CASE REPORT

PEDIATRIC CARDIOLOGY

POST RIGHT VENTRICULAR OUTFLOW TRACT (RVOT)

STENTING IN TETRALOGY OF FALLOT

OLEH : dr. RUTH DIAN GIOVANNI

SUPERVISOR : dr. ALI NAFIAH NASUTION, SP.JP (K)

PROGRAM PENDIDIKAN DOKTER SPESIALIS

ILMU PENYAKIT JANTUNG DAN PEMBULUH
DARAH
DEPARTEMEN KARDIOLOGI DAN KEDOKTERAN
VASKULAR FAKULTAS KEDOKTERAN UNIVERSITAS
SUMATERA UTARA 2021
 CHAPTER 1
INTRODUCTION

Tetralogy of Fallot (ToF), the most common type of cyanotic congenital

heart disease (CHD), has an incidence of 0.34 per 1000 live births. The classic
tetrad was first described in 1673 by Bishop and anatomist Nicolas Steno, but the
anatomy was more extensively described by the French physician Étienne-Louis
Fallot in 1888. Patients with ToF have varying degrees of cyanosis depending on
the severity of right ventricular outflow tract (RVOT) stenosis and pulmonary
artery (PA) anatomy. The anatomic abnormalities seen in ToF vary from milder to
more severe phenotypes.
3.5% of all infants born with congenital heart disease have tetralogy of
Fallot, with males and females affected equally. The ventricular septal defect is
almost always large and non-restrictive in the tetralogy of Fallot, ensuring that the
pressure is equal in the two ventricles. Consequently, the loud systolic murmur
typical in affected infants originates from dynamic narrowing of the right
ventricular outflow tract. The direction and magnitude of flow through the defect
depend on severity of obstruction of the right ventricular outflow tract. If
obstruction to the right ventricular outflow is severe or if there is atresia, a large
right-to-left shunt with low pulmonary blood flow and severe cyanosis requiring
intervention at birth are present. However, most patients have adequate pulmonary
blood flow at birth but there is increasing cyanosis during the first few weeks and
months of life. In countries with well-developed pediatric cardiac services, severe
cyanosis, recurrent hypercyanotic spells, squatting, and other consequences of
severely reduced pulmonary blood flow are rare today. It is because a diagnosis is
seldom delayed and infants undergo palliative procedures or frequently complete
repair within the first few days, weeks, or months of life. Temporary treatment
with propranolol, which decreases right ventricular hypercontractility and heart
rate and increases systemic vascular resistance, is sometimes used to reduce the
incidence of hypercyanotic spells before surgery. Although an experienced
pediatrician or cardiologist usually suspect the diagnosis clinically; transthoracic
cross-sectional echocardiography provides a comprehensive description of the
intracardiac anatomy. With the exception of patients with major aortopulmonary
collateral arteries and rare cases in whom an echocardiographic assessment is

2
incomplete, any other diagnostic investigations (e.g., cardiac catheterization) are
now rarely performed before palliative or corrective surgery.
Before the advance of surgical intervention, more than 50% of patients
with tetralogy of Fallot died in the first few years of life, and it was unusual for a
patient to survive longer than 30 years. Nowadays, almost all those born with this
disease in all its variants can expect to survive with surgical correction and reach
adult life. Since the first reported intracardiac repair of the tetralogy in 1955, the
age of patients receiving primary corrective surgery has gradually decreased, with
some units advocating surgery at diagnosis, even within the first few days of life.
Most centres prefer to operate on children aged 3–6 months, preserving earlier
open-heart surgery for those presenting with severe cyanosis or hypercyanotic
spells. Some centres continue to offer surgical palliation by constructing a
systemic-to-pulmonary arterial shunt, balloon dilation, or placement of a stent in
the right ventricular outflow, in neonates and young infants, thereby deferring
intracardiac repair.
More studies are needed to determine the best strategy for the patient
group requiring early intervention. Management strategies likely need to be
individualized for optimal outcome. A case of right ventricular outflow tract
stenting in 40 years old man with TOF will be reported in this paper.

3
 CHAPTER 2
CASE REPORT

IN, a 40 years old Indonesian man, come to outpatient clinic with history of
worsening shortness of breath since the last 3 months. Shortness of breath also
accompanied by clubbing in the fingers since young age. Dyspnea on exertion was found
with increased activity. Orthopnea and paroxysmal nocturnal dyspnea was not found.
Ankle swelling was not found. Patient also complaint cyanosis in the hands, feet or mouth
if he did heavy activity. Chest pain is also denied. Patient said that during school age, he
was significantly smaller than his friends and had respiratory infection easily. History of
cyanosis in extremities or mouth was also found in young age, if patient did sports with his
friends. History of dyspnea released by squatting was found. History of paroxysmal
nocturnal dyspnea, orthopnea, chest pain, syncope, ankle swelling, and palpitation were not
found.
His mother did not complain any symptoms during pregnancy and no history of
taking drugs or alcohol. He is the second child of 4 siblings, with history of labour by
vaginal delivery, assisted by midwife with body weight 3000 grams, cried spontaneously
without cyanosis on delivery. Another siblings do not have the same symptoms.
On physical examination, he was fully alert with blood pressure 130/80 mmHg,
heart rate 102 beats per minute regular, respiratory rate 20 times per minute, and body
temperature 36.4o C. There is no pale on the conjunctiva and icteric on the sclera. The
jugular venous pressure was in normal range. From auscultation of the chest, the first and
second heart sound were normal, ejection systolic murmur grade III/6 in upper left sternal
border was found. There were no gallop or thrill. The respiratory sound was vesicular, and
there were no rales and wheezing. Liver and spleen were not palpable. All extremities were
cyanotic and clubbing fingers. The pulse oximetry 70% on the arms and legs.

