310

TETRALOGY OF FALLOT
+ MULTIPLE CARIES
KASUS

• An. V, 4 tahun, BB 12,6 kg

• Dk/ Multipel Caries + TOF
• R/ Mouth Preparation

• Riwayat kebiruan dari lahir

• Kebiruan muncul jika menangis atau saat anak
kedinginan saat kebiruan, anak menungging
• Riwayat kelainan bawaan lain disangkal
• Riwayat penyakit penyerta lain disangkal
PEMERIKSAAN FISIK

• Kes adaran: compos mentis

• HR : 110-115x/menit
• RR : 22x/menit
• SpO2 : 70-80% udara bebas
• Kepala : bibir sianotik (+)
• Thorax : VBS kanan=kiri, Rh -/-, Wh -/-, murmur
(+)
• Abd : Kesan Normal
• Ekstremitas : clubbing finger (+), sianosis (+)
Lab
Hb Ht L Tr PT INR APTT
15,9 47,3 6320 505.000 14,0 1,0 27,1

Ur Cr
28,4 0,23

Foto Toraks
Tidak terdapat bronkopneumonia
Tidak terdapat kardiomegali
Echocardiography 7/11/19
• Dextrocardia
• Situs Inversus
• Semua vena pulmonalis bermuara di atrium kiri
• Hypoplastic confluence PA
• Kontraksi LV baik (eyeballing)
• VSD perimembran 17-18mm
• R-L shunt overriding aorta ± 50%
• Arcus aorta ke kiri, tidak terdapat coartasio aorta, tidak ada MAPCAs
• Pulmonal Stenosis infundibular valvular PG 70 mmHg

• Kesan  TOF with confluence PA, Left AO arch, No visible MAPCAs

 SVC PV SpO2 70-
80%

RA
IVC dilatasi LA

RV
dilatasi LV

PA STENOSIS
Aorta
Overriding
PVR SVR
TETRALOGY OF FALLOT
 MANAJEMEN ANESTESI

• Goal :
Mencegah R to L Shunt
 Menghindari PVR naik dan SVR turun
FAKTOR-FAKTOR YANG MEMPENGARUHI PVR

Gregory GA, Andropoulos DB. Gregory’s Pediatric Anesthesia. 2012.

FAKTOR-FAKTOR YANG MEMPENGARUHI SVR

Increase SVR Decrease SVR

Alpha agonist Inhalation agents

B-blocker Vasodilator

Cuff in lower extremities Alpha antagonist

Raise in intrabdominal pressure Ca chanel blocker

Aortic compression Nitropusside, nitroglicerine

Prostaglandine (E1, E2)

Jacob R, Understanding Paediatric Anesthesia. 2008

PREOPERATIF

• Informed Consent
• I.V line
• Persiapan ephedrine dan vasokonstriktor
INDUKSI

• Preoksigenasi 3-5 menit

• Midazolam 0,1 mg/kg i.v (2mg)
• Ketamin 1-2 mg/kg i.v (12,6-25mcg)
• Intubasi ETT No. 5
• Post intubasi pasien dispontankan
 Maintenance
Sevoflurane 2-3 vol % O2 : udara 50% : 50%

Post Operatif
Analgetik : Paracetamol 4 x 250mg
Hypercyanotic Spell (“Tet Spell”)

• The etiology of spells is not completely understood, but infundibular

spasm or constriction plays a role. Crying, defecation, feeding, fever,
and awakening all can be precipitating events
• Paroxysmal hyperpnea is the initial finding. There is an increase in
rate and depth of respiration, leading to increasing cyanosis and
potential syncope, convulsions, or death. During a spell, the infant
will appear pale and limp secondary to poor cardiac output

Yao 6th ed
 Hypercyanotic Spell (“Tet Spell”)
Agents Effects
High FiO2 Spesific pulmonary vasodilator,
↓PVR, reduce hypoxic pulmonary
vasoconstriction
Hydration (15-30cc/kg) Open the RV outflow tract
Morphine (0.05-0.1 mg/kg) Sedation, ↓PVR
Phenylephrine (5-10 mcg/kg), ↑SVR
NE (0.05-0.5 mcg/kg/mnt)
Esmolol (50 mcg/kg iv) titrate, ↓HR, ↓ infundibular spasm
propanolol (0.1 mg/kg iv) slow
Compress the abdomen or to directly compress the aorta , ↑
knee-chest position SVR.

If all these measures fail and the patient continues to deteriorate, the chest may
have to be opened quickly, and the aorta may need to be compressed to reverse
shunting.
Bell C, Kain ZN, The Pediatric Anesthesia Handbook. 1997
 Agent Contraindicated/Not Useful
in Spell
Agents Effects
Atropine ↑HR  ↓PBF
N2O ↓FiO2, may ↑PVR
Iso/Enflurane ↓SVR, ↑HR
Epinephrine May cause spasm of RV outflow
tract, ↑HR
Dopamine May cause spasm of RV outflow
tract, ↑HR
Halothane (in Negative inotropy
extreme RV
failure)

Bell C, Kain ZN, The Pediatric Anesthesia Handbook. 1997

THANK YOU
What are cyanotic heart defects?

