About Us

Welcome to our website dedicated to the exploration of Tetralogy of Fallot (TOF), a complex congenital heart disorder often
diagnosed in neonates. Specifically tailored for anatomy, medical, and biology students, our platform offers comprehensive
insights into the anatomical, physiological, and pathological aspects of TOF.
At the heart of our mission is a commitment to providing accessible and in-depth resources that empower students to
understand the intricacies of this condition. From detailed discussion on the functional and clinical aspects of anatomy to
discussions on pathophysiology and clinical manifestations and modern-day surgical approaches to the disorder, our content
aims to facilitate a holistic comprehension of TOF.
INTRODUCTION: Understanding TOF at a surgical and clinical anatomy level is paramount for medical professionals, as it entails a
comprehensive grasp of the anatomical intricacies, physiological consequences, and surgical interventions essential for effective
patient care. At its core, the Tetralogy of Fallot comprises four primary defects: pulmonary stenosis, ventricular septal defect
(VSD), overriding aorta, and right ventricular hypertrophy. These anomalies collectively result in complex hemodynamic
alterations, leading to cyanosis, cardiac dysfunction, and compromised systemic perfusion. For surgeons and clinicians,
proficiency in dissecting and comprehending these anatomical aberrations is indispensable, as it forms the foundation for
accurate diagnosis, risk stratification, and therapeutic decision-making.

Moreover, the surgical and clinical anatomy of TOF extends beyond mere anatomical description, encompassing a deep
understanding of embryological origins and developmental pathways. Appreciating the intricate interplay between embryonic
cardiac development and the formation of these anomalies provides critical insights into the timing, mechanisms, and potential
etiological factors contributing to TOF. Such knowledge not only informs preoperative planning but also facilitates the
identification of associated anomalies and informs prognostication post-surgically.
From neonates presenting with cyanotic spells to adults experiencing late-onset complications, the clinical course of TOF varies
widely, necessitating a nuanced approach to diagnosis, monitoring, and intervention. Surgeons and clinicians must navigate this
complexity with precision, leveraging their understanding of surgical and clinical anatomy to tailor treatment strategies to
individual patient needs.
The tetralogy of Fallot emerges as a pivotal topic in surgical and clinical anatomy, commanding attention for its intricate
anatomical underpinnings, embryological origins, and functional and clinical implications. Through a multidimensional
exploration of its anatomical complexities, developmental origins, and clinical manifestations, medical professionals at the
surgical and clinical anatomy level embark on a journey of discovery, equipping themselves with the knowledge and skills
necessary for effectively managing this challenging cardiac anomaly and optimizing patient outcomes.
1.1 HISTORY OF Tetralogy of Fallot: How the name was coined?
 Niels Stenson first described the Tetralogy of Fallot in 1671, but William Hunter at St. Georges in 1784 provided an
accurate anatomical description. He explained the ventricles had a gap large enough to fit a thumb, while the opening
from the right ventricle into the pulmonary artery was as broad as a goose quill.
 Etienne-Louis Fallot refined his description of a wide outlet ventricular septal defect, together with subpulmonary and
pulmonary valve stenosis, and the consequent physiology, in his description of L'anatomie pathologique de la maladie
bleu in 1888.
 In 1777, Sandiford published the first description of the tetralogy of congenital cardiac abnormalities, today known as
Tetralogy of Fallot.
 The term Tetralogy of Fallot is used because Fallot recognized the clinical recognition of anatomic lesions, a challenge
previously considered impossible.
 Fallot's abnormality consists of tetralogy including pulmonary artery stenosis, interventricular communication, aorta
deviation to the right, and constant right ventricle hypertrophy.
 Fallot recognized that the most common form of pulmonary stenosis was the Tetralogy of Fallot, which was found in
77% of 110 cases of congenital anomalies studied (Pollack 1948)

