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TETRALOGY OF FALLOT +
PULPITIS REVERSIBLE
IDENTITAS

An. Laki laki,

Usia : 3 tahun
Berat badamn : 18 kg
Diagnosis : Tetralogy Of Falot + Pulpitis reversible
Tindakan : Mouth Preparation + ekstraksi
Anamnesis

• Riwayat Kebiruan sejak usia 1,5 tahun

• Pasien menjadi kebiruan jika menangis kencang dan setelah aktivitas
berat. kebiruan menghilang setelah pasien tenang
• Riwayat operasi sebelumya dan penyakit bawaaan di tempat lain
tidak ada
• Riwayat sedang menjalani pengobatan tertentu tidak ada
• Riwayat Alergi dan penyakit penyerta lain tidak ada
PEMERIKSAAN FISIK

• Kes adaran: compos mentis

• HR : 118x/menit
• RR : 22 x/menit
• SpO2 : 86-90 % 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 50,5 6210 276.000 4,8 0,93 28,2

SGOT SGPT Na K
55 19 137 4
Rontgen Thorax

Expertise :

Kardiomegali

Tidak tampak
bronkopneumonia
Echocardiography
• Situs Solitus
• Semua vena pulmonalis bermuara di atrium kiri, flow tidak terlalu deras
• ASD (-), PDA (-)
• IVS paradox (+), LV D shaped (+)
• Good LV function LVEF 82%
• RV function TAPSE 1,9 cm
• VSD subaortic dengan overriding aorta ± 50% R-L shunt
• deviasi septum infundibular ke anterior
• RVOT sempit, RV-PA gradient 89 mmhg
• confluent PA, RPA=LPA= 6 mm
• left aortic Arc, Coarc (-)

• Kesan  TOF
ASSESMENT

• Diagnosis : Tetralogy Of Falot + pulpitis reversible

• ASA III

• PLANING :
• Informed Consent
• I.V line
• Persiapan vasokonstriktor
• Premedikasi : Midazolam 0,05-0,1mg/kg  0,9 mg IV
• Intra Op:
• Induksi:
• Preoksigenasi 3-5 menit
• Midazolam 0,1-0,4 mg/kg 3,6 mg
• Ketamin 2 mg/kg36 mg
• Atracurium 0,5 mg/kg 9 mg
• Maintenance:
• Sevoflurane 1-2,5vol%, O2 : Air: 50:50

• Post Operasi
• Deep Ekstubasi
• Paracetamol 4 x 150 mg IV
 SVC PV SpO2 54 – 60
%

RA
IVC dilatasi LA

RV
dilatasi LV

PA STENOSIS
Aorta
Overriding
PVR SVR
MANAJEMEN ANESTESI

• Goal :
Mencegah semakin beratnya R to L Shunt
 Menghindari PVR naik dan SVR turun
Simple Shunt

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

TOF
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

Hypercyanotic Spell (“Tet Spell”)

• Terjadi karena spasme infundibular, PH

rendah, low PaO2
• Dapat terjadi saat pasien sadar : akibat
sianosis akut, hiperventilasi, saat pasien
menangis, stress, atau saat defekasi
• Dapat terjadi saat tindakan anestesi: spasme
infundibular
 Hypercyanotic Spell (“Tet Spell”)
Agents
High FiO2
Hydration (15-30cc/kg)
Morphine (0.05-0.1 mg/kg)
Phenylephrine (5-10 mcg/kg), NE
(0.05-0.5 mcg/kg/mnt)
Esmolol (50 mcg/kg iv) titrate,
propanolol (0.1 mg/kg iv) slow
Compress the abdomen or knee-chest
position
Effects
Spesific pulmonary vasodilator, ↓PVR, reduce
hypoxic pulmonary vasoconstriction
Open the RV outflow tract
Sedation, ↓PVR
↑SVR
↓HR, ↓ infundibular spasm
to directly compress the aorta , ↑ 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
SpellAgents 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 extreme Negative inotropy
RV failure)

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

HATUR NUHUN
Treatment of a “Tet spell” includes the following:
1. Administration of 100% oxygen.
2. Compression of the femora l arteries or placing the patient
in a knee-chest position transiently increases SVR and
reduces the R-L shunt.
3. Administration of morphine sulfate (0.05 to 0.1 mg/kg),
which sedates the patient and may have a depressant
effect on respiratory drive and hyperpnea.
4. Administration of 15 to 30 mL per kg of a crysta lloid so
lution. Enhancing pre load will increase heart size, which
may increase the diameter of the right ventricle outflow
tract (RVOT).
5. 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.
6. 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.
7. β-Adrenerg ic agonists are absolutely contra ind icated. By
increasing contractility, they will cause further narrowing of the
stenotic infundibulum.
8. Administration of propranolo l (0.1 mg/kg) or esmolol (0.5 mg/kg
followed by an infusion of 50 to 300 µg/kg/minute) may reduce
infund ibular spasm by depressing contractility.
9. Manual compression of the abdominal aorta will increase SVR
10. extracorporeal membrane oxygenation (ECMO) resuscitation is
another alternative in refractory episodes when immediate
operative intervention is not possible.