4
 ECG showed sinus rhythm, QRS rate 98 bpm, QRS axis RAD, P pulmonal, PR int
0.16 seconds, QRS duration 0.08 seconds, ST segment was isoelectric, T wave normal, R
(+) in V1, S persistent in V5, LVH (+), VES (-). ECG was concluded with normal sinus
rhythm, Right Axis Deviation, RAE, LAE, RVH and LVH.

Figure 2.2. ECG of the patient showed sinus rhythm, RAD, RAE, LAE, LVH, RVH

Chest x-ray showed CT ratio 59%, normal aortic and pulmonary segment,
downward apex, infiltrate was not found, pulmonary vascular marking was decrease. Chest
X-ray conclusion was cardiomegaly and oligaemia.
 The laboratory finding on September 19 : Hb : 19,1 g/dL; Ht : 70%; WBC :
5.340/µL; PLT : 51.000/µL; Ureum : 28 mg/dL; Creatinin : 0.72 mg/dl; Sodium : 137
mEq/L; Potasium : 5,1 mEq/L; Chlorida : 104 mEq/L; Blood glucose level : 80 mg/dl;
Protrombin Time : 24.5 (14.7) second; INR : 1.8; APTT : 39.5 (32.2) second; Trombin
Time : 13.6 (15.7) second; pH : 7.3; pCO2 : 32 mmHg; pO2 : 148 mmHg; HCO3 : 15.7
U/L; Total CO2 : 16.7 U/L; BE : -9.4 U/L; O2 saturation : 99.7%.
Trans-thoracic echocardiography (TTE) on April 12, reported with: Atrial situs
solitus, AV-VA concordance, Pulmonary veins drained to LA, reduce PV flow. VSD
perimembranous diameter 14mm with overriding aorta 50%, without ASD or PDA with
RA-RV dilatation and right ventricular hypertrophy is present. The patient had a good left
ventricular systolic function with ejection fraction 56% (Teich), severe tricuspid
regurgitation with TRPG 104 mmHg and TR Vmax 5.1 m/s and severe infundibular
pulmonary stenosis (with TRPG 101 mmHg), with good right ventricular contractility
TAPSE 21 mm, PA confluence with RPA 1.15 and LPA 1.24, left aortic arch with no
coarctatio aorta. The conclusion were tetralogy of fallot.
 Figure 2.4. Echocardiography showed tetralogy of fallot

The patient was diagnosed with Tetralogy of Fallot. Patient underwent cardiac
catheterization on May 5th to determine source of pulmonary blood flow and delineate size
and distribution of true pulmonary arteries. RV graphy was performed with 35 mL
injection of contrast in RV, showed the contrast filled in RV, LV, PA, and Aorta. It showed
Tetralogy of Fallot (VSD, pulmonary infundibular stenosis), and overriding aorta. Then Ao
graphy was performed and showed two MAPCAS that came from descending aorta.
PA confluence with diameter RPA 12.6 mm, LPA 11.6 mm, and AoD 19.8 mm.
The pressures account are RV = 157/9; AO = 166/110/32; LV = 153/8 mmHg. From
data above, calculations as Nakata index and McGoon ratio were made. Patient had
confluent PA with Nakata index was 150.49 mm2/m2 and McGoon ratio was 1.2. After
catheterization patient was diagnosed with Tetralogy of Fallot, PA confluence and two
MAPCAS from AoD to upper lobe of right lung.
 Data were collected and patient then decided to be referred to Harapan Kita
Hospital to get treatment because lack of facility in Adam Malik Hospital. The
echocardiography was then performed there, the right ventricle is dilated and there is right
ventricular hypertrophy. Right ventricular systolic function is normal with TAPSE 18 mm.
Left atrial size is normal and right atrium is dilated. There was intact interatrial septum and
there was gap at IVS, with diameter of 22 mm, R-L shunt. There was moderate-severe
tricuspid regurgitation with TR Vmax 5.3 m/s. The pulmonic valve was not well visualized,
there was pulmonary stenosis (infundibulum and valvar with max RV-PA gradient 54
mmHg). BPV was performed on September 18 th with Thysak II Balloon 12.0 mm x 3.0 cm
x 90 cm (Figure 2.7). The balloon inflate in 2 atm once and then the balloon was deflated.
The balloon was pull a little and inflate in 3 atm twice. The saturation decrease when the
balloon inflate but then increase again when the procedure was complete
, with SaO2 60% before procedure and SaO2 93% after procedure (Figure 2.7). Residual PS
was found. Furosemide 60 mg (iv) given when the balloon inflate and then continue drips
Furosemide 10 mg/hour until urine output 3-4 L.
 Figure 2.7. The Thysak II Balloon 12 mm x 3.0 cm x 90 cm inflate at the pulmonal valve

