Prepared by : HO Farah Nadia

Supervised by : Dr Krisha
TETRALOGY OF FALLOT (TOF)
• Most common cyanotic heart lesion

• Has 4 components:
• Pulmonary Artery Stenosis (determinant factor related to severity)
• VSD (usually large)
• Overriding Aorta
• RV hypertrophy

• Results in a right to left shunting of blood with low oxygen levels in

the arteries and in the body tissues
• Resulting in cyanosis, easy fatigability, fainting and shock.
• Clubbing may be observed
• oxygen-poor blood in the
right ventricle cannot go
through the VSD into the
left ventricle
• and cannot squeeze through
the pulmonary valve
• forced to travel up through
the displaced aorta that lies
directly over the VSD
• oxygen-rich blood from the
left ventricle, and carries
this mixture to the rest of
the body.
Signs & symptoms
1) Pink during newborn --> Cyanosis next few weeks or months
2) Dyspnea on exertion - Squatting to relief dyspnea
3) Hypercyanotic spell
– Sudden severe episodes of intense cyanosis caused by reduction of
pulmonary flow in patients with underlying TOF or other cyanotic heart lesion
– Due to spasm of right ventricular outflow tract or reduction in systemic
vascular resistance with resulting increased right to left shunt.
– Age: 3-6 mo
– Often in morning, precipitated by crying, feeding, defecation
– Severe case: syncope, seizure, stroke, death
4) Delayed growth and development
Hypercyanotic spell (Tet spell)
Physical examination
–Clubbing of the fingers and toes
–Retarded growth

Cardiac Examination:
•Normal pulses & BP
•Left anterior hemithorax bulge anteriorly due to right ventricular
hyperthrophy
•Systolic thrill may be felt at the 3rd and 4th parasternal spaces
•Systolic murmur most intense at left sternal border (due to pulmonary
stenosis)
•Single second heart sound or pulmonic component is soft
Investigations
• CHEST X-RAY (AP-
VIEW)
– Narrow base,
concavity of left
heart border
– Elevation of cardiac
apex
– “boot-shaped
heart”
– Hilar areas and lung
fields relatively clear
Radiopaedi
a.org
Investigations

• ECG
– Right axis deviation
and evidence of right
ventricular
hypertrophy.
– The P wave is tall
and peaked
suggesting right
atrial enlargement
Complications
• Cerebral thromboses – Sequelae of extreme
polycythemia and dehydration
• Brain abscess
• Bacterial Endocarditis
• Heart Failure in “Pink Tetralogy”
MANAGEMENT
• HYPERCYANOTIC SPELLS
– Medical emergency
– Knee-chest/Squatting position
• Place baby o mother’s shoulder with knees tucked up underneath
• Calming effect, reduce systemic venous return & increase systemic vascular resistance
– Oxygen 100%
– IV/IM/SC Morphine 0.1-0.2 mg/kg- to reduce distress and dyspnea
– If above measures fail:
• IV Propranolol 0.05-0.1mg/kg slow bolus for 10 mins
• Volume expander (crystalloid/colloid) 20mg/kg rapid IV- to increase preload
• IV NaCO3 1mEq/kg- to correct metabolic acidosis
• Heavy sedation, incubation and mechanical ventilation

– Resistant cases, consider:

• IV Phenylepinephrine (0.01-0,02mg/kg slow bolus)/Noradrenaline infusion (0.1-0.5mcg/kg/min)- ↑
SVR and ↓ right to left shunt
• Emergency Blalock Tausig shunt
MANAGEMENT
• MEDICAL
i. Pre-operative: Prostaglandin E1 – to maintain dilatation of PDA maintain adequate
pulmonary blood flow
ii. Pink Tetralogy: Anti-congestive (diuretics, digoxin, ACE-inhibitor)
• SURGICAL
– Corrective Surgery
• At 1-2 years of age
• Relief of the right ventricular outflow tract obstruction
• Patch closure of the VSD
– Modified Blalock-Taussig Shunt
• A single episode of Hypercyanotic spells /severe cyanosis <6 months old (child is
too young for total surgical repair)
• Small pulmonary arteries; to promote growth before definitive repair
• Anomalous coronary artery crossing in front of right ventricular outflow tract
TRANSPOSITION OF GREAT ARTERIES
• Failure of truncus arteriosus
to septate normally

