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DEFINISI

Merupakan

kelainan
struktural/fungsional jantung, yang
ada saat lahir meskipun gejalanya
baru timbul kemudian
Insiden terjadi 4 dari 1000
kelahiran neonatus hidup di
indonesia

Congenital
Heart
Diseases
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POKOK MASALAH

LATAR BELAKANG

Penyebab sulit dikendalikan terutama


yang bawaan sehingga usulanusulan -usulan
pencegahan masih minimal
Derajat kelainan anatomi/fungsi
jantung sangat bervariasi, sering tidak
jelas atau mirip gejala penyakit lain.
Kesibukan ortu/ketidaktahuan
pengasuh/guru.
Proses penyakit berjalan terus
terus tiba
pada stadium lanjut oleh gejala yang
akut atau mengejutkan

Anak/bayi bukan miniatur orang


dewasa
Keadaan kedaruratan cenderung
meningkat
Setiap soal dapat dijumpai
Perlu pengenalan diri
Perlu penatalaksanaan adekuat
Perlu landasan teori yang memadai

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Sering

baru diketahui pada masa


sekolah sudah dgn kegawatan jantung
Tata laksana
laksana::
Stadium Awal
Awal:: Hasil memuaskan
memuaskan,,
walaupun sering perlu biaya yang sangat
besar,, sarana lengkap seperti dokter ahli
besar
yang kompak
kompak..
Stadium Akhir
Akhir:: Hasil sering tidak
memuaskan,, biaya lebih banyak
memuaskan
banyak,, bahkan
sudah inoperable.

Penyakit Jantung Bawaan


Faktor
Lingkungan
Genetik

Penyakit ibu : Rubella, DM, HT


Pajanan:: Sinar X, bahan keratogenik
Pajanan
Kebiasaan
: Perokok, alkohol
Usia
: kurang dari 18 tahun dan lebih
32 tahun
Jumlah anak : lebih 3

Kegawatan

jantung sering muncul pada


usia sekolah
sekolah.. Gejala dini sebelum
kegawatan harus sudah dikenal
dikenal..
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Gejala dini tergantung beberapa faktor:


Derajat cacat anatomis jantung
Macamnya
Usia, makin dini makin sullit deteksinya.
Perhatian dan kepedullian ortu

ETIOLOGI

FAKTOR LINGKUNGAN
Radiasi, Polusi, Kimiawi, Virus, ObatObatobatan
2. FAKTOR IBU
Usia ibu > 40 thn, IDDM, alkoholik,
Pengguna obatobat-obatan
3. HEREDITER
Orang tua KJB kemungkinan besar
anak dgn KJB, sindrom down, sindrom
turner.
1.

Pembuluh

Mengapa harus kenal gejala dini?


Periksakan cepat
Pertolongan adekuat
Cegah penyulit lebih lanjut
Optimalisasi proses TUMBUH KEMBANG

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darah besar :

1. Arteri :
coronaria , bronkiosefalik ,
subclavikula , pulmonal

2. Vena :
cava inferior , cava superior ,
pulmonal

Lapisan

jaringan :
1. Miokardium
2. Endokardium
3. Perikardium :
terdiri dari serosa viseral & serosa
parietal yg terdapat ruangan
diantaranya , mempunyai sedikit
cairan pericaldial
memungkinkan adanya gesekan otot
jantung tampa gesekan

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Pengendalian

frekwensi denyut jantung


1. Diatur oleh saraf otonom , terdiri dr
simpatis
meningkatkan
frekwensi & para simpatis
menurunkan frekwensi denyut
jantung

Aorta
Pulmonary artery

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Left atrium

Aortic valve
Pulmonary valve
Mitral valve
Right atrium

2. Dipengaruhi hormon katekolamin


Tricuspid valve

Right ventricle

Left ventricle
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Volume

15

sekuncup dikendalikan :
1. Kontraktilitas : kemampuan otot
jantung sbg pompa .
Dipengaruhi Oleh : katekolamin ,
neurunal simpatis
simpatis,, obat ttt digitalis
meningkatkan .
hipoksemia & asidosis
menurunkan
2. Preload : vol darah yg kembali ke
jantung
3. Afterload :tahan ketika ventrikel
memompa darah saat diejeksikan

Fungsi

sistem konduksi
Nodus SA : pemicu jantung , Implus
menyebar dr nosus SA sepanjang
atrium menyebabkan depolariasi

implus dr nodus AV menyalurkan ke


ventrikel

Implus menyebar ke berkas AV &


serat purkinje yg menyebabkan
depolarisasi ventrikel yg simultan

PERKEMBANGAN SISTEM CARDIOVASKULER


Bayi

:
- TD : 80/40

Nadi : 120 130

- BBL : denyut jantung 120 160


kemudian melambat 100 120
- Ukuran jantung meningkat dua kali pd
usia 1 tahun
- Terletak lebih tinggi di dlm toraks

Anak usia sekolah


- TD : 94 112/56 60 Nadi : 70 80
- Berat jantung 10 kali berat lahir saat
pubertas
- Ukuran pd usia sekolah scr
proposrsional lebih kecil dibandingkan
pd setiap fase
alasan
mengapa usia sekolah lebih cepat
lelah
- letak jantung lebih vertikal pd R.toraks