Follow up on September 19th patient had no shortness of breath, blood pressure

120/80 mmHg, heart rate 64 beats/min, respiratory rate 20 x/minute, and saturation 88-89%
on the arms and 87-88% on the foot. Patient was discharge on September 21 th with stable
condition and medication with Furosemide 1x40 mg, propranolol 3x15 mg, and Ramipril
1x2.5 mg. For further management, data were collected for surgical conference.
 CHAPTER 3
DISCUSSION

3.1 Tetralogy of Fallot

3.1.1 Epidemiology
Tetralogy of Fallot is the most common cyanotic heart condition in children who
have survived untreated beyond the neonatal age, with the need for an intervention in the
first year of life. It accounts for 7% to 10% of congenital defects, affecting males and
females equally and occurring in 3 to 5 of every 10,000 live births.1 It represents 10% of
congenital heart disease in children. Coronary artery anomalies (CAA) have been reported
in 2% to 14% of patients with TOF according to angiographic, surgical, and autopsy
series.2

3.1.2 Embryology
The development of the human heart starts around the 20th day of gestation, with
the fusion of the outer endocardial tubes into a single tubular structure, the cardiac tube.
Subsequently, the cardiac tube folds and loops, with the development of an atrium that is
cranial and dorsal, and a primitive ventricle is moving downward, ventrally and to the right.
The right ventricle is the dominant chamber in the embryo and fetus, receiving 65% of the
venous return, and is the main contributor to the lower part of the body, the placenta, and
the lungs. The right ventricle can be described by three components: the inlet, which
consists of the tricuspid valve chordae tendineae and papillary muscles; the trabeculated
apical myocardium; and the infundibulum or conus.1

Tetralogy of Fallot is the most commonly

occurring conotruncal
anomalies.
8
It is a failure in the last step of cardiac looping,
wedging,
and essentially is the result of an arrest of the
rotation of the outflow
tract at the base of the great arteries.
9
The embryological origin of the
four anomalies that make up Tetralogy of
Fallot is a misalignment of
the outflow tract and the ventricles in the
primitive heart.
9
Due to the
misalignment, the septa fail to fuse, creating a
VSD
Tetralogy of Fallot is the most commonly
occurring conotruncal
anomalies.
8
It is a failure in the last step of cardiac looping,
wedging,
and essentially is the result of an arrest of the
rotation of the outflow
tract at the base of the great arteries.
9
The embryological origin of the
four anomalies that make up Tetralogy of
Fallot is a misalignment of
the outflow tract and the ventricles in the
primitive heart.
9
Due to the
misalignment, the septa fail to fuse, creating a
VSD
Tetralogy of Fallot is the most commonly
occurring conotruncal
anomalies.
8
It is a failure in the last step of cardiac looping,
wedging,
and essentially is the result of an arrest of the
rotation of the outflow
tract at the base of the great arteries.
9
The embryological origin of the
four anomalies that make up Tetralogy of
Fallot is a misalignment of
the outflow tract and the ventricles in the
primitive heart.
9
Due to the
misalignment, the septa fail to fuse, creating a
VSD.
9,10
Due to this misalignment, the aortic root then
overrides the inter-
ventricular septum, and, therefore, the VSD.
10,11
The outflow tract
becomes narrow due to both the misalignment
factor as well as
hypertrophy of the setpoparietal trabeculations
(which attach to the
moderator band on one end and the parietal
wall of the right ventricle
on the other).
12
The deviated outlet septum and the
hypertrophied
septoparietal trabeculations produce the
characteristic right ventricu-
lar outflow tract obstruction of tetralogy of
Fallot
Tetralogy of Fallot is the most commonly occurring conotruncal anomalies. It is a
failure in the last step of cardiac looping, wedging, and essentially is the result of an arrest
of the rotation of the outflow tract at the base of the great arteries. The embryological
origin of the four anomalies that make up Tetralogy of Fallot is a misalignment of the
outflow tract and the ventricles in the primitive heart. Due to the misalignment, the septa
fail to fuse, creating a VSD. Due to this misalignment, the aortic root then overrides the
interventricular septum, and, therefore, the VSD. The outflow tract becomes narrow due to
both the misalignment factor as well as hypertrophy of the septoparietal trabeculations
(which attach to the moderator band on one end and the parietal wall of the right ventricle
on the other). The deviated outlet septum and the hypertrophied septoparietal trabeculations
produce the characteristic right ventricular outflow tract obstruction of tetralogy of Fallot.3

