Vasoconstrictors like Phenylephrine raise SVR (0.02 mg/kg IV) depends on the size of the VSD and the presence and
severity of pulmonic stenosis.
Propranolol may stabilize vascular reactivity of the systemic
Pulmonary blood flow may be augmented by or be totally
arteries preventing a sudden decrease in SVR (0.01-0.25 mg/kg
dependent on a PDA.
slow IV)
The inflow portion of the RV is always missing in these
Ketamine (1-3 mg/kg IV over 60 secs) increases systemic
patients, but the outflow portion is of variable size.
vascular resistance

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 If the ventricular septum is intact, the RV is completely
hypoplastic and pulmonary atresia is present
Most patients with tricuspid atresia are recognized in the
early months of life by decreased pulmonary blood flow and
cyanosis.
Less often, a large VSD in the absence of right ventricular
outflow obstruction can lead to high pulmonary flow;
patients have mild cyanosis and heart failure.
CLINICAL MANIFESTATIONS
Cyanosis
Increased left ventricular impulse
o In contrast to most other causes of cyanotic heart
disease, in which an increased right ventricular impulse
is usually present.
Holosystolic murmurs
o Audible along the left sternal border
o 2nd heart sound is usually single
Diagnosis is suspected in 85% of patients before 2 mo of
age.
In older patients, cyanosis, polycythemia, easy fatigability,
exertional dyspnea, and occasional hypoxic episodes occur
as a result of compromised pulmonary blood flow.
Patients with tricuspid atresia are at risk for spontaneous
closure of the VSD, which can occur rapidly and lead to a
marked increase in cyanosis.
DIAGNOSIS
1. Roentgenographic studies
Pulmonary undercirculation
Overcirculation
2. Electrocardiogram
Left axis deviation and left ventricular hypertrophy
Combination of cyanosis and left axis deviation is
highly suggestive of tricuspid atresia.
3. 2D echocardiography
Fibromuscular membrane in place of a tricuspid valve
Variably small right ventricle
VSD
Large LV and aorta
The can be determined by direct measurement and by
4. Direct Measurement and Doppler examination
Degree of obstruction at the level of the VSD or right
ventricular outflow tract
5. Cardiac Catheterization
Absence of direct flow to the RV results in an
angiographic filling defect between the RA and LV
TREATMENT
Severely cyanotic neonates
o Infusion of prostaglandin E1 until a surgical
aortopulmonary shunt procedure can be performed
o The Blalock-Taussig procedure is the preferred
anastomosis.
Next stage of palliation: Bidirectional Glenn Shunt
o Creation of an anastomosis between the SVC and the
PA.
o This procedure is performed after the patient has
shown signs of outgrowing a previous aortopulmonary
shunt (4-12 mos.)
Later surgical management: Modified Fontan Operation
o During 1.5 -3 yo, usually after the patient is
ambulatory.
o IVC is anastomosed to PA, either via a baffle that runs
along the lateral wall of the RA or via a homograft or
Gore-Tex tube running outside the heart.
TRUNCUS ARTERIOSUS
Pulmonary arteries arise from aorta
Presence of a common trunk that supply the systemic, pulmonary
and coronary circulation
Almost always associated with VSD; Large VSD
Truncus overrides the VSD and receives blood from both the
right and left ventricles
Truncal valve occasionally quadracuspid stenotic and/or
insufficient;
1.2-2.5% of all congenital heart disease
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Cyanosis is often present
Associated with Di-George Syndrome linked to deletion of
chromosome 22q11
Truncus arteriosus is a total mixing lesion with total admixture of
pulmonary and systemic venous return
Cyanosis due to increasing pulmonary arteriolar resistance
PATHOPHYSIOLOGY
In truncus arteriosus, a single arterial trunk (truncus
arteriosus) arises from the heart and supplies the systemic,
pulmonary, and coronary circulations.
A VSD is always present, with the truncus overriding the
defect and receiving blood from both the right and left
ventricles.
The number of truncal valve cusps varies from 2 -6.
Type I Truncus Arteriosus
o The pulmonary arteries may arise together from the
posterior left side of the persistent truncus arteriosus
and then divide into left and right pulmonary arteries
Types II and III Truncus Arteriosus
o No main pulmonary artery is present, and the right and
left pulmonary arteries arise from separate orifices in
the posterior (type II) or lateral (type III) aspects of the
truncus arteriosus.
Type IV Truncus Arteriosus
o No identifiable connection between the heart and
pulmonary arteries, and pulmonary blood flow is
derived from major aortopulmonary collateral arteries
arising from the transverse or descending aorta
o Called pseudotruncus but is essentially a form of
pulmonary atresia with a VSD
Truncus arteriosus is a total mixing lesion with total
admixture of pulmonary and systemic venous return.
Because of the large volume of pulmonary blood flow,
clinical cyanosis is usually minimal.
If the lesion is left untreated, pulmonary resistance
eventually increases, pulmonary blood flow decreases, and
cyanosis becomes more apparent.
Physical Examination
Tachypnea, tachycardia, excessive sweating, symptoms begin to
appear due to increase volume load on heart by excessive blood
flow
Systolic thrill along left sternal border / suprasternal notch
Normal S1 followed by loud ejection click, S2 loud and single
Apical diastolic low-pitched murmur caused by increased flow
through the mitral valve
CXR: Cardiomegaly (right, left, or combined ventricular
hypertrophy), increased pulmonary circulation
Management
Anticongestive medications could be given in the 1st few weeks of
life
Delay of surgery much beyond 48 wk may increase the
likelihood of pulmonary vascular disease
SURGERY: Rastelli Repair
o VSD is closed, the pulmonary arteries are separated from the
truncus, and continuity is established between the right
ventricle and the pulmonary arteries with a homograft
conduit
Immediate surgical results are excellent, but after repair the
conduit must be replaced, often several times, as the child grows.
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 In older patients who already have pulmonary vascular
obstruction, routine surgical treatment is contraindicated and
heart-lung transplantation is the only option.