 The Canadian Maude Abbott is credited for coining the phrase "tetralogy of Fallot," which describes a tetrad of (i)
ventricular septal defect with (ii) over-riding of the aorta, (iii) right ventricular outflow blockage, and (iv) right ventricular
hypertrophy. (Apitz, Webb et al. 2009)
1.2 INCIDENCE OF TOF:
Tetralogy of Fallot (ToF) in Infants
• Approximately 1 in 3600 live births.
• Accounts for 3.5% of congenital heart disease infants. Before the development of surgical intervention, 50% of patients
with tetralogy of Fallot died in their first few years, with survival rates notably lower.
• In the US, four to five cases per 10,000 live births.
• One of the most common congenital heart defects.
• Accounts for 7-10% of congenital heart disease cases.
• Experienced equally by males and females.
A 97% survival rate was reported in 2001, according to data that was compiled from all 13 cardiac surgery centers in the
United Kingdom and independently confirmed.
• 97% survival rate reported in 2001.
• 98% of patients survive for 20 years post-surgery.
• Over 90% of children post-surgery survive for 30 years.
• The age of patients undergoing primary corrective surgery has continuously lowered since the first documented
intracardiac repair of the tetralogy in 1955. Certain units even advocate for surgery to be performed as soon as a patient
is diagnosed, sometimes even in the first few days of life. (Shinebourne, Babu-Narayan et al. 2006)
 2.1Embryological Defect Causing of TOF
• Infundibular septum exhibits anterior and cephalad deviation throughout development.
•As a result, Misaligned ventricular septal defect (VSD) is overridden by aortic root.
• Obstruction of right ventricle's outflow tract (RVOT).
• Systemic RV systolic pressure results from succeeding RV hypertrophy, which is believed to be a reaction to the massive VSD
and RVOT blockage. (Starr 2010)
It is suggested that the underdevelopment of the sub-pulmonary infundibulum and its consequences constitute the
fundamental "monology" of the tetralogy of Fallot. Whereas the septa band is regularly formed, the parietal band (crista supra-
ventricularis) and the nearby infundibular free wall combine to produce an unusually tiny cone (conus) of muscle underneath
the pulmonary artery. The idea that the core of tetralogy is an abnormally tiny sub-pulmonary conus is supported by anatomic
and angiographic findings in typical vs normal tetralogy. (van Praagh, van Praagh et al. 1970)
• Fundamental defect: subpulmonary infundibulum underdevelopment without major artery transposition.
• Moderate pulmonary outflow tract stenosis.
• Underdevelopment of pulmonary valve's infundibulum. [fig3]
 Pulmonary outflow tract obstruction:
• In general, there is moderate pulmonary outflow tract stenosis if there is moderate infundibular underdevelopment.
Infundibular underdevelopment can cause severe pulmonary outflow tract stenosis or atresia.
• The undeveloped infundibulum frequently grows ever more hypertrophied with time, swelling both its myocardium and
endocardium. This results in the infundibulum becoming a "mighty midget" and the seeming conundrum of coexisting
infundibular hypertrophy and hypoplasia.
• Ventricular septal defect: The distal canal septum, also known as the parietal band or the crista supra-ventricularis, is
typically described in tetralogy as "deviated" from the tricuspid valve in three directions: anteriorly, superiorly, and leftward.
To say that the cristae have not been carried in a posterior, inferior, or rightward direction, however, appears more
appropriate. Space remains above the ventricle when the posterior positioning of the crista fails. The ventricular septal
 Aortic overriding :
• The location of the whole aortic valve is slightly aberrant when anomalous aortic overriding is present. It is a little too
superior, anterior, and rightward.
• The mildly aberrant placements of the aortic sinuses of Valsalva, which are frequently best understood with
angiocardiography, reflect this. It is usual for the noncoronary sinus to face straight rightward rather than rightward and
posteriorly.
• The orientation of the right coronary sinus and artery is not immediately anterior, but rather anterior and somewhat to the
left. It appears that this explains why the right coronary artery can give rise to the anterior descending coronary artery in
severe therapy, a point of considerable surgical relevance. The right coronary artery has an abnormally leftward origin,
placing its ostium unusually close to the anterior interventricular sulcus.
• Thus, it would seem that anomalous aortic overriding is a real anomaly that can arise in tetralogy with severe pulmonary
infundibular underdevelopment; this overriding is arguably the most noticeable feature of the somewhat disturbed
relationship between the major arteries in severe tetralogy.
 . Right ventricular hypertrophy :
• According to reports in tetralogy, right and left ventricular pressures were similar during pregnancy, which may have
prevented right ventricular hypertrophy from developing at birth. The pulmonary outflow tract blockage and the massive
ventricular septal defect, which are both caused by infundibular underdevelopment, appear to be the primary causes of the
postnatal development of right ventricular hypertrophy. (van Praagh, van Praagh et al. 1970)
2.2 Basic anatomy of normal heart :
Heart Components and Aortic Valve Position Interpreting abnormalities require an understanding of normal anatomy. The
aortic valve's central placement is crucial to comprehending the general arrangement of the heart components. The aorta
leaves the left ventricle, although its valve is positioned inferiorly to the pulmonary valve on the right side. The most inferior
cardiac valve, the tricuspid valve, guards the intersection of the right atrium and right ventricle. It is oriented about vertically.
Adjacent to one other, the aortic and mitral valves are concealed by the right ventricle.
Atrial Appendages and Atrium Characteristics
• Distinct atrial appendages distinguish between the left and right atrium.
• Right atrium exhibits comb-like trabeculations from the terminal crest.
• Pectinate muscles extend into the postero-inferior wall.
• Left atrium wall smoother due to pectinate muscles in finger-like appendage.
• The posterior region of the venous component houses pulmonary veins.
• Right atrium features coronary sinus and inferior cava vein valves.
Another helpful marker is the path of the coronary sinus that passes under the left atrium's inferior wall and is visible through
incisions directed toward the diaphragmatic surface.
Ventricles characteristics:
• Ventricles are divided into intake, apical trabecular, and output.
• Apical trabecular section often differentiates left and right morphologies.
• Right ventricle distinguished by broad moderator band and coarse apical trabeculations.
• Left ventricle’s apical region has no trabeculation issues.
• Atrioventricular valves, and ventricular inlets, are also useful indicators.
• Right ventricle's tricuspid valve, protected by septal, anterosupe­rior, and mural leaflets.
• Direct chordal attachments of the septal leaflet to the ventricular septum.
• Mitral valve protected by aortic and mural leaflets.
• Septal surface smooth and free of valvar insertions due to leaflets attached to paired papillary muscles.
The Basic Anatomical contrast between the Normal heart and Tetralogy of the Fallot :
aorta is the name of the body's main artery. Usually, it is joined to the lower left chamber of the heart. The aorta is positioned
incorrectly in the tetralogy of Fallot. It is positioned precisely above the opening in the heart wall after being moved to the right.
The aorta in TOF is dilated and displaced across the intraventricular septum in comparison to the normal heart. Increased blood
flow through the aorta when it gets blood from both ventricles via the VSD is the cause of aortic dilatation.
TETRALOGY OF FALLOT
The ventricular septal defect, biventricular connec­tion (overriding) of the aorta, sub-pulmonary stenosis, and right ventricular
hypertrophy are the four basic hallmarks of this abnormality.
The anterior and cephalad departure of the outflow septum's septal insertion from the septo-marginal trabeculation in the right
ventricle is the morphological signature.
This characteristic explains both the ventricular septal defect and sub-pulmonary stenosis when compared to a normal heart.
There are differences in the degree of aortic override, aortic arch abnormalities, coronary arterial anomalies, shape and
quantity of ventricular septal defects, and pulmonary valvar and arterial stenosis. Fallot syndrome, also known as absent
pulmonary valve syndrome, is linked to the underdevelopment or absence of the pulmonary valve, which results in pulmonary
arteries that are noticeably enlarged and may clog airways.
The precordial long-axis slice clearly shows the biventricular connection of the aorta. The right ventricular outflow tract, the
outlet septum's deviation, and the ventricular septal defect may all be seen by rotating the long-axis and short-axis planes. The
deformity is most visible in neonates from the subcostal window along the parasagittal and para-coronal planes. Examining the
aorta and pulmonary arteries from suprasternal or high precordial incisions. (Ho, McCarthy et al. 2001) [ IMAGE 4]
2.3 Clinical Anatomy of TOF: The French physician Etienne-Louis Fallot described the first case series in 1888 with anatomical
and pathological descriptions of the four fundamental symptoms
(i) VSD
(ii) RVOTO Overriding aorta
(iii) RVH
 Ventricular septal defect :
• Characterized by significant non-restrictive defect, does not impede the flow across the VSD.
• Allows blood flow between ventricles (from right to left in a traditional cyanotic ToF)
• Often seen in peri-membranous and muscular areas of the ventricular septum.
 RV outflow tract obstruction: RV Blockage in Outflow Tract
• RV blockage in the RV outflow tract (RVOT) is a key component of ToF.
• Worsening RVOTO leads to a right-to-left shunt through the unrestrictive VSD, lowering pulmonary blood flow and causing
hypoxemia.
• If collateral supply is absent and patent ductus arteriosus is blocked, hypoxemia may be more severe and henceforth, the more
severe the RVOTO.
 Overriding aorta:
• This problem refers to the ventriculoarterial connection wherein the malalignment of the septum outflow component allows
the aorta to partially overcome VSD.
• ToF results in the aorta mainly emerging from the left ventricle and partially from the right.
• Double Outlet Right Ventricle (DORV) physiology determined by VSD location and any left ventricular or RV outflow blockage.
• DORV is more likely to enter arterial circulation than other lesions, making it crucial to comprehend.
 RV Enlargement :
• RVOTO results in RV hypertrophy due to increased pressure. [fig 9]
• Changes RV cavity size and muscle mass.
• Significant problems post-ToF repair. (Wilson, Ross et al. 2019)
2.4 Fallot's Case Summary: INDEX CASE
• Noted deficit in the basal section of the ventricular septum in the 19-year-old heart.
• Observed aortic root overriding ventricular septum crest due to equal ventricle support.
• Noted flatter, longer infundibulum, and more valvar stenosis.
• Identified concave edge of incomplete ventricular closure visible posteriorly.
 He highlighted that right ventricular hypertrophy was present. He emphasized the existence of pulmonary trunk
constriction, an interventricular connection, right ventricle hypertrophy, and rightward deviation of the aorta while
summarizing the "true anatomopathological tetralogy.“
 In a normal heart, the supraventricular crest fills most of the space between the ventricles, which is visible from the right
ventricle. In the context of the tetralogy, it is also evident that the leading edge of the muscular outlet septum—sometimes
referred to as the infundibular or Conal septum—is the "edge of incomplete ventricular closure," as observed once more
from the perspective of the right ventricle.
 The right ventricular hypertrophy is a hemodynamic result of the anatomic abnormalities, and there is further variation in
the other two morphological components of the tetralogy.
• Variation in other tetralogy components.
• Significant heterogeneity in aortic override degree.
• Biventricular connection degree better explains this characteristic.
• "Aortic override" may confuse due to the right coronary aortic sinus overriding the muscular ventricular septum crest.
(Anderson, Spicer et al. 2013)
2.5 Surgical Anatomy of TOF: Ventricular septal defect: The diverging arms of the trabecula septo-meginalis formed the caudal
boundary of these deformities. The ventricular-infundibular fold, which supported the aortic valve and kept it apart from the
tricuspid valve in this region, was located in the posterior region of the cephalad border. The aortic valve and the tricuspid valve,
however, joined in fibrous continuity toward the septum in the posterior caudal region of fibrous tissue, which also included the
atrioventricular part of the membranous septum. Thus, these anomalies can be classified as "peri-membranous" deficiencies
since they are linked to trabecular and infundibular muscle malalignment in the ventricular septum.
Peri-Membranous Defects in Surgery :
• The major “danger area” for peri-membranous defects is the atrioventricular bundle entry.
• Tetralogy shares properties with isolated peri-membranous defects.
• Tetralogy infundibular defect, surrounded by muscle, is similar to a single infundibular muscular defect.
• The muscular inferior rim is composed of the ventriculo-infundibular fold and trabecula septo-marginalis.
• Disagreements exist over intra- or infracristal defect.
• Aorta divided by muscular tissue. It's crucial to remember that muscle tissue divides the aorta and tricuspid valves and divides
the defective margin from the conduction tissue.
Infundibular Obstruction :
• Obstructive trabeculations and fibrous tissue accretions on the infundibular septum caudal aspect worsen stenosis.
• Creates distinct circular aditus to smooth the infundibular chamber.
• Lack of infundibular septum in heart with sub arterial defect.
• Blockage caused by arterial septum deviating at pulmonary trunk's expense.
Conduction Tissues: The tissues involved in conduction. The surgeon’s top priority is to address the atrioventricular conduction
tissues’ condition. The existence of the branching atrioventricular bundle astride the free border of the sepsis is the main variable
with surgical relevance. The confluence of the aortic, tricuspid, and mitral valves on the posteroinferior border of a
perimembranous ventricular septal defect is known to be the primary "danger area." The ventricular-infundibular fold fuses with
the trabecula septo-marginalis cephalad to the membranous septum, making this region "safe" in hearts lacking aortic-tricuspid
The coronary arteries: Anomalous epicardial branching of the coronary arteries, specifically the origin of the anterior
descending artery from the right coronary artery, is another important trait of surgical relevance in the correction of tetralogy of
Fallot. (Anderson, Allwork et al. 1981) { image 6}
2.6 There are several subtypes of ToF, and they are mostly identified by the level of cyanosis they exhibit :
• Causes low or no RVOTO in acyanotic children with normal oxygen saturations.
• PINK’S FALLOT :

• Functions like a large, unconstrained VSD with a left-to-right shunt.