Figure 3.1 Fetal cardiac circulation in normal fetal heart and heart with severe pulmonary
stenosis as part of Tetralogy of Fallot

3.1.2 Classification
The two main variations of Tetralogy of Fallot are Tetralogy of Fallot with
pulmonary atresia and Tetralogy of Fallot with pulmonary stenosis. In pulmonary atresia,
the RVOT ends in a blind pouch.3
 Figure 3.2 Tetralogy of Fallot with pulmonary stenosis vs pulmonary atresia

Fallot-type pulmonary atresia (15% of ToF), is the severest variant, characterized by

complete atresia (i.e. complete occlusion) of the pulmonary valve; therefore, there is no
forward flow of blood from the RV into the pulmonary artery. Intracardiac mixing is essential,
and all pulmonary blood flow must be supplied from the aorta either by a PDA (‘duct-
dependent pulmonary circulation’) or from major collaterals from the aorta to the pulmonary
arteries (major aortopulmonary collateral arteries, MAPCAs). In the neonatal period,
prostaglandin (either alprostadil [prostaglandin E1] or dinoprostone [prostaglandin E2]) may be
required acutely to maintain any pulmonary blood flow through the PDA.
ToF with absent pulmonary valve (6% of ToF), there is no pulmonary valve, but the
RVOT is open. These infants are often acyanotic because there is no RVOTO, but the
condition is notable for respiratory complications that develop secondary to massive
aneurysmal dilatation of the pulmonary arteries caused by absence of the pulmonary valve,
with obligatory pulmonary regurgitation. These aneurysmal dilatations externally compress the
distal trachea and bronchi causing intrathoracic airway obstruction, lung atelectasis, and even
pulmonary hypoplasia.4
 Figure 3.3 Representation of different anatomical correlates in Tetralogy of Fallot

3.1.2 Pathophysiology
The anatomy of ToF allows mixing of blood between the pulmonary and systemic
circulations. This mixing usually occurs at the VSD, with a right-to-left shunt adding
deoxygenated blood to the systemic circulation, causing cyanosis. The right-to-left shunt
through the VSD is determined by the relative pressure gradient between the RV and LV.
The amount of pulmonary blood flow (the RV stroke volume) is determined by the degree
of the RVOTO. A large reduction in systemic vascular resistance (SVR), in the presence of
RVOTO, will exacerbate any right-to-left shunt by reducing the LV end-diastolic pressure
(LVEDP), thus increasing the right-to-left pressure gradient; an increase in SVR will do the
opposite and forms the basis of treatment for spelling episodes.1

In the neonatal period pulmonary vascular resistance (PVR) is higher than in adults
and can be fluctuant. Any increase in PVR may further increase the RV afterload adding to
any RVOTO, and drive a greater right-to-left shunt. With any of the physiological changes
described previously that cause an increased right-to-left shunt, it is important to remember
that cardiac output is often maintained initially because the shunt desaturates systemic
blood, but maintains the LV preload and cardiac output, albeit with deoxygenated blood. In
addition to the right-to-left-shunt dynamics, the secondary RVH also has additional
implications for the clinical management of these infants.1
 Muscle fibre changes in RVH cause RV diastolic impairment, leading to impaired
lusitropic function (poor relaxation and diastolic filling). Any additional tachycardia will
cause a proportional reduction in diastolic filling, further reducing the stroke volume whilst
increasing the oxygen demand. Because of the diastolic dysfunction, these patient have an
increased RV end-diastolic pressure and are dependent on increased venous return for RV
filling. The physiological process surrounding the hypercyanotic episodes or “Tet spells” in
tetralogy of Fallot consist of either a decrease in systemic vascular resistance or an increase
in pulmonary resistance contributing to a right-to-left shunt across the ventricular septal
defect, causing marked desaturation.1,4

Figure 3.3

Mechanism of cyanotic spell

3.1.3 Clinical Manifestations
Due to the presence of collaterals, onset of symptoms in neonatal age is rare, and
clinical manifestation of the disease can vary widely depending on the anatomy and
physiology of pulmonary blood sources. Clinical presentation also varies based on the
severity of the right ventricular outflow tract obstruction, more commonly presenting as
neonates with a certain degree of cyanosis. In some patients cyanosis present months later in
life, when the rate of obstructions worsens. “Tet spells” or hypercyanotic episodes present
during the infancy or toddler age and decrease after 4 to 5 years of age.1

Dehydration or agitation commonly precipitate tet spells, and if patients do not

receive prompt and adequate treatment, they can develop severe cyanosis and hypoxia that,
subsequently, can cause syncope and even death. Rarely, patients present with clubbing, this
is usually seen in those with severe, longstanding cyanosis. (Jose Diaz) During the spell the
infant turns bluer because of increased right to left shunt from decreased systemic vascular
resistance followed by hyperventilation to compensate for metabolic acidosis and then
becomes limp, listless, and may develop seizure and even cerebrovascular accident (CVA).5

