Tetralogy of Fallot

An abnormal anterior cephalad

displacement of the infundibular
portion of the interventricular septum
 Tetralogy of Fallot (TOF)
Defek jantung kongenital sianotik terbanyak
yang mana kelainan utamanya adalah deviasi
anterior dari septum infundibulum (septum otot
yang memisahkan aliran darah ke pb. darah
aorta dan pulmonal).
Insidensi 5:10.000 kelahiran
Berhubungan dengan mikrodelesi kromosom
22 (22q11)
 Tetralogy of Fallot (TOF)
Ditandai dengan adanya
1) VSD caused by anterior malalignment of the
interventricular septum
2) subvalvular pulmonic stenosis because of
obstruction from the displaced infundibular
septum (often with valvular pulmonic stenosis)
3) An overriding aorta that receives blood from
both ventricles
4) right ventricular hypertrophy owing to the high-
pressure load placed on the RV by the
pulmonic stenosis.
 Pathophysiology
Resistensi meningkat karena
stenosis subvalvular pulmonik

Darah deoksigenasi di RV,

melewati VSD ke LV

Darah akan dipompa ke

sirkulasi sistemik

Hipoksemia, sianosis
Pathophysiology
 Clinical findings
Symptoms Pysical examination
Dyspnea on exertion Mild cyanosis  lips,
Spells  exertion, mocous membranes and
feeding, crying digits
Spells (irritability, Clubbing fingers and
cyanosis, toes
hyperventilation,
syncope, convulsions) RVH  palpable
To alleviate the Systolic ejection murmur
symptoms  squatting at upper left sternal
down border
7
 Anak dengan
Tetralogy of fallot Jongkok
Penurunan resistensi pembuluh
darah sistemik (Squatting)
Meningkatkan pirau kanan ke Mencegah darah mengalir ke
kiri paru

SIANOSIS

Jongkok
(squatting)

Berjongkok pada pangkal paha

dengan lutut menghadap ke atas
menekan dada

Menahan darah vena yang tidak

Mencegah aliran kembali ke Menaikkan tekanan vaskular sistemik
tersaturasi O2 pd tungkai bawah jantung dengan menghambat a. femolaris

Meningkatkan aliran darah ke

Menurunkan pirau kanan ke kiri
paru
 Diagnostic studies
Chest radiographs ECG
Prominence of RV RVH
Decreased size of the RAD
main pulmonary artery
segment  boot shaped
heart
Pulmonary vascular
marking ↓
 Diagnostic studies
Echo
Details the RV outflow
tract anatomy
Malaligned VSD
RVH
 Treatment
Palliative therapy
Creating anatomic communications between the
aorta (or one of its major branches) to the
pulmonary artery
Establishing a left-to-right shunt to ↑ pulmonary
blood flow.
Complete surgical correction
Closure of the VSD and enlargement of the
subpulmonary infundibulum with the use of a
pericardial patch.
Elective repair (6 to 12 months of age)  to decrease
the likelihood of future complications.
Antibiotic prophylaxis to prevent endocarditis is
required in some patients
Each great vessels inappropriately arises from the
opposite ventricles; that is, the aorta originates from
RV and the pulmonary artery originates from LV

Transposition of the great arteries

Pathophysiology
• TGA adalah penyebab sianosis paling
sering pada periode neonatal
• Penyebab yang jelas TGA belum diketahui
• Masalah dasar: kegagalan septum
aorticopulmonal menjadi spiral dalam
bentuk normal selama perkembangan
fetus atau perkembangan abnormal dan
absorpsi subpolmonik dan infundibuli
subaortik
 Symptoms & pysical
examinations
Apear blue  intensity of cyanosis
degree of intermixing between the
parallel circuit
Generalized cyanosis
CHF symptoms
Loud and single S2
No heart murmur
 Diagnostic studies
Chest radiographs ECG
Usually normal RVH
Base of heart may be
narrow  anterior-
posterior orientation of
aorta and pulmonary
artery (egg shaped
cardiac)
 Diagnostic studies
Echo
Abnormal orientation of
the great vessels
 Treatment
Medical emergency
Initial treatment
Prostaglandin
infusion→maintenance of
the ductus arteriosus
Creating of an interatrial
communication using a
baloon catheter (Rashkind
Procedure)
Corrective surgery → arterial
switch (Jatene Procedure) →
transection of the great
vessels above the semilunar
valves and origin of the
coronary arteries