• May evolve into a traditional ToF over time.

• Fallot-type pulmonary atresia (15% of ToF) :

• Characterized by total atresia of the pulmonary valve.

• Prevents blood flow from RV into the pulmonary artery.

• All pulmonary blood flow originates from the aorta via PDA or major aortopulmonary collateral arteries.

• Crucial for intracardiac mixing.

• ToF with pulmonary valve missing (6% of total ToF)

• Syndrome results in respiratory difficulties due to large aneurysmal dilatation of pulmonary arteries.

• Newborns are generally acyanotic due to a lack of RVOTO.

• Aneurysmal dilatations compress the distal trachea and bronchi, leading to airway blockage, lung atherosis, and pulmonary hypoplasia.

• Treatment often results in trachea-bronchomalacia, requiring extended ventilatory assistance.

(Wilson, Ross et al. 2019)
 SaO2 SaO2
100%
30%

PINK” TOF
BLUE” TOF “PROFOUND CYANOSIS”

Little aortic override

Classic TOF with aortic
Function similar to a left-to- Profound RVOTO
override+ RVOTO
right VSD
2.7 Clinical Features of TOF:
Clinical characteristics of TOF might differ widely. The majority of patients have varying degrees of cyanosis at first, with
newborns with severe valvar and infundibular stenosis exhibiting the most severe cases as the PDA begins to constrict.
• Arterial oxygen saturation may drop to less than 60%, causing agitation, tachypnea, sweating, and poor eating.
• Initial blood pressure is well-maintained, but continued acidosis may cause irregular pulses, paleness, and mottling.
• Cyanosis develops gradually and worsens with age (Chrysostomou, Domnina et al. 2010)
• Low SpO2 and hypoxia combined with no oxygen treatment response.
• Heart murmurs: systolic and pan systolic ejection.
• Epistolary episode: disturbed, frightened, severely cyanotic.
• Arrhythmias, especially ventricular or supraventricular.
• Correlates with additional genetic disorders.
• Late presentation: poor exercise tolerance, clubbing, polycythemia, neurodevelopmental delay, heart failure, recurrent
respiratory infections, brain abscess, and stroke.( Wilson, Ross et al. 2019)
2.8 Clinical Presentation of TOF:
The severity of the RVOTO determines how TOF presents clinically.
• Newborns are often acyanotic and asymptomatic.
• Majority experience mild-to-moderate cyanosis without respiratory difficulty.
• Small obstructions may go unnoticed or misdiagnosed.
• Increased blockage may lead to additional symptoms.
• Often have an oxygen saturation of 80–90%.
• Most TOF patients struggle to feed themselves, increasing the risk of failure to flourish.

On auscultation reveals a single, loud second heart sound. Blood flow over the constricted pulmonary valve causes a noisy
crescendo-decrescendo systolic ejection murmur near the upper left sternal border. However, the murmur will be less noticeable
if the RVOT is significantly occluded. Stated differently, there exists an inverse relationship between the severity of the RVOTO and
the systolic murmur's duration and intensity. Due to the defect's size and lack of blood flow obstruction, the VSD is frequently
silent. (Iecho 2021)
Pulmonary Atresia in Newborns
• Over 80% show present with Pulmonary Atresia
continuous murmur indicating TOF.
• Murmur audible over PDA or MAPCA location.
• Murmur intensity may increase with PVR drop.
 Infants and children with uncorrected TOF:
• Severe episodes of cyanosis in infants and children with uncorrected TOF.
• Less frequent than 9-12 months in children.
• Frequency declines beyond 4-5 years.
 Older kids with TOF frequently squat naturally to alleviate their symptoms and regain their breath. Squatting results in an
increase in the SVR, which decreases right-to-left shunting while simultaneously increasing pulmonary blood flow. Babies and
younger children can be helped to break tet spells by putting their knees to their chests. This appearance of squatting is
called Fallot's sign.
 Long-Term Cyanotic TOF in Children:
• Leads to hypertrophic osteoarthropathy.
• Symptoms: arthritis, periostitis, finger clubbing. . (Iecho 2021)
3.1Pathophysiology of TOF:
 The degree of right ventricular (RV) outflow blockage determines a lot of the physiologic effects of TOF.
 The pressure in the RV is similar to that of the left ventricle because the ventricular septal defect (VSD) is usually wide and
unrestrictive. Therefore, rather than the VSD's size, the path of least resistance for blood flow will decide the direction of
blood flow across it.
 Blood spontaneously shunts from the left ventricle to the RV and pulmonary bed if resistance to flow across the RV outflow
tract (RVOT) is less than the resistance to flow out of the aorta into the systemic circulation.
 Blood Flow Barrier in Pulmonary Bed :
• RV outflow blockage increases the barrier to blood flow into the pulmonary bed.
• Large RV blockage increases resistance, facilitating easier crossing of VSD into the left ventricle and aorta.
• Desaturated blood enters systemic circulation, leading to cyanosis.
• Erythrocythemia can occur from prolonged cyanosis in unrepaired TOF patients.
 RVOT Blockage Variability in TOF :
• Variability in RVOT blockage is a physiological feature of TOF.
• Even mild cyanosis can result in a dynamic increase in RVOT blockage.
• Extreme cases, known as “hyper cyanotic spells" or "Tet spells," can cause significant cyanosis and almost complete RVOT
blockage.
What is a TET Spell?
Hypercyanotic Spells in Infants with TOF
• Known as Tet spells, deep blue coloration and rapid breathing in infants with TOF.
• Frequent between ages two and four months, often caused by dehydration.
• Treatment involves calming the infant and adjusting the heart’s blood flow pattern.
• Spells can cause fainting, seizures, or strokes.
• Rapid surgery is often necessary to prevent a recurrence. (Starr 2010)
3.2 Mechanism spiral of the cyanotic spell:

Infundibular muscle spasm

Reduced systemic vascular resistance

Increase in right to
left shunt

Increase in Hypoxia, Acidosis,

tachypnoea and rise in PaCO2
venous return

Increase in
Pulmonary vascular
Resistance
3.3 MORPHOLOGY OF TOF:
 ToF Characteristics :
• Hypertrophy of trabeculations on the infundibular free wall.
• Anterior craniocaudal or cephalad deviation of muscular outlet septum insertion.
• Displaced outlet septum of trabecula septo-marginalis causes malalignment ventricular septal defect (VSD).
• Muscular sub-valvar RVOTO caused by misplaced outlet septum and parietal or free wall trabeculations. (Shinebourne, Babu-
Narayan et al. 2006)
Postnatal Development and Heart Formation :
• Cardiomyocytes transition from hyperplastic to hypertrophic during early postnatal development.
• Changes in cyclins, CDK, inhibitors, and transcription factors regulate the cell cycle.
• Myocardium undergoes morphological alterations, increasing ploidy, size, and differentiation of CMCs.
• CMCs exit the final cell cycle before cytokinesis, leading to more binucleated, terminally differentiated CMCs at the G0 stage.
(Sukhacheva, Serov et al. 2022)
•
Myocardial hypoxemia and pressure and volume RV overload happen right after birth in TF patients. In patients with TF,
compensatory remodeling of the RV myocardium includes pulmonary hypertension, increased blood oxygen capacity (higher
amount of hemoglobin in the blood), and RV hypertrophy. The goal is to maintain acceptable hemodynamics and enhance
blood oxygenation. It is established that SaO2 values are related to the proliferative activity of RV CMCs in TF patients.
Several experimental investigations have demonstrated that pressure and volume RV overload, similar to that found in TF,
during the early stages of postnatal development activates proliferative markers in CMCs and increases angiogenesis and
myocardial fibrosis in the long run.
 [contd]
During the early years of life, the immature myocardium of children with TF experiences ontogenetic expansion and
differentiation of CMCs, which accelerates their response to hemodynamic stress and myocardial hypoxemia.
• RV outflow tract myocardium exhibited morphological alterations.
• CMCs increased in size and ploidy, active assembly of myofibrils.
• Maturation of intercalated discs with accumulation of GJs carrying Cx43.
• Incomplete differentiation in ultrastructure.
• Less active proliferation.
• Infants with TF showed signs of CMC differentiation, including decreased ploidy, diploid CMCs filled with myofibrils, delayed
differentiation of intercalated discs, and degenerative ultrastructural changes.
• Interstitial tissue fraction did not significantly contribute to cardiac remodeling in children with TF, only the ontogenetic growth
rate.
• Hemodynamic markers of CHD and hypoxemia are crucial in differentiating RV CMCs.
• Ontogenetic growth pace affects CMC morphology in TF children.
• Change sequence doesn't vary throughout differentiation. (Sukhacheva, Serov et al. 2022)
3.4 Etiology of TOF: Although the etiology is complex and multifactorial
• Untreated maternal diabetes, phenylketonuria, and retinoic acid consumption have been linked to the condition.
• Chromosome defects include Trisomy 21, 18, 13, and 22 microdeletions.
• Recurrence risk in families is 3%. There is an increased risk of TOF in additional children in the family if a parent or sibling has
the condition. (Bailliard and Anderson 2009)