• Rare, cccounts for ~5% of

CHD
• Untreated  90% die within
1st year of life
• Prognosis better in TGA
with VSD & pulmonary
stenosis  life expectancy
of 5 years

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Normal D-transposition of Great
Heart Arteries

• With VSD
• Without VSD
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Pathophysiology
D-transposition of Great • Desaturated blood enters the right
Arteries atrium → right ventricle → transposed
aorta → body. Severe aortic desaturation.
• Fully saturated blood → left atrium →
left ventricle → transposed pulmonary
artery → lungs. Pulmonary arterial saturation
increased.
• 2 IMPORTANT shunts:
i. Patent Foramen Ovale (PFO): Left To Right or
Bidirectional
ii. Patent Ductus Arteriosus (PDA): Aorta →
Pulmonary Artery
* Systemic vascular resistance > Pulmonary
vascular resistance
• 50% also has VSD
• Can be medical emergency
Pathophysiology
L-transposition of Great Arteries-
Congenitally corrected
transposition
• Desaturated blood:
Right Atrium → “Inverted” Left
Ventricle → Transposed Pulmonary
Artery → Pulmonary Circulation.
• Saturated blood:
• Left Atrium → “Inverted” Right
Ventricle → Transposed Aorta.
• This circulation would be totally
“corrected” where it not for the frequent
association of other congenital
anomalies, in this case, VSD + PS.
• Because of the stenotic pulmonary
valve, some left ventricular blood flow
crosses the VSD and into the right
ventricle and the ascending aorta, and
systemic desaturation results
CLINICAL MANIFESTATIONS
SIMPLE D-TGA D-TGA + VSD L-TGA
• Cyanosis & tachypnea • Cardiac failure • Variable acccording
within the 1st hour or manifestation to associated lesions
days of life • Subtle cyanosis-may • E.g:
• Hypoxemia (moderate only be detected after
i. Unobstructed
to severe) & Acidosis measure SPO2
• MEDICAL • Murmur – Harsh pulmonary flow +
EMERGENCY systolic/Holosystolic Isolated VSD
*depends on VSD size ii. PS + VSD = TOF
May be present:
• Parasternal heave
• Loud 2nd heart sound –
single/split
• Soft systolic ejection
murmur – midleft
sternal border
Investigations

Radiopaedi
a.org
• ECHOCARDIOGRAPHY
– Diagnostic
– Confirms transposed ventricular-
arterial connections
– Determine the degree of mixing
assessed by pulsed and color
Doppler examination
– Visualise size of PFO and PDA
– Detect other associated lesions:
• Pulmonary outflow obstruction
• VSD
Management
SIMPLE TGA
1. Infusion of Prostaglandin E1
• Maintain patency of ductus
arteriosus
2. Rashkind balloon atrial
septostomy- create ASD
3. Arterial switch (Jatene) procedure
• 1st 2 weeks of life
TGA WITH VSD
1. Elective one-stage arterial
operation + VSD closure
<3months of age
2. Late intervention predispose to:
• Heart failure
• Failure to thrive
• Pulmonary vascular disease
JATENE ARTERIAL SWITCH
TRUNCUS ARTERIOSUS
• Common
aortopulmonary
trunk
• VSD is always
present

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Truncus Arteriosus

Clinical Features
• Vary with age & degree of pulmonary vascular resistance
• Newborn - murmur, minimal cyanosis
• 1 to 2 mo - sx of heart failure, mild cyanosis

Physical Examination
• Runoff blodd to pulmonary circulation: Wide pulse pressure & bounding pulse
• Cardiomegaly, hyperdynamic precordium
• 2nd heart sound loud and single
• Systolic ejection murmur at LSE
Investigation
• ECG-right, left or combined ventricular hypertrophy
• CXR- cardiomegaly 25
Repair of Truncus
Arteriosus
TRICUSPID ATRESIA
• Represents approximately
3% of all CHD. Initial
survival is dependent on an
intra-atrial communication.
• Presentation and treatment
depend on the relationship
of the great arteries (normal
or transposed), presence of
a VSD, and severity of
pulmonary valve stenosis.
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 • Cyanosis • Increase left • ECG- left

Investigation
Clinical Features

Physical Examination
depend on ventricular axis
degree of impulse deviation +
limitation to • Single 2nd left
pulmonary heart sound ventricular
blood flow • Systolic hypertrophy
• Cyanosis ejection • CXR-
worsen if murmur at pulmonary
VSD close LSE under
circulation

28
 Management
• If the source of pulmonary
blood flow is not reliable, the
patient should be treated with
prostaglandin E1 until a
systemic-to-pulmonary artery
shunt can be established.

