Radiologi

Penyakit Jantung Kongenital

Risa Muthmainah - I4061171014
Pembimbing : dr. Indria Fajrianita, Sp.Rad
Normal Chest X-ray(PAview)
• Index
• RA -right atrium
• RDPA - right descending pulmonary
artery
• RPA - right main pulmonary artery
• SVC- superior vena cava
• AA - aortic arch
• DA - proximal descending thoracic aorta
• LPA - left pulmonary artery
• RV - right ventricle

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Normal chest X-ray(LateralView)
• Index:
• RV- right ventricle
• RSS - retrosternal clear space
• AA - ascending aorta
• LPA - left pulmonary artery
• RPA - right pulmonary artery en face
• IVC - inferior vena cava
• LA - left atrium
• LV - left ventricle

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TOF
• Tetralogi Fallot merupakan penyakit jantung bawaan sianotik yang
banyak ditemukan yakni berkisar 7-10% dari seluruh penyakit jantung
bawaan
• Tetralogi Fallot merupakan kelainan yang terdiri dari kombinasi 4
komponen : Defek septum ventrikel, over-riding aorta, stenosis
pulmonal, serta hipertensi ventrikel kanan
• Pada Tetralogi Fallot yang ringan pada waktu istirahat maupun
melakukan aktivitas fisik tidak tampak adanya sianosis. Pada TF yang
moderat hingga berat sianosis akan tampak bahkan pada saat anak
istirahat. Seorang anak yang mengidap TF akan mudah merasa lelah,
sesak dan hiperpnu karena hipoksia
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Patofisiologi
● Komponen yang paling penting, yang
menentukan derajat beratnya penyakit, adalah
stenosis pulmonal, yang bervariasi dari sangat
ringan sampai sangat
● Tekanan yang meningkat akibat stenosis
pulmonal menyebabkan darah yang
terdeoksigenasi keluar dari ventrikel kanan
menuju ventrikel kiri melalui defek septum
ventrikel dan ke sirkulasi sistemik melalui aorta 
hipoksemia sistemik dan sianosis.
● Bila stenosis pulmonal semakin berat, maka
semakin banyak darah dari ventrikel kanan
menuju ke aorta.
● Semakin bertambahnya usia, maka infundibulum
akan semakin hipertrofik, sehingga pasien akan
semakin sianotik. 5
TOF

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Tetralogy of Fallot
BOOT SHAPED SIGN

▫ Small heart.
▫ Boot shaped heart (due
to ventricular
hypertrophy). It means
apex is lifted up.
▫ Concavity in the region
of pulmonary artery
▫ Right sided aortic arch.
▫ Oligaemic lung fields.
▫ Hilar vessels are few
lung vessels also few.
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 Contrast-enhanced CT image

6-day-old girl with tetralogy of Fallot, absence of pulmonary valve, and bilateral superior vena cavae. Coronal oblique
reformatted contrast-enhanced CT image shows right (black arrow) and left (white arrow) superior vena cavae that are
connected by bridging vein (arrowheads). (B) and posterior oblique volume-rendered images show massive central
pulmonary artery dilatation (asterisks) with peripheral pulmonary arteries closer to normal caliber. 11
AorticCoarctation
● Koartasio aorta adalah suatu penyakit
jantung bawaan berupa penyempitan
pada arkus aorta distal atau pangkal aorta
desendens torakalis
● Koartasio aorta merupakan 2-7% dari
penyakit jantung bawaan dengan insidensi
0,3-0,4 tiap
● Koartasio aorta menyebabkan kenaikan
afterload pada ventrikel kiri,
menyebabkan tekanan dinding yang
meningkat, hipertrofi ventrikel kiri
kompensata, disfungsi ventrikel kiri dan
pembentukan arteri kolateral
Classic radiologic signs : Figure-of-three sign (formed by prestenotic and
poststenotic dilatation of the descending aorta.) , Reverse figure-of-three sign, Rib
notching on CXR pathognomonic (related to collateral blood flow through the
intercostal arteries
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Ct- angiografi

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EbsteinAnomaly
• 0.5%–0.7%of casesof congenital heart disease.
• Characterized by the downward displacement of
the septal leaflets and posterior leaflets of the
tricuspid valve into the inflow portion of the
right ventricle.
• Results in the formation of a common right
ventriculoatrial chamber and causes tricuspid
regurgitation.
• Insufficiency of the tricuspid valve leads to
dilatation of the right ventricular outflow tract
and all proximal right heartstructures,
• Most consistent imaging feature is right atrial
enlargement
Findings on chest radiographs largely depend on the severity of the abnormality and the degree to which the
tricuspid valve is displaced downwards.. There is often severe right-sided cardiomegaly due to an elongated and
enlarged right atrium which may result in an elevated apex. Classically, the heart is described as having a "box
shape" on a frontal chest radiograph.
Contrast-enhanced chest CT axial (Fig. 2A-2F); images demonstrate cardiomegaly with marked
dilatation of both the right atrium and right ventricle. Note the compensatory rotation and
displacement of the left heart. There is apical displacement of the septal and posterolateral 18
leaflets of the tricuspid valve into the right ventricle. A right pleural effusion is also present.
Thanks!
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