• Tetralogy of Fallot in Pregnancy

• • Possible tetralogy of MTHFR gene polymorphism with TOF gene.
• • Prevalence linked to maternal alcohol consumption, poor prenatal nutrition, maternal rubella, and mother age.
• • More common in pregnancies with diabetes, phenylketonuria, birth defects, or older than 40.
• • Prevalence in newborns with prenatal carbamazepine syndrome, fetal hydantoin syndrome, and Down syndrome. (Elsaka,
Noureldean et)
GENETIC FACTORS ASSOCIATION WITH TOF:
Study on Non-syndromic TOF Mutations
• 4% of 114 non-syndromic TOF patients had NKX2-5 mutations.
• De novo copy number variations found in 10% of sporadic TOF cases.
• TOF linked to mutations in ZFPM2, GATA5, and TBX1. Additionally, MTHFR polymorphism has been linked to a higher chance of
developing TOF.
About 15% of patients with TOF were associated with certain syndromes at the presentation time.
●Alagille syndrome, caused by JAG1 mutations. It has also been reported that TOF is the only sign of JAG1 mutations without any
other Alagille syndrome symptoms.
• Chromosome 22q11 deletion causes velocardiofacial disorders and DiGeorge syndromes.
• Children lacking extracardiac abnormalities may have TOF susceptibility genes.
• Adult patients with TOF are often unaware of 2 deletion syndrome.
Tetralogy of Fallot (TOF): Pathophysiology, clinical features, and diagnosis (medilib.ir)
4.1 Clinical assessment and identification :
Prenatal diagnosis:
As early as 12 weeks of gestation, a tetralogy of Fallot diagnosis can be made prenatally. However, only half of the instances in a
population-based investigation were found during routine obstetric ultrasound screening. Individuals who are suspected of
having tetralogy of Fallot and are referred for fetal echocardiography typically have the most severe phenotypes. Fetal
echocardiography referrals can also be made for known chromosomal abnormalities or the identification of extra-cardiac
anomalies.
Thus, compared to individuals who receive a postnatal diagnosis, those referred for fetal echocardiography often have poorer
results. Prenatal fetal ultrasound screening is used to diagnose most occurrences of transfusion-associated fever. This process
makes it possible to schedule postnatal care, deliver the baby to a cardiac center, and provide the parents with appropriate
counseling. (Bailliard and Anderson 2009)

Diagnosis of ToF in Infants:

• After delivery, the severity of ToF can be assessed.
• Many infants can be identified by regular pulse oximetry monitoring and murmurs.
• Conventional presentation: tiny infant with a high cardiac output, or cyanotic neonate.
SpO2 readings of 75–80% are considered “normal” for these infants.
• A moribund baby with severe metabolic acidosis, diminished cardiac output, hypoxemia, and heart failure may result from non-
recognition.
It’s crucial to keep in mind that the presence of heart lesions does not always equate to the presentation of clinical cyanosis;
children with polycythemia may seem cyanosed more readily than anemic ones.
Cardiac Axis Rotation and Fetal Disease Diagnosis :
• Rare leftward rotation of the cardiac axis can distort the four-chamber image.
• Aberrant views of large vessels, especially at the upper mediastinum, account for many referrals.
• Diagnosis of fetal disease involves a big VSD, lower pulmonary outflow tract diameter, and a larger aorta.
• Main pulmonary artery (PA) size and ratio to ascending aorta indicate outflow tract blockage.
• Main PA is smaller in the duct-dependent pulmonary circulation.
• Branch PAs usually have normal or almost normal sizes at mid-gestation.
• Pregnancy can lead to normal or diminished growth of pulmonary arteries, often linked to severe outflow tract blockage.
• Fetuses with Tof have narrower arterial ducts, but forward flow is preserved.
• Left-to-right ductal flow indicates pulmonary atresia or significant outflow tract blockage.
Serial scans are therefore necessary to record ductal flow patterns and fetal pulmonary artery development, which is
necessary for counseling and proper infant care.
• Recording ductal flow patterns and fetal pulmonary artery development is crucial for counseling and infant care.
• Absent pulmonary valve syndrome cases range from 3–6%.
• Missing or surviving valve leaflets cause stenosis and pulmonary regurgitation.
• Arterial duct is typically present, but not always, and pulmonary arteries are dilated.
• Larger pulmonary arteries compress the bronchial tree, leading to postnatal respiratory problems.
Some fetuses may have hydrops and a poor prognosis due to right ventricular dysfunction, which may be attributed to
significant pulmonary regurgitation during fetal life.
• Tei index shows non-survivors have higher values than survivors, indicating global ventricular dysfunction.
• Branch Pas are not significantly dilated when the arterial duct is patent. (Huehnergarth, Gurvitz et al. 2008)
4.2 COMPLICATIONS of ToF:
• Infective endocarditis can lead to a bacterial heart valve or heart lining infection.
• Antibiotics may be administered before dental procedures to prevent infection.
• Untreated tetralogy can cause severe problems, potentially leading to death or disability in adulthood.
• Open heart surgery for tetralogy of Fallot can result in long-term issues, despite successful recovery.

• Potential issues include a leaky tricuspid valve leading to pulmonary regurgitation.

• Ventricular septal defects can cause blood leaks or require repair.
• Enlarged or dysfunctional left ventricle can cause sudden cardiac death, aortic root dilatation, coronary artery disease, and
arrhythmias.
• Regular examinations with a cardiac specialist are crucial for treating congenital heart disease.
(Elsaka, Noureldean et al. 2022)
PROGNOSIS OF TOF:
Fallot Therapy Prognosis
• Long-term survival in patients with tetralogy of Fallot is 85%.
• Patients with tetralogy 30 years ago can have normal lives, with normal pregnancies and healthy babies.
• Modern adults face issues with ventricular fibrillation, recurrent or residual pulmonary stenosis, and hemodynamic symptoms.
• Modern patients have better prognoses due to recent developments in surgical and medical treatment.
• Congenitally defective heart can lead to a better prognosis.
• Significant advancements in surgical and medical therapy.
• Prognosis improvement is expected in this era.
• Patient care for Fallot's tetralogy continues beyond full recovery.
• Cardiologists monitoring patients for life.(Anderson, Bailliard et al. 2009).
4.3 Preoperative Evaluation for TOF Patients :
• Chest X-ray shows normal heart shape with a characteristic “Boot-shaped” heart configuration: inverted apex, small primary
pulmonary artery segment.
• Electrocardiogram: persistent upright T wave in the precordial leads, right axis deviation, and RV hypertrophy
• Echocardiography in TOF:
• • Identifies heart structure and large arteries.
• • Determines degree and form of pulmonary valve obstruction.
• • Assesses the size of main and peripheral pulmonary arteries.
• • Identifies the number and size of VSDs.
• • Identifies the presence of PDAs or MAPCAs.
• • Evaluate the origin and proximal course of coronary arteries.
• • Considers aortic arch position.
• • Excludes related lesions like left superior vena cava.
• Typically, cardiac catheterization is not recommended in TOF. Nonetheless, it is advised if the architecture of the coronary
arteries cannot be identified or if it is not possible to rule out the existence of numerous VSDs.
• Exclude the possibility of DiGeorge syndrome (22q11-deletion): Get FISH probe investigations and chromosomal analyses for
every newborn with TOF, and keep a careful eye on calcium levels. (Domnina, Kerstein et al. 2020)

AHA 2018 guidelines:

• Adult congenital heart lesions require cardiac surgery, interventional cardiac procedures, or electrophysiologic procedures.
• AHA/ACC’s Anatomic/Physiologic (AP) categorization system places repaired tetralogy of Fallot in the moderate difficulty
category.
• Experts in echocardiography, CMRI, and ultrasonography preferred for cardiac imaging.
• Standard 12-lead ECG used in CHD patients, serial examination based on unique CHD AP classification.
• Physicians should use ambulatory electrocardiogram monitoring for arrhythmic etiology.
5.1 TOF Treatment Overview :
After a TOF is detected, nearly all patients have surgical resection within the first year of life. Prostaglandin therapy could be
required in the near term to preserve patent ductus arteriosus. Furthermore, if a patient exhibits symptoms of heart failure, they
might need to take digoxin or diuretics. The goal of hyper-cyanotic spell treatment is to increase pulmonary blood flow. These
include medications like oxygen, knee/chest position, morphine, venous fluid, sodium bicarbonate, beta-blockers, or
phenylephrine that raise systemic vascular resistance to drug delivery.
• Surgical resection is common for TOF patients within the first year.
• Prostaglandin therapy may be needed to preserve patent ductus arteriosus.
• Heart failure symptoms may necessitate digoxin or diuretics.
• Hyper-cyanotic spell treatment aims to increase pulmonary blood flow.
• Medications include oxygen, morphine, venous fluid, sodium bicarbonate, beta-blockers, or phenylephrine.
• Surgical intervention is required for hyper cyanosis symptoms or increasing cyanosis.
• Two common techniques: Blalock-Taussig shunt and subclavian artery shunt.
• Used for babies with unresolved headaches due to coronary artery architecture, hypoplastic pulmonary arteries, or premature
ejaculation.
• Further surgery is required as this is not a surgical method.
The simplest and most recommended remedy for individuals with TOF is intracardiac correction. This involves relieving all causes
of blockage and extending the RVOT in addition to closing the ventricular septal defect. In certain situations, removing the valve
could be necessary to remove the obstruction.
 5.2 The Blalock-Taussig Shunt for Tetralogy of Fallot: On November 29, 1944, Dr. Blalock and Vivien Thomas accompanied a
very sick, cyanotic, 4-kg, 15-month-old newborn girl with TOF to the surgery room.
The split proximal end of the left subclavian artery was anastomosed to the side of the left pulmonary artery via a left
anterolateral thoracotomy. Less than an hour and a half was needed for the procedure. The girl experienced turbulent
postoperative care due to many pneumothoraxs; nonetheless, she recovered well and was released from the hospital two
months following the procedure.[ contd] (Elsaka, Noureldean et al. 2022)
The Blalock-Taussig Shunt for Tetralogy of Fallot (Elsaka, Noureldean et al. 2022)

Classic Blalock Taussig shunt Modified Blalock Taussig shunt Pott’s shunt

Fixes the pulmonary artery to

the descending aorta

Bridges pulmonary artery to

Bridges the left /right subclavian artery with the
pulmonary artery to left help of Gore-Tex graft Waterston shunt
/right subclavian artery managing the proper blood
flow

Links the main /right

pulmonary artery to the
ascending aorta

[Image 7]
 5.2 Surgical interventions over the years: Lillehei et al. originally described surgical correction of ToF in 1955. A ventriculotomy
into the right ventricular anterior wall was used to approach the right ventricular outflow tract obstruction (RVOTO), and if
necessary, a transannular patch (TAP) was inserted to provide relief. Very few surgeons repaired total orthopedic failure (TOF) in
children under the age of five until the 1970s. Variable findings were found, with early death rates ranging from 5% to 14%.
Owing to a greater frequency of TAP and its possible long-term effects, including pulmonary insufficiency, some surgeons
advocated against early one-stage primary surgery. Nonetheless, neither longevity nor independence from reintervention
significantly differed between patients with and without TAP in a long-term follow-up study from Boston. When it comes to time
for a full repair, the two-stage surgery shunt may promote the development of the pulmonary valve and branch pulmonary
arteries, lowering the risk of a TAP. Pulmonary :
• Pulmonary regurgitation was found to be more frequent in patients with TAPs.
• Predicts worsening exercise performance and RV dilatation.
• Previously considered benign hemodynamic residual lesion.
• Biventricular dysfunction and ventricular arrhythmia linked to RV dilatation.
• Increased risk of sudden cardiac death in patients.
Proponents of complete repair during the neonatal period assert that there could be advantages, including normal somatic
growth and development, the removal of chronic hypoxemia, a reduction in the need for right ventricular muscle excision,
improved late ventricular function, and a lower risk of late dysrhythmias. Furthermore, one-stage restoration may reduce the
likelihood of hyper-cyanotic episodes, associated after-effects, and parental concern. Physicians who are against early one-stage
primary repair assert that there is a greater prevalence of TAP and that it may have long-term effects, including pulmonary
insufficiency.
Fraser and associates developed a method for modifying the surgical care for each patient. Patients with symptoms and those
without symptoms who weigh less than 4 kilograms underwent palliation with a BT shunt in the face of imminent pulmonary
artery isolation, followed by full repair at 6 to 12 months of age. At six months of age or later, primary repair was performed on
all other individuals. There were three late deaths (2.1%) and no early fatalities. The rate of reoperation was 3%.
5.3 Present days surgical approaches: While infundibulotomy, with or without an incision across the pulmonary valve annulus, is
the standard technique for complete surgical repair,
• Transarterial/transpulmonary or infundibular sparing may improve RV function and reduce arrhythmia risk.
• Valve-sparing procedures may cause chronic RV hypertension but offer long-term pulmonary valve competence.
• Mono-cusp repair approaches lack competency in late pulmonary valve replacement.
• Intraoperative pulmonary balloon angioplasty with full trans ventricular repair may maximize pulmonary annular development.
• Transarterial/transpulmonary approach is unaffected by an aberrant anterior descending coronary artery.
(Villafañe, Feinstein et al. 2013)
Differences in the current approaches to therapy:
Early Primary Repair of ToF
• Protects against RV pressure loading and decreased oxygen saturations.
• Preserves brain and cardiovascular function.
• Disagreements exist on "early" vs "later" repair.
• Neonatal repair, performed before one month, is possible but not commonly used.
• Neonatal repair often requires TAP, lowering event-free survival.
• Initial repair may wait until three to six months old with great results. (Van der Ven, van den Bosch et al. 2019)
Neonatal Intervention for Symptomatic ToF Patients :
• Neonatal stage intervention may be necessary.
• Systemic-to-pulmonary shunt, usually a modified Blalock-Taussig (mBT) shunt, enhances pulmonary flow, minimizes hypoxemia,
and allows PA development.
• Offers potential benefits of no or less extensive TAP, enabling repair at older age.
• Palliative shunt surgeries are linked to an early death risk of 3% to 5%. [contd]
Palliative Shunt Surgery Benefits :
• Potentially requires no or less extensive TAP.
• Enables repair at older age.
• Link to early death risk of 3%-5%.
Another method of preventing pulmonary blood flow after delivery is by stenting the ductus arteriosus (DA), which creates a
systemic-to-pulmonary shunt. Nevertheless, the architecture of the DA in cyanotic CHD may be intricate and inappropriate for
stenting. (Mouws, de Groot et al. 2019)
The expected procedural success rate is 83%.
RVOT Stenting in Newborn Patients :
• Used as a bridge to repair or palliative measure.
• Shows safety and encourages coronary artery development.
• No difference in survival between systemic-to-pulmonary shunt and RVOT stent.
• The RVOT stenting group has better perioperative problems, longer hospital stays, and intensive care.
• Greater risk of re-intervention. (Van der Ven, van den Bosch et al. 2019)
Residual Pulmonary Stenosis:
RVOT Desobstruction Success in TAP Repair
• Echocardiographic Doppler flow and systemic pressures in the RV are crucial for successful RVOT desobstruction.
• Muscle bundle resection in the RVOT is insufficient for limited transannular incision.
• Subvalvular desobstruction of the RVOT can extend residual PS independence without reintervention.[contd]
Vanderlaan, R. D. and D. J. Barron (2023).
ToF With PS: When to Perform Surgical Intervention:
• RVOTO degree and branch PAs size determine therapy.
• Symptoms range from valvular level, supravalvular area, or sub-valvular region.
• Severe infundibular constriction in sub-valvular area due to deviated conal septum and RVMB.
• Pulmonary valvular stenosis caused by dysplastic pulmonary leaflets and annular hypoplasia.
• Multilevel blockage may occur due to narrating of the main pulmonary artery and branch PAs at the supravalvular level. Surgical
Considerations for ToF with complete Surgical Repair :
• Can be performed as the first operation for newborns between three and six months of age, with ToF, or as the last step in a
stepwise repair plan for those with significant comorbidities or severe cyanosis.
• Lower surgery risk and lack of data to postpone repairs.
• Requires closing misaligned VSD and relieving RVOTO.
Ventriculotomy Repair Strategies :
• Minimize ventriculotomy necessity and complete repair transarterially.
• Decide whether to preserve the pulmonary valve's size and annulus or sacrifice it for a suitable outflow tract.
• Two main strategies: TAP repair and VSR (pulmonary valve-sparing repair).
• Primary concerns: intervention rates and RV function preservation.Vanderlaan, R. D. and D. J. Barron (2023).
TAP Method for RVOT Repair
• TAP is the most common method for RVOT repair.
• Involves an incision from the major pulmonary artery to the infundibular area.
• Removes pulmonary valve leaflet tissue and uses patch material to augment RVOT.
• Dissected RV muscle bundles to open the infundibular region.
• Restrictive physiology and poor cardiac output may complicate early postoperative course and lengthen ICU stay.
• Residual RVOTO at the infundibular level may persist.[ image 8]
Valve Sparing Procedure :
5.4 Reoperation: The most typical sign of late reoperation is PR
• Early reoperation is beneficial in youth or early adulthood.
• Options include stentless porcine, polyethylene terephthalate porcine, and bovine jugular conduits.
• Adults prefer stented pig valves due to their longevity and ease of future per-cutaneous valve replacement.
• Polytetrafluoroethylene bivalved prostheses lack late data, making percutaneous valve replacement impossible.
• Extracardiac conduits should be avoided, especially in TOF with pulmonary atresia.
• Homograft conduit is preferred in newborns and young children due to its durability.
• Secondary causes include RV dilatation, structural valve irregularity, chordal rupture during initial repair, ICD leads, or
permanent pacing. Tetralogy of Fallot in the Current Era - ScienceDirect
• Repair methods include eccentric, purse-string, or ringed annuloplasty.
• Tricuspid replacement may be necessary if previous treatments fail or leaflet abnormalities are significant.
• Atrial tachyarrhythmias are the most common, with radiofrequency ablation, cryoablation, right atrial maze, and biatrial maze
being the most effective treatments.
Re-interventions in Neonatal Repair :
• Most common re-interventions: re-ballooning and re-stenting.
• Lower incidence of re-shunt surgery or early full repair compared to initial mBT.
• Similar short- and long-term results were observed with neonatal repair and RVOT stenting.
• Higher risk of catheter-based re-interventions in RVOT stent group after 10-year follow-up
Reintervention Rates in TAP and Valve-Sparing Procedures :
• No difference was found in reintervention rates between TAP and valve-sparing procedures.
• Reintervention is mostly caused by persistent RVOT stenosis.
• Reintervention rates depend on age; less independence from reintervention is observed in children under two months.
• Previous studies linked less than three months before surgery to greater postoperative morbidity and higher reintervention
rates(Van der Ven, van den Bosch et al. 2019)
Significant Pulmonary Regurgitation* and repaired tetralogy of Fallot: Indications for pulmonary valve replacement:
(Shinebourne, Babu-Narayan et al. 2006)