• Cyanotic heart defects are defects that allow oxygen-rich blood and oxygen-poor
blood to mix.

• In cyanotic heart defects, less oxygen-rich blood reaches the tissues of the body. This
results in the development of a bluish tint-cyanosis-to the skin, lips, and nail beds.

• Cyanotic heart defects include:

• Tetralogy of Fallot.
• Transposition of the great vessels.
• Pulmonary atresia.
• Total anomalous pulmonary venous return.
• Truncus arteriosus.
• Hypoplastic left heart syndrome.
• Tricuspid valve abnormalities.
What are acyanotic heart defects?

• Congenital heart defects that don't normally interfere with the amount of oxygen or
blood that reaches the tissues of the body are called acyanotic heart defects. A bluish
tint of the skin isn't common in babies with acyanotic heart defects, although it may
occur. If a bluish tint occurs, it often is during activities when the baby needs more
oxygen, such as when crying and feeding.

• Acyanotic congenital heart defects include:

• Ventricular septal defect (VSD).

• Atrial septal defect (ASD).
• Atrioventricular septal defect.
• Patent ductus arteriosus (PDA).
• Pulmonary valve stenosis.
• Aortic valve stenosis.
• Coarctation of the aorta.
• The term pink Tet refers to any noncyanotic patient with tetralogy of
Fallot with pulmonary stenosis (TOF/PS) or TOF/pulmonary artery. In
these patients QP : QS is sufficiently high (QP : QS generally greater
than 0.8:1 in the presence of a normal mixed venous saturation and
pulmonary vein saturation) to maintain a deoxyhemoglobin
concentration less than 5 g per dL (arterial oxygen saturation [SaO2]
generally >80%). The designation of “pink Tet” would apply to TOF/PS
patients with minimal valvular and subvalvular PS and to all patients
with TOF/pulmonary atresia with pulmonary blood flow supplied
from a large patent ductus arteriosus (PDA) and/or major
aortopulmonary collateral arteries (MAPCAs).

Yao
• β-adrenergic antagonists (Esmolol, Atenolol, Metoprolol,
• Bisoprolol)
• This drug class:
•  Reduces myocardial oxygen consumption
•  Improves coronary blood flow
•  Prolonged diastolic perfusion period
•  Improved collateral flow
•  Increased flow to ischemic areas
•  Improves supply/demand ratio
•  Stabi lizes cellular membranes
•  Improves oxygen dissociation from hemoglobin
•  Inhibits platelet aggregation
Eisenmenger syndrome

• Eisenmenger syndrome refers to any untreated congenital cardiac

defect with intracardiac communication that leads to pulmonary
hypertension, reversal of flow, and cyanosis. The previous left-to-
right shunt is converted into a right-to-left shunt secondary to
elevated pulmonary artery pressures and associated pulmonary
vascular disease.
There are four basic types of VSD:

• Muscular VSD. The most common type of VSD, it is an opening in the

muscular portion of the lower section of the ventricular septum. A
large number of these close spontaneously and do not require
surgery.
• Perimembranous VSD. This opening is in an area of the upper section
of the ventricular septum called the membranous septum, located
near the valves. This type of VSD is the one that is most commonly
treated by surgery because most do not close on their own.
• Atrioventricular canal type VSD. This VSD is associated with
atrioventricular canal defect. The VSD is located underneath the
tricuspid and mitral valves.
• Conal septal VSD. The rarest of VSDs, it occurs in the ventricular
septum just below the pulmonary valve.
Tipe VSD
There are four different types of
pulmonary stenosis:

• Valvar pulmonary stenosis. The valve leaflets are thickened and/or

narrowed
• Supravalvar pulmonary stenosis. The pulmonary artery just above the
pulmonary valve is narrowed
• Subvalvar (infundibular) pulmonary stenosis. The muscle under the
valve area is thickened, narrowing the outflow tract from the right
ventricle
• Branch peripheral pulmonic stenosis. The right or left pulmonary
artery is narrowed, or both may be narrowed
Tipe pulmonary stenosis
Tipe
murmur
TOF
Echocardiography 16/1/19
• Semua vena pulmonalis bermuara di atrium kiri
• RV dan RA dilatasi
• Kontraksi RV baik
• LA LV dimensi normal, L-R shunt PDA (-) EF 61 %
• VSD perimembran besar
• Overriding aorta ± 50%
• Arcus aorta ke kiri, tidak terdapat coartasio aorta
• Pulmonal Stenosis infundibular valvular PG 70 mmHg
• Patent L-BT shunt (kecil ?)
• PA confluens RPA 8 mm LPA 6 mm (tak jelas)

• Kesan  TOF, paten Left BT shunt (partially blocked?)