On auscultation, patients have a normal first heart sound with a single second heart
sound that is loud. The greater the degree of obstruction, the more prominent the murmur,
usually described as crescendo-decrescendo with a harsh systolic ejection quality and best
heard at the left mid to upper sternal border with posterior radiation. Sometimes the murmur
can have a regurgitant quality, and an early systolic click may be auscultated along the left
sternal border. Heart murmur disappears during the spell because of diminished blood flow
to the pulmonary artery. A prominent ventricular impulse and a systolic thrill may be
appreciable, hepatomegaly is uncommon, and if there are prominent pulses present, it may
be an indirect indication of a patent ductus arteriosus or aorticopulmonary collaterals.1

Very rarely congestive heart failure is seen at around 6 weeks of age only when the
RVOT obstruction is mild therefore, left to right shunt through VSD. This is also known as
“acyanotic TOF”. Usually infants and children are not failure to thrive unless associated
with genetic disorder. Older children present with easy fatigue and squatting during physical
activities. Cyanotic spell is less common at this age because they learn their limitation of
physical activities and squatting prevents cyanotic spell. Clubbing of the finger and toe nail
beds develops at around 18 months of age.4,5

3.1.4 Electrocardiography
Preoperatively, the electrocardiogram in TOF characteristically shows sinus rhythm
with normal or rightward axis deviation and right ventricular hypertrophy. As previously
mentioned, there may be left axis deviation in TOF associated with AVSD. Surgical repair
of TOF often disrupts the electrical conduction pathways and afterwards >90% of patients
exhibit right bundle branch block (RBBB), although this has become less marked with the
transatrial and transpulmonary repair. As discussed below, in patients left with residual PR,
the QRS duration (QRSd) tends to increase over time and may have prognostic implications.
prominent R waves anteriorly and S waves posteriorly, upright T wave in V1 (abnormal
after 7 days of life up to 10 years of age) and a qR pattern in the right precordial leads.6,7
3.1.6 Chest X-Ray
The typical chest radiograph findings in TOF are well known and consist of a
normal cardiac size but with an abnormal morphology characterized by an upturned apex
("bootshaped" heart) related to RV hypertrophy, deficiency of the main PA segment ("PA
bay" visualized as a notch in the upper left cardiac border), and reduced pulmonary
vascularity. However, historically, an upturned apex was only actually present in two-thirds
of cases and a more consistent sign of RV hypertrophy was said to be increased proximity
of the anterior cardiac border to the sternum on a lateral film. Today, since most patients
are operated during infancy and before they develop significant RV hypertrophy, the
typical "bootshaped heart" is much less commonly seen. It is usually possible to diagnose a
right-sided aortic arch from the chest x-ray by absence of the usual left-sided aortic
knuckle, a bulge to the right of the upper mediastinum, and an impression to the right of the
trachea. After surgery, right bundle branch block with prolongation of the QRS duration
is frequent.6,7

3.1.7 Echocardiography
The implementation of the “three-vessel view” in fetal morphology ultrasounds has
shown a significant increase in the antenatal detection rates of Tetralogy of Fallot. the
three-vessel view is found superior to the four-chamber view and assesses the aorta,
pulmonary artery, and superior vena cava. Usually the fetal pulmonary artery width
measures equal to, or slightly larger than, the aortic width. An AA/PA ratio greater than 1
is a proven indicator of some sort of cardiac complication, one study suggesting an 81%
chance of Tetralogy of Fallot.1

Transthoracic cross-sectional echocardiography usually allows clear demonstration

of all the intracardiac anatomy. As always, a sequential approach is used. The subcostal
paracoronal view shows the narrowed subpulmonary outflow tract, with malalignment of
the anteriorly displaced muscular outlet septum. Parasternal long-axis views demonstrate
the aortic override. Biventricular connection is seen also in the anteriorly tilted four-
chamber view, but this is less reliable at defining the degree of override. If present, a
straddling or overriding tricuspid valve is also seen in the four-chamber view, as is the
common atrioventricular junction in hearts with deficient atrioventricular septation. These
views, together with a parasternal short-axis view, allow the margins of the ventricular
septal defect to be identified.1

Views of the aortic arch show the pattern of branching of the brachiocephalic
arteries, a right brachiocephalic artery usually being found when the aortic arch is left-
sided. When the first branch from the arch itself divides into left carotid and subclavian
arteries, it can be inferred that this vessel is the brachiocephalic artery and that the arch
itself is right-sided. These views will also show a persistent arterial duct, if present, while
color-flow Doppler can confirm patency of previous palliative shunts and may show
collateral arteries. Anomalous arteries can be identified crossing the subpulmonary
infundibulum and their precise origin now determined. Similarly, the postoperative
assessment of the early surgical result, the presence of residual lesions, and their
hemodynamic sequalae, are all well demonstrated by transthoracic echocardiography. The
assessment of pulmonary regurgitation and its secondary effects on right ventricular
performance can be assessed qualitatively.1,3