Compelling Strong Debatable

• Physical Deterioration • QRS lasting RV volume .170
and ECG Symptoms longer than 180
• Subjective decline in ml/m2
physical capacity.
ms or increasing • 150 ml/m2
• Persistently low in sequence
objective exercise • Surgery is
capacity. recommended
• Syncope and VT
symptoms. for other
• Persistent VT on ECG reasons (such as
watch. persistent VSD)
• RV ejection fraction
reduction and serial
dilatation.
• Increases in RV end-
systolic volume.
5.4 Postoperative management: In the current era of surgical and postoperative care, the study details the 15-year result of
transarterial transpulmonary complete ToF correction. An excellent 15-year survival rate is shown by our data. Nonetheless, the
risk of death was increased for preterm or dysmature babies.
A small percentage of patients experienced tachyarrhythmias in the early postoperative period, and none of them needed
permanent pacemaker implantation as a result of early postoperative conduction abnormalities.
We saw a high proportion of residual PS and PR during long-term follow-up, which meant that a significant percentage of
patients needed reintervention. Following valve replacement surgery, the incidence of PR was reduced.
The occurrence of PR is concerning because it might cause dilatation of the RV and RA, which can eventually worsen cardiac
function and result in arrhythmia development. It is advised to have a prospective cardiac MRI follow-up.
Postoperative Issues in Young Patients :
• The primary cause of low cardiac output syndrome (LCOS) is right ventricular diastolic and systolic dysfunction.
• Less common left ventricular dysfunction.
• Uncontrolled arrhythmias like atrial ectopic tachycardia and junctional ectopic tachycardia (JET) documented.
• Remaining VSD due to serious VSD patch leak or previously unidentified VSD.
Heart Irregularities Post TOF Repair the following haemodialysis-significant arrhythmia types are most commonly encountered:
• Ectopic Tachycardia Junctional (5-20%)
• Ectopic Tachycardia atrial
• Re-entry type supraventricular tachycardia (Re-SVT)
• Whole AV Block (<5%) :
• Temporary but sporadically permanent (Domnina, Kerstein et al. 2020)

Post-surgery monitoring:
• Majority have pleural and mediastinal chest tubes, foley catheters, arterial line, central venous line, peritoneal drainage tube,
and temporary pacing wires.
• Older patients admitted post-extubation.
 REFERENCE LIST

Anderson, R. H., et al. (1981). "Surgical anatomy of tetralogy of Fallot." The Journal of Thoracic and Cardiovascular Surgery 81(6):
887-896.

Anderson, R. H., et al. (2013). "Tetralogy of Fallot: nosological, morphological, and morphogenetic considerations." Cardiology in
the Young 23(6): 858-866.

Apitz, C., et al. (2009). "Tetralogy of fallot." The Lancet 374(9699): 1462-1471.

Bailliard, F. and R. H. Anderson (2009). "Tetralogy of fallot." Orphanet journal of rare diseases 4: 1-10.

Chrysostomou, C., et al. (2010). "Tetralogy of Fallot." Critical Care of Children with Heart Disease: Basic Medical and Surgical
Concepts: 199-206.

Domnina, Y. A., et al. (2020). "Tetralogy of Fallot." Critical Care of Children with Heart Disease: Basic Medical and Surgical
Concepts: 191-197.

Elsaka, O., et al. (2022). "Tetralogy of Fallot: Diagnosis and Management." Asian Journal of Research in Medicine and Medical
Science: 18-31.

Ho, S., et al. (2001). "Anatomic–echocardiographic correlates: an introduction to normal and congenitally malformed hearts."
Heart 86(suppl 2): ii3-ii11.
Iecho, E. (2021). Tetralogy of Fallot, University of Zagreb. School of Medicine.

Mouws, E. M., et al. (2019). Tetralogy of Fallot in the current era. Seminars in Thoracic and Cardiovascular Surgery, Elsevier.

Pollack, R. W. (1948). "Tetralogy of fallot."

Shinebourne, E. A., et al. (2006). "Tetralogy of Fallot: from fetus to adult." Heart 92(9): 1353-1359.

Starr, J. P. (2010). "Tetralogy of Fallot: yesterday and today." World journal of surgery 34: 658-668.

Sukhacheva, T. V., et al. (2022). "Accelerated growth, differentiation, and ploidy with reduced proliferation of right ventricular
cardiomyocytes in children with congenital heart defect tetralogy of Fallot." Cells 11(1): 175.

Van der Ven, J. P., et al. (2019). "Current outcomes and treatment of tetralogy of Fallot." F1000Research 8.

van Praagh, R., et al. (1970). "Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and its sequelae." The
American journal of cardiology 26(1): 25-33.

Villafañe, J., et al. (2013). "Hot topics in tetralogy of Fallot." Journal of the American College of Cardiology 62(23): 2155-2166.