• All patients ultimately need a

modified Fontan's operation.
Long-term survival exceeds
85% at 10-year post-surgery
follow-up.
Total Anomalous Pulmonary Venous Drainage
(TAPVR)
• TAPVC results in fully oxygenated blood
from the lungs being directed back to the
right atrium, and survival requires a
defect in the atrial septum
• without ASD blood cannot reach the aorta

• Types :
1.  supra-cardiac (commonest),
- The pulmonary veins drain into the right
atrium through the superior vena cava 
2. infra-cardiac 
- The pulmonary veins drain into the right
atrium through the liver (hepatic) veins and
the inferior vena cava
3. mixed

30
Signs & symptoms
Cyanosis
Fast or troubled breathing
Fast heart rate
Cool, clammy skin
Tiredness and little movement
Poor feeding
Poor pulses
Enlarged liver
Heart murmur

TAPVC can be complicated by obstruction of the veins that drain the pulmonary venous
confluence, resulting in pulmonary hypertension and congestion.
Management
Management strategy depends on presence of pulmonary venous obstruction:
• Obstructed pulmonary venous drainage
- Presents with respiratory distress and heart failure.
- Initial stabilization: oxygen, diuretics, positive pressure ventilation.
- Surgical repair immediately after initial stabilization.

• Unobstructed pulmonary venous drainage

- Early surgical repair is required.
Case Study 
• Term baby boy delivered via EMLSCS for flexed breech in labour at 38w5d. 
• AS 9@ 1, IS CL, BW 3.92kg
• Mother is a 41yo malay lady, P3. 
• ANC :  GDM on D/C & advanced maternal age. 

• Birth history : 
Baby was born vigorous, pink,moving all 4 limbs with AS 9@1min. Indirect suction was clear. Initial steps
was done. Temperature was optimised. Noted baby tachypnic with mild SCR, RR 68 and nasal flaring.
SPO2 under RA 78-80%.However no grunting. Was placed under headbox, SPO2 picked up to 98-99%.
RR improved to 62x/min and intermittent scr.GM stat was 3.0mmol/l. Admitted to NICU ivo tachypneic.

Upon examination in NICU , child active, cyanosed, AFNT, good perfusion, warm peripheries, RR 44 under
NP 3L/min, no jittery

VS stable, GM pickup to 4.3 after feeding

Lungs : clear CVS : systolic murmur grade 2/6 at left sternal edge
PA : soft, no hepatosplenomegaly
ECHO (D1OL)
• Single ventricle
• single functional AV valve
• Transposed aorta with PA
• Mild PS with PG 22mmHg across
• MPA good size
• RPA LPA 3.5mm each
• PDA 1.8-2.0mm, bidirectional shunt
• Left arch. no CoA
• normal pulmonary venous drainage
• Acceptable function
Revised imp: complex cyanotic heart disease
-single ventricle with mild PS and small PDA

Plan :
Wean off oxygen
Keep SPO2 >80%
Started feeding accordingly
Repeated ECHO (D3 OL)
- Situs Inversus ECHO (D6OL) (HSAJB)
- Large PFO/ASD with 5mm left to Rt - tricuspid atresia
- Transposed Aorta with PA - moderate PS with peak PG 66mmHg,PA
- Moderate P/S with 46.6mmHg PG A arising from small and hypoplastic RV
- PDA seen with 3.3mm bidirectional shunt - Good size PA branches, RCA and LPA 5mm
- Large VSD - Large VSD, almost like single ventricles
- No MR - Aorta is arising from LV, anterior to PA side
- Triscupid Atresia by side
- Dilated LA & LV - Closed PDA
- No CoA - Unrestricted PFO
- Left arch, no COA
- normal pulmonary venous drainage
Revised impression :
1) Tricuspid atresia, ASD,
large VSD with pulmonary
stenosis
2) Transposition of great
arteries

Plan :
- under HSA and IJN f/up
- done Glenn shunt & main
pulmonary artery transection
on 20/1/2020, repair of left
pulmonary vein on 21/1/20
References

• BMJ best practice Congenital Heart Disease

• Nelson essentials of paediatrics 7th edition
• Paeds protocol for Malaysian Hospital 4th edition