TOTAL ANOMALOUS PULMONARY VENOUS RETURN

All four pulmonary veins drain to the RA
RV volume overload, hypertrophy
Manifestation: pulmonary vein obstruction is present or absent

SNOWMAN SIGN or FIGURE OF 8 on CXR

OBSTRUCTIVE LESIONS

PATHOPHYSIOLOGY
Abnormal development of the pulmonary veins may result
in either partial or complete anomalous drainage into the
systemic venous circulation.
Partial anomalous pulmonary venous return is usually an
acyanotic lesion.
TAPVR allows total mixing of systemic venous and
pulmonary venous blood flow within the heart and thus
produces cyanosis.
In TAPVR, the heart has no direct pulmonary connection
into the left atrium.
All forms of TAPVR involve mixing of oxygenated and
deoxygenated blood before or at the level of the right atrium
(total mixing lesion).
RA blood either passes into the RV and PA or passes
through an atrial septal defect (ASD) or patent foramen
ovale into the LA.
The RA, RV and the PA are generally enlarged, whereas the
LA and LV may be normal or small.
The manifestations of TAPVR depend on the presence or
absence of obstruction of the venous channels.
If pulmonary venous return is obstructed, severe pulmonary
congestion and pulmonary hypertension develop; rapid
deterioration occurs without surgical intervention.
Obstructed TAPVR is a pediatric cardiac surgical emergency
because prostaglandin therapy is usually not effective.
CLINICAL MANIFESTATIONS
3 major clinical patterns:
1. Severe obstruction to pulmonary venous return- cyanosis
and severe tachypnea. Murmurs may not be present. Dont
respond to mechanical ventilation.
2. Mild to moderate obstruction to pulmonary venous return
heart failure in early life and LR shunt, with pulmonary
hypertension, systolic murmurs are audible along the left
sternal border, and a gallop rhythm. Mild cyanosis
3. Pulmonary venous obstruction is not present- total mixing
of systemic venous and pulmonary venous blood and a large
left-to-right shunt. No pulmonary hypertension. Less likely
to be severely symptomatic during infancy.
Without obstruction: mild cyanosis, tachypnea
With obstruction: rapid progression to dyspnea, cardiac
respiratory failure
Large supracardiac shadow can be seen, which together with the
normal cardiac shadow forms a snowman
Heart is enlarged, the pulmonary artery and right ventricle are
prominent, and pulmonary vascularity is increased
ECG: large right ventricle and usually identifies the pattern of
abnormal pulmonary venous connections
TREATMENT: Van Praagh surgical procedure
PULMONARY STENOSIS
5 to 8% of CHD
Associated with congenital rubella, Noonan and William
syndrome
TYPES: vlavar, subclavian, infundibular, supraclavicular.
MANIFESTATION: asymptomatic to severe
Hemodynamics:
o RV pressure overload
o RV pressure hypertrophy RV failure.
o RV pressures maybe > systemic pressure
PATHOPHYSIOLOGY
The obstruction to outflow from the RV to the PA results in
increased systolic pressure and wall stress, which
eventually leads to RV hypertrophy.
The severity of these abnormalities depends on the size of
the restricted valve opening.
In severe cases, right ventricular pressure may be higher
than systemic arterial systolic pressure,
Whereas with milder obstruction, right ventricular pressure
is only mildly or moderately elevated. PA pressure is normal
or decreased.
Arterial oxygen saturation will be normal even in cases of
severe stenosis, unless an intracardiac communication such
as a VSD or ASD is allowing blood to shunt from right to left.
When severe pulmonic stenosis occurs in a neonate,
markedly decreased right ventricular compliance may lead
to cyanosis due to right-to-left shunting through a patent
foramen ovale, a condition termed critical pulmonic
stenosis.
Physical Examination
RV tap
Ejection click
Systolic thrill
Systolic murmur LUSB to back; soft P2
o Similar to ASD: systolic murmur LUSB but difference is, in PS,
there is radiation to back
Murmur of PS radiates to the back, ASD does not.