Wilson, R., et al. (2019). "Tetralogy of fallot." BJA education 19(11): 362.
Match the following questions:
1] Compare the following cardiac abnormalities with their descriptions that are observed in Tetralogy of Fallot:
A] blockage of the blood supply from the right ventricle to the pulmonary artery
B] improper interaction between the right and left ventricles
C] The location of the aorta above the left and right ventricles
D] Expanding the right ventricle's muscular wall
1] Right ventricular hypertrophy
2] Pulmonary stenosis
3] Ventricular septal defect
4] Overriding aorta
Ans 1]d, 2]A,3]B, 4]C
2] match the following signs or symptoms to the appropriate Tetralogy of Fallot subtype:
A] A chest X-ray showing a boot-shaped heart
B] "Tet" spells, abrupt dyspnea and cyanosis episodes
C] Due to right ventricular outflow tract obstruction
D] Due to right-to-left shunting of deoxygenated blood
1] Cyanosis
2] Cyanosis
3] Boot-shaped heart on X-ray
4] Tet spells
Ans:1 – D,2 – C,3 – A, 4 – B
3] Match the following Tetralogy of Fallot surgical treatments to their descriptions:
1]Tausig-Blalock shunt A. The interaction between the right pulmonary and right subclavian arteries
2]Rastelli technique B. Resolving the blockage of the right ventricular outflow tract and fixing the ventricular septal defect
3]The Waterston Shut C. Installing a conduit to connect the pulmonary artery and right ventricle
4]Assemble the following diagnostic tools or techniques (tetralogy of Fallot) according to their indicated uses:
1]Echocardiography A] Evaluate the extent of pulmonary stenosis
2]Cardiac catheterization B]. Assessing the structure and function of the ventricles
3]X-ray of the chest C.] to visualize the structure of the heart and recognize a boot-shaped heart
4]MRI or CT scan D.] Determining shunt ratios and oxygen saturation values
Ans :1 – B, 2 – D , 3 – C , 4 – A
5] Match the following Tetralogy of Fallot complications to their descriptions:

1]The polycythemia A. Increased synthesis of red blood cells due to reduced blood supply to the lungs
2]Paradoxical embolism B. Right heart chamber thrombus development with embolization to the systemic circulation
3]Brain abscess C. Septic emboli-induced brain tissue infection
4]Right-sided endocarditis D: Infection of the right side of the heart's endocardium or heart valves
Ans:1 - A , 2 – B, 3 – C , 4 - D
6]Assign the responsibilities to the following cardiac chambers involved in the Tetralogy of Fallot:

1] Right atrium A. Takes blood from the body that is deoxygenated

2] Right ventricle B: Delivers blood deficient in oxygen to the lungs
3] Left atrium C. Gets blood from the lungs that is oxygenated.
4] Left ventricle D: Provides the body with oxygenated blood
Ans: 1 – A, 2 - B ,3 - C , 4 – D
7]Sort the arteries implicated in the Tetralogy of Fallot according to their anatomical
connections:
1]Pulmonary artery A. Artery originating from the right ventricle carrying deoxygenated
blood to the lungs
8]Compare and contrast the descriptions of the following anatomical structures with their roles in the Tetralogy of Fallot

1] Pulmonary valve A. Narrowing of the pulmonary artery just above the valve
2]Right ventricular outflow tract B. Abnormal positioning, overriding both the right and left ventricles
3]Ventricular septum C. Enlargement due to increased pressure from right ventricular hypertrophy
4] Infundibular region D. Deficiency in the muscular portion leading to septal defect

Ans:1 – A, 2 – B, 3 – D, 4 - C
9]Match the following postoperative complications after Tetralogy of Fallot repairwiththeirdescriptions:
1]Arrhythmias
2]Pulmonary regurgitation
3]Right ventricular dysfunction
4] Residual ventricular septal defects.

A]Abnormalities in heart rhythm potentially requiring treatment.

B] Backflow of blood from the pulmonary artery to the right ventricle
C. Impaired function of the right ventricle leading to heart failure symptoms.
D]Persistence of communication between the ventricles after surgical repair
ANS: 1 - A ,2 - B ,3 - C , 4 – D
10] Assemble the following intraoperative factors for Tetralogy of Fallot repair according to
priority:
1]Cardiopulmonary bypass
2]Coronary artery assessment
3]Electrophysiological monitoring
4]Intraoperative transesophageal echocardiography
11] Compare the basic goals of the following surgical methods used to repair Tetralogy of Fallot:
1]Patch closure of the ventricular septal defect
2]Resection of infundibular stenosis
3]Placement of a conduit between the right ventricle and pulmonary artery
4]Creation of an anastomosis between systemic artery and pulmonary artery
A]. Relieve right ventricular outflow tract obstruction.
B] Improve pulmonary blood flow.
C] Prevent recurrence of ventricular septal defect.
D] Redirect systemic blood flow to the pulmonary circulation
ANS; 1 - C ,2 - A ,3 - B , 4 - D
Answer the following single best-choice questions :
1] Shortly after delivery, cyanosis is seen in a newborn baby. A systolic murmur is discovered on physical examination, and a
boot-shaped heart is shown on the chest X-ray. Which diagnosis fits the patient the best?
A) Ventricular septal defect (VSD)
B) Atrial septal defect (ASD)
C) Tetralogy of Fallot (TOF)
D) Patent ductus arteriosus (PDA)
E) Transposition of the great arteries (TGA)
ANS C

2] During weeping or nursing, a two-year-old infant exhibits bouts of abrupt onset dyspnea and cyanosis. For this patient,
which of the following is the best course of action for initial management?
F) An Oxygen supplementation
G) Intravenous fluids
H) Intravenous antibiotic
I) Surgical repair
J) Beta-blockers

ANS A
3] During cardiac catheterization, a patient with Tetralogy of Fallot shows a significant decrease in systemic vascular resistance
(SVR). Which of the following is the expected effect on the right-to-left shunt in this patient?
K) Increase in right-to-left shunt
L) Decrease in right-to-left shunt
M) No change in right-to-left shunt
N) increase in left-to-right shunt
4]Surgery is planned to correct the Tetralogy of Fallot in a 5-year-old child. Which surgical treatment entails the relief of right
ventricular outflow tract blockage and the patching of the ventricular septal defect?
A) Blalock-Taussig shunt
B) Waterston shunt
C) Rastelli procedure
D) Glenn shunt
E) Norwood procedure
F) ANS C
5] A 10-year-old boy with a corrected Tetralogy of Fallot has palpitations and sensitivity to activity. Right bundle branch block
and right axis deviation are visible on an ECG. Which of the following explanations fits these facts the best?
G) Recurrent Tetralogy of Fallot
H) Ventricular septal defect (VSD)
I) Atrial septal defect (ASD)
J) Right ventricular hypertrophy
K) Left ventricular hypertrophy
Ans D
6] What is the main goal of Tetralogy of Fallot surgical repair?
L) prevent ventricular septal defect recurrence
M) To reduce the size of the right ventricle
N) To relieve cyanosis and improve oxygenation
O) To correct left ventricular hypertrophy
ANS C
7] In Tetralogy of Fallot, what surgical treatment entails joining the left pulmonary artery to the left subclavian artery?
A) Waterston shunt
B) Blalock-Taussig shunt
C) Rastelli procedure
D) Glenn shunt
ANS A
8] Which structure is frequently employed as a conduit between the right ventricle and pulmonary artery during surgical repair
of Tetralogy of Fallot?
A) Left atrium
B) Aorta
C) Pulmonary valve
D) Homograft or prosthetic material

ANS : D
9] Which surgical technique is best for treating right ventricular hypertrophy and severe pulmonary stenosis in Tetralogy of
Fallot?
E) Waterston shunt
F) Glenn shunt
G) astelli procedure
H) Blalock-Taussig shunt
ANS C
10] Which surgical treatment in Tetralogy of Fallot attempts to enhance pulmonary blood flow by establishing an artificial link
between the aorta and pulmonary artery?
A) Glenn shunt
B) Waterston shunt
C) Rastelli procedure
D) Blalock-Taussig shunt
ANS D

11] What is the main purpose of expanding the right ventricular outflow tract during Tetralogy of Fallot surgery?
E) To reduce pulmonary blood flow
F) To relieve right ventricular hypertrophy
G) To improve pulmonary blood flow
H) D) To prevent ventricular septal defect recurrence
ANS C

12] Which surgical method, to enhance blood flow in Tetralogy of Fallot, entails installing a conduit between the pulmonary
artery and the right ventricle?
I) Blalock-Taussig shunt
J) lenn shunt
K) Rastelli procedure
L) Waterston shunt
ANS : C
13] In Tetralogy of Fallot, what surgical procedure entails establishing a link between the right pulmonary artery and the
superior vena cava to enhance pulmonary blood flow?
M) Blalock-Taussig shunt
14] In Tetralogy of Fallot, which surgical procedure aims to patch the ventricular septal defect and relieve right ventricular
outflow tract obstruction?
A) Glenn shunt
B) Rastelli procedure
C) Waterston shunt
D) Blalock-Taussig shunt
ANS: B
15] In Tetralogy of Fallot, what surgical treatment entails joining the right pulmonary artery to the right subclavian artery to
enhance pulmonary blood flow?
A) The Rastelli method
B) Blalock-Taussig shunt
C] Waterston shunt
D]Glenn Shunt
ANS B

16] Which physical characteristic of Tetralogy of Fallot increases the risk of infective endocarditis?
A) Ventricular septal defect
B) Right ventricular hypertrophy
C) Aortic override
D) Pulmonary stenosis
ANS A
17] Which structural abnormality causes the aorta to be displaced across the left and right ventricles in tetralogy of fallot?
E) Pulmonary stenosis
F) Aortic override
G) Ventricular septal defect
18]Which Tetralogy of Fallot clinical symptom is marked by abrupt bouts of dyspnea and cyanosis, usually brought on by eating
or weeping?
A) Tet spells
B) Clubbing of fingers
C) Right ventricular hypertrophy
D) Pulmonary stenosis
ANS A
19] What is the main reason why newborns with Tetralogy of Fallot exhibit cyanosis?