3.1.8 Cardiac Catheterization

In most large units, diagnostic cardiac catheterization is now rarely performed prior
to palliative or corrective surgery. Cardiac catheterization is reserved only for patients with
specific unanswered questions after echocardiographic study. Patients with multiple
aortopulmonary collateral arteries are a frequent exception to this rule, although the origin
and course of such vessels will often have been demonstrated by prior MRI or CT. The role
of cardiac catheterization in such patients is therefore to assess the hemodynamics within
and connections between the individual vessels when necessary. Interventional
catheterization remains applicable for some patients. Dilation and stenting of the right
ventricular outflow tract, with prior radiofrequency perforation if atretic, has a role in some
instances. Similarly, preoperative balloon dilation of the pulmonary arteries, stenting of the
arterial duct, and interventions on the aortopulmonary collateral arteries may be part of a
combined surgical and medical program of management, often with the need for additional
postoperative interventions in the more challenging cases.6,8

Cardiac catheterization is not commonly used but can help to assess the level of
obstruction, pulmonary stenosis or hypoplasia, coronary artery anatomy, and the presence
of collaterals and accessory septal defect s.1 ‘Suspected coronary artery anomalies’ can
be difficult to detect by echocardiography. Coronary angiography is also indicated if
surgical re intervention is necessary. Balloon dilatation of the pulmonic valve is rarely
indicated to delay neonatal surgery in some centers. Cardiac CT scan can define the cardiac
anatomy and the need for catheterization is declining in most of the cardiac center.5

3.1.9 Treatment
3.1.9.1 Medical
When treating patient undergoing a hypercyanotic crisis, the first actions should be
to place the patient in the knee-chest position and administer oxygen by face mask. If the
patient is extremely restless, an intravenous line should be inserted and a small dose of
morphine sulphate, 0.1 mg/kg, may be all that is required to abort the crisis. If this fails,
treatment with a β-blocking agent such as propranolol will reduce tachycardia and increase
systemic resistance. It may also reduce hypercontractility in response to the endogenous
release of catecholamine, but there is no evidence for a specific effect to reduce
infundibular muscular spasm. The drug should also be administered intravenously. Half of
this should be given rapidly and the remaining half more slowly over the next few minutes.
If this fails to lead to prompt improvement, arterial blood gases should be assessed.
Accompanying metabolic acidosis should be corrected. Intubation and ventilation may be
required in extreme cases, and at this stage an intravenous vasoconstrictor, such as
phenylephrine, is often effective. Rarely it will be necessary to construct an emergency
systemic to-pulmonary arterial shunt. In any case, the patient should immediately be
referred for definitive intervention and treated orally with propranolol during the interim.6

The pathophysiology of hypercyanotic spells should be thought of as an acute

imbalance between systemic and pulmonary blood flow resulting from a vicious spiral of
changes in inotropy secondary to endogenous catecholamine release, increased systemic
oxygen consumption, reduced systemic vascular resistance, and reduced RV preload due to
increased heart rate. The aim of therapy is to redress this imbalance and disrupt the
pathophysiologic spiral by relieving pain and anxiety (to reduce heart rate and systemic
oxygen consumption), increase systemic vascular resistance, and increase pulmonary blood
flow. Since most hypercyanotic spells are provoked, or worsened, by crying, the infant
should be picked up and comforted as soon as an episode begins, ideally while being held
in a position of flexed knees and hips that kinks or compresses the femoral arteries and
increases peripheral systemic vascular resistance. If no improvement is seen within a few
minutes, oxygen should be administered, and intravenous access obtained. Most spells are
self-limiting and do not require intensive medical therapy. Many groups consider their
onset as an indication for surgical correction, but interval prophylaxis with beta-receptor
antagonists (oral propranolol in a dose of 0.25 to 1 mg/kg,2 to 3 times per day) may be
helpful if surgery is delayed.6–8

3.1.9.2 Surgical
The historical evolution in treatment for TOF is of importance to the current
management of patients with cyanotic congenital heart disease. Alfred Blalock, Helen B.
Taussig, and Vivien Thomas developed the first Blalock-Taussig-Thomas (BTT) shunt as a
palliative procedure to augment pulmonary blood flow in a 4 kg, 15 month-old girl with
TOF at Johns Hopkins in 1944. The operation involved the formation of a systemic (left
subclavian artery) to pulmonary artery shunt, creating an artificial ductus arteriosus.
Taussig had previously observed the benefits of an open ductus arteriosus in children with
cyanotic heart disease (in addition to the fatal effects of early ductal closure in this same
patient population), recognizing that these cyanotic babies were dying of anoxemia and not
cardiac failure, as previously believed. The original BTT shunt was the first attempt to
build an “artificial ductus” in a cyanotic child to restore pulmonary blood flow and improve
cyanosis.7,9