Todler

pra sekolah :
- TD : 80 100/64 nadi : 80 105

- Berat jantung meningkat empat kali pd


usia 5 thn berkaitan dgn penurunan
denyut jantung & peningkatan TD
- Sinus aritmia tampak jelas slm pra
sekolah
- Murmur fisiologis mungkin terdengar

Remaja

- TD : 100 120/50 70 Nadi :60 68


- Pertumbuhan terus berlanjut ,
pembuluh darah memanjang &
melebar
- Pertumbhan jantung relatif lebih
besar
dr PD

KLASIFIKASI

KLASIFIKASI

Asianosis

Sianosis

Peningkatan aliran
darah pulmonal

Obstruksi aliran darah


Dari ventrikel

Penurunan aliran
darah pulmonal

Aliran darah teroksigen


& anoksigen tercampur

ASD, VSD, PDA


Atrioventrikular

COA, SA, SP

TOF, Atresia trikuspid

TGA, TAPVR, HLHS


Trunchus arteriosus

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Patent Ductus Arteriosus (PDA)


Kegagalan penutupan ductus
arteriosus (arteri yang
menghubungkan aorta dan
pulmonal)

Murmur, TD , turbulensi darah dari


aorta ke arteri pulmonalis, obtruksi
pulmonal, penebalan atrium &
ventrikel kiri
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Coarctation of Aorta (COA)


Definition
A congenital narrowing of upper descending
thoracic aorta adjacent to the site of attachment of
ductus arteriosus
Penyempitan aorta yang menyebabkan obstruksi
aliran darah pada sisi kiri jantung
Nadi femoral lemah & hilang
hilang,, ekstremitas
bawah dingin & pucat
pucat,, hipotensi
hipotensi,, murmur,
distress nafas
nafas,, pusing
pusing,, sakit kepala
kepala,, CRT >2
dtk

Coarctation of Aorta
Developmental

2. Presence of ectopic ductal tissue in the aorta

Coarctation of Aorta

Aortic Arch Hypoplasia


Definition

Hypoplastic arch has higher ratio of elastin lamellae to


vessel diameter & increase in collagen and decrease in

factor

1. Underdevelopment or hypoplasia of aortic


arch or isthmus
Definition of hypoplasia
* Proximal arch : 60% of ascending aorta
* Distal arch
: 50% of ascending aorta
* Isthmus
: 40% of ascending aorta

Morphology
1.

Localized stenosis
* Usually intimal hypertrophy ( intimal veil ) extends
the shelf circumferentially and further narrows the
lumen (Rodbard
(Rodbard))

alpha--actin
alpha
actin--positive cell that may hinder the ability of
arch to distend.
1. 50% reduction of terminal end of ascending aorta, sometimes,
because of small ascending aorta in coarctation
coarctation,, descending
thoracic aorta is compared.
2. Transverse arch diameter less than body weight in Kg plus 1
3. ZZ-value less than 2 or more

2. Tubular hypoplasia
*
*
*
*

Severe with lesser narrowing


Proximal aortic & arterial wall
Distal aortic arch narrowing
Fetal flow pattern (Rudolph)

Coarctation of Aorta
Evolution

Coarctation of Aorta

Pathophysiology

Narrowed

aorta produces increased left


ventricular afterload and wall stress, left
ventricular hypertrophy, and congestive
heart failure.
Systemic perfusion is dependent on the
ductal flow and collateralization in severe
coarctation

Coarctation of Aorta
Associated

CoA

Localized

pathology

1. Collateral circulation
* Inflow

: primary from branches of both subclavian arteries


. internal mammary artery . vertebral artery
. costocervical trunk
. thyrocervical trunk
* Outflow : into descending aorta, two pairs of intercostal arteries

2. Aneurysm formation of intercostal arteries


* 3rd, & 4th rib notching

* rare before 10 years of age

3. Coronary artery dilatation and tortuosity


* due to LVH

4. Aortic valve
* bicuspid (27(27-45%)

* stenosis ( 6 - 7%)

5. Intracranial aneurysm
* berry type intracranial aneurysm in some patients

6. Associated cardiac anomaly


* 85% of neonates presenting COA

Coarctation of Aorta

CoA
Tubular Hypoplasia

Natural

Distal arch

PDA

history

1. Incidence
* 55-8% of CHD (5 per 10000 live births)
* Isolated CoA (82% of total CoA) ; male:female = 2:1
CoA + VSD 11%, COA + other cardiac anomalies 7%
* Complex CoA ; no sex difference
2. Survival of pure CoA
* 15% : CHF in neonate or infancy
* 85% : survive late childhood without operation
* 65% : survive 3rd decade of life (2% at 60 years)
3. Bacterial endocarditis : common in 1st 5 decades
4. Aortic rupture
: 2~3rd decade
5. Intracranial lesion
: subarachnoid hemorrhage(cong. Berry
aneurysm)