E) Decreased cardiac output

F) Ventricular septal defect
G) Pulmonary stenosis
H) Right-to-left shunting of deoxygenated blood
ANS D

20 ] Which anatomical abnormality causes blockage of blood flow from the right ventricle to the pulmonary artery in Tetralogy
of Fallot?
I) Pulmonary stenosis
J) Ventricular septal defect
K) Aortic override
L) Right ventricular hypertrophy
ANS A
Solve the following Drag and Drop type questions
1] Match the surgical procedures used in Tetralogy of Fallot with their anatomical outcomes:

 Blalock-Taussig shunt
 Rastelli procedure
 Waterston shunt
 Glenn shunt
Drag the surgical procedure to its corresponding anatomical outcome:

1. Abnormal positioning of the aorta, overriding both ventricles.

2. Narrowing of the pulmonary artery just above the valve.
3. Connection between the left subclavian artery and left pulmonary artery.
4. Connection between the superior vena cava and the right pulmonary artery.
Ans:
 Blalock-Taussig shunt: 2
 Rastelli procedure: 1
 Waterston shunt: 3
 Glenn shunt: 4
2] Match the anatomical structures involved in Tetralogy of Fallot with their surgical
interventions:
•Pulmonary valve
•Right ventricular outflow tract
•Ventricular septum
•Infundibular region
Drag the anatomical structure to its corresponding surgical intervention:
3]Match the surgical procedures used in the management of Tetralogy of Fallot with their descriptions:

 Blalock-Taussig shunt
 Rastelli procedure
 Waterston shunt
• Glenn shunt
Drag the surgical procedure to its corresponding description:

1. Connection between the right subclavian artery and the right pulmonary artery.
2. Patching the ventricular septal defect and relieving right ventricular outflow tract obstruction.
3. Placement of a conduit between the right ventricle and pulmonary artery.
4. Connection between the superior vena cava and the right pulmonary artery.
ANS:
•Blalock-Taussig shunt: 1
•Rastelli procedure: 2
•Waterston shunt: 4
•Glenn shunt: 3
4] Match the surgical procedures used in Tetralogy of Fallot with their primary goals:
•Blalock-Taussig shunt
•Rastelli procedure
•Waterston shunt
•Glenn shunt
Drag the surgical procedure to its corresponding primary goal:
1.To reduce pulmonary blood flow.
2.To relieve right ventricular hypertrophy.
5]Match the anatomical abnormalities commonly associated with Tetralogy of Fallot with their descriptions:

 Pulmonary stenosis
 Ventricular septal defect
 Overriding aorta
 Right ventricular hypertrophy
Drag the anatomical abnormality to its description:

1. Narrowing of the pulmonary artery just above the valve.

2. Abnormal communication between the right and left ventricles.
3. Aorta positioned over both the right and left ventricles.
4. Thickening of the muscular wall of the right ventricle.
•ANS:
•Pulmonary stenosis: 1
•Ventricular septal defect: 2
•Overriding aorta: 3
•Right ventricular hypertrophy: 4
6] Match the diagnostic tests or procedures used in Tetralogy of Fallot with their primary
purposes:
•Echocardiography
•Cardiac catheterization
•Chest X-ray
•MRI or CT scan
Drag the diagnostic test or procedure to its primary purpose:
1]TRUE or FALSE: Cyanosis in Tetralogy of Fallot is primarily caused by right-to-left shunting of deoxygenated blood.

Answer: TRUE
2] TRUE or FALSE: Tetralogy of Fallot is characterized by four distinct anatomical
abnormalities, including pulmonary stenosis, ventricular septal defect, overriding aorta, and
left ventricular hypertrophy.
•Answer: FALSE (Left ventricular hypertrophy should be replaced with right ventricular hypertrophy)
3] TRUE or FALSE: Tet spells, sudden episodes of cyanosis and dyspnea, are caused by
increased pulmonary blood flow during crying or feeding.
•Answer: FALSE (Tet spells are triggered by decreased pulmonary blood flow and increased right-to-left
shunting)
4] TRUE or FALSE: A boot-shaped heart on chest X-ray is a characteristic finding in Tetralogy
of Fallot, resulting from right ventricular hypertrophy.
•Answer: TRUE
5] TRUE or FALSE: The most common type of cyanotic congenital heart disease in children is
Tetralogy of Fallot.
•Answer: TRUE
6] TRUE or FALSE: Echocardiography is the gold standard diagnostic modality for evaluating
the anatomy and severity of cardiac abnormalities in Tetralogy of Fallot.
Answer: TRUE
7] TRUE or FALSE: Paradoxical embolism, leading to systemic embolization of thrombi, is a
common complication of Tetralogy of Fallot.
•Answer: TRUE
8] TRUE or FALSE: In Tetralogy of Fallot, surgical repair aims to increase pulmonary blood
flow and relieve cyanosis by creating a direct connection between the pulmonary artery and
Answer the following multiple-choice questions [ more than one option correct]
1]Which of the following anatomical abnormalities are typically seen in the Tetralogy of Fallot? (Select all that apply)
A) Pulmonary stenosis
B) Ventricular septal defect
C) Atrial septal defect
D) Aortic coarctation

Answer: A) Pulmonary stenosis, B) Ventricular septal defect

2] Which clinical manifestations are commonly observed in patients with Tetralogy of Fallot?
(Select all that apply)
A) Cyanosis
B) Clubbing of fingers
C) Bradycardia
D) Systolic murmur
Answer: A) Cyanosis, B) Clubbing of fingers, D) Systolic murmur
3] Which diagnostic tests or procedures are used to evaluate the Tetralogy of Fallot? (Select
all that apply)
E) Echocardiography
F) Cardiac catheterization
G) Electrocardiography
H) Chest X-ray
Answer: A) Echocardiography, B) Cardiac catheterization, D) Chest X-ray
4] Which complications are associated with Tetralogy of Fallot? (Select all that apply)
I) Paradoxical embolism
J) Polycythemia
5]Which anatomical features contribute to the characteristic boot-shaped heart appearance on chest X-ray in Tetralogy of
Fallot? (Select all that apply)
A) Right ventricular hypertrophy
B) Aortic override
C) Pulmonary stenosis
D) Ventricular septal defect

Answer: A) Right ventricular hypertrophy, B) Aortic override, C) Pulmonary stenosis

6] Which surgical procedures are commonly performed to manage Tetralogy of Fallot? (Select
all that apply)
A) Blalock-Taussig shunt
B) Rastelli procedure
C) Arterial switch operation
D) Fontan procedure
Answer: A) Blalock-Taussig shunt, B) Rastelli procedure
7] Which anatomical anomalies may lead to an increased risk of infective endocarditis in
Tetralogy of Fallot? (Select all that apply)
A) Ventricular septal defect
B) Pulmonary stenosis
C) Aortic coarctation
D) Atrial septal defect
Answer: A) Ventricular septal defect, B) Pulmonary stenosis
8] Which anatomical features are typically addressed during surgical repair of Tetralogy of
Fallot? (Select all that apply)
E) Pulmonary stenosis
9]Which surgical procedures aim to improve pulmonary blood flow in the Tetralogy of Fallot? (Select all that apply)
A) Blalock-Taussig shunt
B) Rastelli procedure
C) Waterston shunt
D) Glenn shunt

Answer: A) Blalock-Taussig shunt, B) Rastelli procedure

10] Which anatomical abnormalities may necessitate surgical intervention in the Tetralogy of
Fallot? (Select all that apply)
A) Pulmonary stenosis
B) Atrial septal defect
C) Right ventricular hypertrophy
D) Aortic coarctation
Answer: A) Pulmonary stenosis, C) Right ventricular hypertrophy