• coartasio aorta  penyempitan aorta
• PA confluens  secara anatomis pulmonarty arteri normal. Dari
ventrikel kanan jadi main pulmonary artery lalu bercabang jadi 2 right
and left
• overriding aorta is a congenital heart defect where the aorta is
positioned directly over a ventricular septal defect (VSD), instead of
over the left ventricle. The result is that the aorta receives some
blood from the right ventricle, causing mixing of oxygenated and
deoxygenated blood, and thereby reducing the amount of oxygen
delivered to the tissues.
BT shunt

• The Blalock–Thomas–Taussig shunt (commonly called the Blalock–

Taussig shunt) is a surgical procedure used to increase pulmonary
blood flow for palliation in duct dependent cyanotic heart defects like
pulmonary atresia, which are common causes of blue baby syndrome
. In modern surgery, this procedure is temporarily used to direct
blood flow to the lungs and relieve cyanosis while the infant is
waiting for corrective or definitive surgery.
• One branch of the subclavian artery or carotid artery is separated
and connected with the pulmonary artery. The first area of
application was tetralogy of Fallot.
• The Blalock–Thomas-Taussig shunt is used in the first step of the
three stage palliation (The Norwood Procedure).
BT shunt

• Blalock-Taussig (BT) shunts helps resolve symptoms of the defect until

the child is older or the defect can be repaired.
• The shunt creates a detour that allows enough blood to pass through
the lungs and pick up more oxygen.
• The shunt is inserted in a closed-heart procedure.
• Shunts are typically used for four to five months until definitive repair
is required.
BT shunt

• The BT shunt is a palliative procedure. It does not correct the defect,

but it helps to resolve symptoms until the child is older and/or the
defect itself can be repaired. It is also called the blue baby operation.
• A shunt is a small tube made out of synthetic material called Gore-
Tex. The BT shunt is about 3 to 3.5 mm in diameter. It attaches a
section of the aorta to the pulmonary artery, creating a sort of
detour. This allows enough blood to pass through the lungs and pick
up more oxygen. The shunt relieves any cyanosis or blueness the
child might have been experiencing. This is a closed-heart procedure.
• The BT shunt mimics the role of the ductus arteriosus. It is often put
in place after the ductus closes naturally. Shunts are usually used for
four to five months, until the child outgrows them and a second
operation or definitive repair is needed.
BT shunt
Simple Shunt

Gregory GA, Andropoulos DB. Gregory’s Pediatric Anesthesia. 2012.

• Shunting is the process whereby venous return into one circulatory
system is recirculated through the arterial outflow of the
same circulatory system.
• Treatment of a “Tet spell” includes the following:
 Administration of 100% oxygen.
 Compression of the femora l arteries or plac ing the patient in a knee-chest position transiently increases SVR and
reduces the R-L shunt.
 Administration of morphine sulfate (0.05 to 0.1 mg/kg), which sedates the patient and may have a depressant effect on
resp iratory drive and hyperpnea.
 Admin istration of 15 to 30 mL per kg of a crysta lloid so lution. Enhancing pre load will increase heart s ize, wh ich may
increase the diameter of the right ventricle outflow tract (RVOT).
 Administration of sodium bicarbonate to treat the severe metabolic acidosis that can be seen during a spell. Correction
of the metabolic acidosis will help norma lize SVR and reduce hyperpnea. Bicarbonate administration (1 to 2 mEq/kg) in
the absence of a b lood gas determ ination is warranted during a spell.
 Phenylephrine (dose 5 to 10 µg/kg IV or 2 to 5 µg/kg/minute as an infusion) can be used to increase SVR and reduce
R-L shunting. In the presence of severe RV outflow obstruction, phenylephrine-induced increases of pulmonary
vascular resistance (PVR) will have little or no effect in increasing RV outflow resistance. It is important to point out
that treatment with α-adrenergic agents to increase SVR does nothing to treat the underlying cause of the spell
although the decrease in unstressed venous volume induced by these agents may augment preload.
 β-Adrenerg ic agon ists are abso lutely contra ind icated. By increasing contractility, they will cause further narrowing of
the stenotic infundibulum.
 Admin istration of propranolo l (0.1 mg/kg) or esmo lo l (0.5 mg/kg followed by an infusion of 50 to 300 µg/kg/minute)
may
reduce infund ibular spasm by depressing contractility. In add ition, s lowing of heart rate (HR)may allow for improved
diastolic filling (increased pre load), increased heart s ize, and an increase in the d iameter of the right ventric le outflow
tract (RVOT).
 Manua l compression of the abdominal aorta will increase SVR; this maneuver is particu larly effective for the
anesthetized patient. After the chest is open, the surgeon can manually
compress the ascending aorta to increase impedance to ejection through the LV. This can be effective in terminat ing a
cyanotic episode.
 extracorporeal membrane oxygenation (ECMO) resusc itation is another alternative in refractory episodes when
immediate operative intervention is not possib le.