Over time, several alternative approaches and modifications to the original BTT
shunt were developed, including a number of central shunts: Potts (descending aorta to left
pulmonary artery anastomosis), Waterson (ascending aorta to pulmonary artery
anastomosis), and Cooley (intrapericardial anastomosis from ascending aorta to right
pulmonary artery) shunts. The original direct systemic to pulmonary artery anastomosis of
the BTT shunt has evolved into the current modified BTT shunt, in which a prosthetic tube
graft is interposed between the subclavian and pulmonary arteries. In current practice, if a
palliative shunt is indicated, the most common approach is by either a modified BTT shunt
or a central shunt from the ascending aorta to main pulmonary artery. Both approaches
utilize a polytetrafluoroethylene prosthetic graft. The modified BTT shunt can be
performed by either a posterolateral thoracotomy or median sternotomy approach, while a
central shunt requires a median sternotomy.9

Figure 3.4 Palliative procedures that can be performed in patients with cyanotic cardiac
defect with decreased pulmonary blood flow. The Gore-Tex interposition shunt (or modified
Blalock-Taussig shunt) is the most popular systemic–to–pulmonary artery (PA) shunt procedure.

Complete corrective TOF surgery is usually performed before 6 months of age as

open intracardiac surgery performed under cardiopulmonary bypass conditions in one
operation. It is performed relatively early in life to reduce the pathophysiological
adaptation of the TOF physiology, this shift in timing has led to an observed reduction in
mortality by improving pulmonary vasculature development early on, reducing RVH and
subsequent fibrosis.4

Intracardiac repair involves reconstituting the ventricular septum and relieving

subpulmonary obstruction. The operation is now rarely performed via a large
ventriculotomy, with transatrial closure of the septal defect now almost universal. Any
ventriculotomy, even when it involves a transjunctional incision to relieve obstruction in
the subpulmonary outlet, is minimized given the late results now being observed in
survivors of correction as they reach adulthood.6

The hypertrophied outlet septum together with its parietal and septal extensions are
excised as indicated. In the past, whether a transjunctional patch, usually described as a
“transannular” patch (although of course the arterial valvar leaflets are not supported in
annular fashion) would be necessary was often decided on the basis of measurements made
on preoperative angiograms to determine the sizes of the pulmonary arteries relative to the
aorta. The need for a transannular patch can also be determined during the operation by
using Hegar dilators to measure the narrowest part of the pulmonary outflow tract. “Free”
pulmonary incompetence is inevitable when a patch is placed across the
ventriculopulmonary junction. A unicusp aortic homograft (“monocusp”) has been used by
some in an attempt to avoid this complication, but no evidence has accrued to show that the
late results of this strategy are superior to the use of a nonvalved patch. The ventricular
septal defect is closed using a patch of knitted Dacron, Teflon, or pericardium secured with
either continuous or interrupted sutures.6

Surgery should ideally result in normal right ventricular pressures, absence of any
gradient in pressures between the right ventricle and the pulmonary arteries, and a
competent pulmonary valve. Unfortunately the nature of the subpulmonary obstruction
rarely allows this ideal result. The last decade has seen a shift away from the obsession
with complete relief of obstruction and toward attempts to preserve the pulmonary valve,
even at the expense of a modest degree of residual stenosis. The belief is that this will
minimize the adverse late effects of pulmonary incompetence and retain the integrity of the
outflow tract, avoiding late dilation and the formation of aneurysms. The success of such
an approach or course remains to be seen. The timing of definitive repair must be based on
the results in the institution offering treatment. Although the literature is replete with
excellent results, even when these procedures are performed routinely in the neonatal
period, such outcomes are of little relevance to a unit that cannot provide an optimal level
of intraoperative and postoperative care.6

Figure 3.4 Total correction of tetralogy of Fallot (TOF). A, Anatomy of TOF showing a large
ventricular septal defect (VSD) and infundibular stenosis seen through a right ventriculotomy. Note
that the size of the ventriculotomy has been expanded to show the VSD. B, Patch closure of the VSD
and resection of the infundibular stenosis. C, Placement of a fabric patch on the outflow tract of the
right ventricle (RV).

Figure 3.5 Surgery approaches for tetralogy of Fallot (TOF).