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 Diagnosis COARCTATION OF THE AORTA
ECG Constriction of aorta ; M > F 3:1
o Right Axis Deviation (RAD) 7% of all CHD
o Right Bundle Branch Block (RBBB) if mild Observed in descending aorta just opposite ligamentum
o RVH pure R wave and upright T wave in V1 arteriosum ( after left subclavian artery)
CXR Aortic valve: bicuspid in more than 70%
o Normal or RV cardiomegaly
Seen in Turners Syndome
o Normal or dilated Main Pulmonay Artery (post stenotic
dilatation) Results in severely reduced blood flow
Increased work on the heart leading to CHF and cardiovascular
collapse.
Natural History
Asymptomatic, progressive unlikely
Easy fatigability, CHF if with chest pain, syncope, sudden death Clinical Manifestations
Arrhythmia Classic signs of coarctation are diminution or absence of femoral
Infective endocarditis - pamper pulses.
Higher BP in the upper extremities as compared to the lower
Management extremities.
Interventional 90% have systolic hypertension of the upper extremities.
o Catheterization (Balloon Valvuloplasty) done initially to Pulse discrepancy between R and L arms.
locate stenotic portion Pressure build up in aorta
Surgical HTN
o Valvotomy (Brocks Procedure)
CHF in infancy if small
Most children asymptomatic
AORTIC STENOSIS
BP is increased in arms than legs
Valve thickened, bicuspid with fused commissure and eccentric
orifice LVH may be seen in ECG, CXR enlargement
Increase in LV pressure due to LVOT obstruction Rib notching in CXR if collaterals have formed (children > 5
LVH and highly intravascular pressure may lead to diastolic years)
filling ( backflow to LV) Grade 2-3/6 systolic ejection murmur

HEMODYNAMICS
Pressure hypertrophy of the LV and LA with obstruction to flow
from LV
HEMODYNAMICS
Clinical Manifestations
Obstruction of the left ventricular outflow => PRESSURE
Symptoms and physical findings in patients with aortic stenosis
hypertrophy of the LV
depend on the degree and severity of left ventricular outflow
obstruction
Usually asymptomatic until LV fails
Syncope and sudden death may occur with exercise
Harsh systolic ejection murmur ( grade 3-4/6) at RUSB ( right
upper sternal border) and radiates to the neck and left
midsternal border
Accompanied by a thrill in the suprasternal notch.
ECG may show ischemia in severe stenosis
Mild AS - may present with exercise intolerance, easy
fatigability, but usually asymptomatic
Moderate AS Chest pain, dyspnea on exertion, dizziness and
syncope RIB NOTCHING (red arrows) RIB NOTCHING (red arrows)
Severe AS Weak pulses, left sided heart failure, sudden death
Management Management
Secondary Bacterial Endocarditis (SBE) prophylaxis Secondary Bacterial Endocarditis (SBE) prophylaxis
Balloon valvuloplasty is indicated for children with moderate to Hyoertension tx
severe valvular aortic stenosis to prevent progression Elective repair at 2-3 yo
Valvotomy
Aggressive management of CHF
Valve replacement (Ross Procedure)
Balloon angiography ( shunt placement )
Resection and end to end anastomoses ( Crafoord and Nyllin)
Prosthetic patch aortoplasty ( Vossschulte)
Subclavian flap aortoplasty ( Waldhausen and Nahrwold)
Bypass between ascending and descending thoracic aorta
Mortality rate varies depending on age

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