3.1.9.3 Transcatheter management

The transcatheter management of congenital heart disease has dramatically evolved
over the past two decades. Modern coronary interventional kit is suitable for catheter
interventions in even premature neonates. As a consequence of this, and with the
experience of stent implantation into the patent arterial duct or stenosed BT shunts, there
has been a re-consideration of stenting the right ventricular outflow tract (RVOT) in
patients with tetralogy of Fallot.10

According to Quandt et al in his study about RVOT stenting and BT shunt, Hospital
stay, post procedure time of ventilation, PICU requirement (22% versus 100%, p < 0.001),
post procedure morbidity and mortality was significantly lower compared to a group of
patients undergoing BT shunt. There was significant growth of the branch pulmonary
arteries over time. In a subgroup of patients with true tetralogy of Fallot the growth of the
branch pulmonary arteries was found to be significantly better after RVOT stenting
compared to BT shunts. Stenting of the RVOT is an effective and safe technique in the
initial palliation of selected patients with Fallot-type lesions. It increases pulsatile forward
flow of systemic venous blood to the PAs. In the group of patients who underwent initial
RVOT stenting, there was better and more uniform growth of the branch PAs compared
with the modified BT shunt. There was an overall benefit of RVOT stenting over mBTS in
terms of pulmonary arterial size before corrective surgery. The time to complete repair was
markedly shorter compared with the mBTS group. This, in part, was related to the
improved growth of the PAs, but also because of a trend to perform complete repair at an
earlier age. Complete repair in either group could be achieved without mortality. There
were no significant differences in the rate of transannular patching or the need for a cardiac
conduit. In contrast, the group who underwent mBTS required pulmonary patch
arterioplasty more frequently. Most of the mBTS surgeries were performed during the
earlier period of this retrospective study, whereas more RVOT stents were performed in
more recent years.11

In Abumehdi et al study, they have demonstrated a significant improvement in

pulmonary artery size in relation to body surface area over a median follow-up duration of
220 days following right ventricular outflow tract stent insertion. Furthermore, there was a
significant improvement in oxygen saturations following the procedure, allowing for a safe
delay of surgical correction in the majority of patients.12

RVOT stenting provides less durable palliation than a conventional surgical shunt
due to tissue reaction and fibrous tissue ingrowth, which tends to prolapse through the stent
struts. The use of newer technologies, such as drug-eluting stents, could overcome this
shortfall and improve the long-term patency of the stented outflow tract. Despite the lower
durability than the conventional surgical shunt, stenting of the RVOT results in a greater
rise of oxygen saturation and promotes better and more constant symmetrical pulmonary
arterial growth than mBTT shunt palliation. It is possibly due to a balanced flow to main
pulmonary artery branches compared with the unfavorable graft geometry of a surgical
shunt, which usually causes overgrowth of the ipsilateral pulmonary artery and lesser
development of the contralateral pulmonary artery.13
3.1.10 Prognosis
Actuarial survival was overall at 86% among patients surviving 30 days after
complete repair compared with 96% in the control population. Further analysis showed
rates of survival after 30 years of 90%, 93%, and 91% in patients undergoing surgery
younger than the age of 5 years, from 5 to 7 years, and at 8 to 11 years, respectively. Of
patients who were 12 years of age or older at the time of operation, only 76% were alive
compared with 93% in controls. Although reflecting a success story in terms of
modification of the natural history of the disease, it became clear and increasingly the case
that the late mortality after definitive repair will far exceed the early postoperative risk.
Consequently the past decade or so has seen a shift toward improved understanding of the
determinants of late outcomes, with concentration on the adverse effects of pulmonary
incompetence, which was previously considered to be a benign side effect of relief of the
obstructed right ventricular outflow tract. It is important to note that although TOF repair is
now associated with little short- or medium-term mortality, longterm survival is not equal
to that of the general population. For the 30-year-old with repaired TOF, there is a 0.5%
annual risk of death.6,7
 CHAPTER 4
CONCLUSION

It has been reported a 18-years-old woman with unusual case of Pulmonary valvar
stenosis, Tetralogy of Fallot and Right Aortic Arch . Her chief complain was shortness of
breath, limitation of activities, cyanosis, and clubbing finger. Physical examination all
extremities were cyanotic and clubbing fingers. The ECG was sinus rhythm, right axis
deviation, RAE and RVH. Decrease pulmonary vascular appearance was found in chest X-
ray. Echocardiography conclusion were pulmonary valvar stenosis, tetralogy of fallot and
right aortic arch
Patient underwent cardiac catheterization to determine source of pulmonary blood
flow and delineate size and distribution of true pulmonary arteries. PA confluence with
diameter RPA 10.6  1 mm, LPA 10.9  1 mm, and AoD 17.2  1.6 mm. The pressures
account are RA = a:12 v:4 m:8; LA = a:88 v:3 m:10; RV = 85/8; AO = 89/62/74; LV =
88/3 mmHg. From data above, calculations as Nakata index and McGoon ratio were made.
Patient had confluent PA with Nakata index was 131 mm2/m2 and McGoon ratio was 1.25.
After catheterization patient was diagnosed with Tetralogy of Fallot ( VSD, pulmonary
valvular stenosis and pulmonary infundibulum stenosis), overriding aorta and right aortic
arch. Transcatheter balloon pulmonary valvuloplasty was performed on September 18th
with Thysak II Balloon 12.0 mm x 3.0 cm x 90 cm. The saturation decrease when the
balloon inflate but then increase again when the procedure was complete , with SaO 2 60%
before procedure and SaO2 93% after procedure. For further management, data were
collected for surgical conference.
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