Collaterals
in CoA

Coarctation of Aorta

Clinical features & diagnosis


1. Infancy
1) Closure of ductus (7(7 -10 days) produces severe obstruction
2) Ductus arteriosus remains patent - differential cyanosis
3) Associated intracardiac defect - more severe, early onset
4) Degree of collateral circulation
2. Childhood
* Asymptomatic without significant associated lesion
* Hypertension (90%)
* Cardiomegaly (33%)
* Rib notching (15%)
3. Adolescence and adult
* Hypertension ; very common * Valvar heart disease
* Heart failure at 30 years of age
4. Associated syndrome
* Turner syndrome (XO) : 2% * Von Recklinghausens D
* Noonans syndrome or congenital rubella

Coarctation of Aorta
Indications

for operation

1. Reduction of luminal diameter greater


than 50% at any age
2. Upper body hypertension over 150mmHg
in young infant ( not in heart failure )
3. CoA with congestive heart failure
at any age

Pediatric Cardiac Surgery


Neurologic complications
Incidence of 2.3% for overt clinical presentation & up
to 60% when sensitive magnetic resonance imaging is
applied in heart surgery of infants & children.
In control of the arch proximal to the left carotid artery,
during COA surgery, this assumes that collateral blood
flow and completeness of the circle of Willis allows for a
favorable and even distribution of cerebral blood flow.
But patients undergoing coarctation repair, proximal
occlusion of the aortic arch results in transient but
significant impairment in contralateral cerebral oxygen
balance

Coarctation of Aorta
Techniques

of operation

1. Subclavian flap aortoplasty


Neonate, infant and child up to 10 years

2. EndEnd-to
to--end anastomosis
Preferred in any age group
* Extended endend-to
to--end anastomosis
* Radically extended endend-to
to--end anastomosis

3. Patch angioplasty or graft replacement

Blood Supply to Spinal Cord

The most important blood supply to spinal cord comes from


spinal artery, a minor supply is from Adamkiewicz artery

CoA
Exposure

CoA Patch Augmentation

CoA End
End--to
to--End Anastomosis

CoA
LSCA flap

CoA Subclavian Artery Flap

CoA

Extended endend-to
to--end Anastomosis

Coactation of Aorta

CoA + VSD, OneOne-stage Repair

Resection & Extended endend-to


to--end Anastomosis

Coactation of Aorta

CoA + VSD, OneOne-stage Repair

Operative results

Hospital mortality
Causes of early death are
acute and chronic cardiac failure or severe
pulmonary insufficiency

Incremental risk factor for death


1) Older age
2) Hypoplastic left heart class
3) Techniques of operation

Coactation of Aorta

Operative results

Mobidity
1) Paraplegia (0.2 ~ 1.5%)
2) Hypertension and abdominal pain
3) Persistent or recurrent coarctation
- more than 20mmHg
- high incidence in young
4) Upper body hypertension without resting gradient
- increased vascular activity in the forearm
- age at operation is risk factor
5) Late aneurysm formation
- higher in onlay patch technique
6) Valvular disease
7) Congestive heart failure with hypertension
8) Bacterial endocarditis

Coactation of Aorta
Special

features of postoperative care

1. Systemic arterial hypertension


Usually, but infant or young child doesnt
need to be treated.

2. Abdominal pain
Usually mild abdominal discomfort for a few days,
and prominent in 5 - 10%.
Control hypertension, nasogastric decompression,

IV maintain

3. Chylothorax
5%

Coactation of Aorta Repair


Postoperative

Coactation of Aorta Repair


Paraplegia

hypertension

1.
2.
3.
4.
5.
6.
7.

Sealy
Altered baroreceptor response with increased
excretion of epinephrine or norepinephrine
Rocchin
Sympathetic nervous system in early phase,
and reninrenin-angiotensin system in late phase

Duration of spinal cord ischemia


Duration of intercostal artery ischemia
Intraoperative proximal hypotension
Postoperative hypotension
Hyperthermia during operation
Anastomosis with tension
Acidosis in the perioperative periods

Atresia Trikuspidalis
Kondisi dimana katup trikuspid tidak
ada atau ada tapi tidak mengalami
perforasi
Sianosis, takikardi, Clubbing, abses
otak, stroke, endokarditis bakterial

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Tricuspid atresia (TA) is a congenital (present at


birth) heart defect that occurs due to abnormal
development of the fetal heart during the first 8
weeks of pregnancy

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Normaly blood low.

In Tricuspid Atresia, the heart has three valves


rather than four. The tricuspid valve, which
connects the right atrium (collecting chamber)
and right ventricle (pumping chamber) in the
normal heart, is abnormal and does not open.
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Etiology..??
The heart is forming during the first 8 weeks of
fetal development
Some congenital heart defects may have a
genetic link,
link, either occurring due to a defect in
a gene,
gene, a chromosome abnormality,
abnormality, or
environmental exposure,
exposure, causing heart
problems to occur more often in certain
families. Most of the time, this heart defect
occurs sporadically (by chance), with no clear
reason for its development.

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Clinical manifestation
each

child may experience symptoms


differently. Symptoms may include:
blue color of the skin, lips, and nail
beds
rapid breathing
labored breathing
rapid heart rate
cool, clammy skin
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As part of the cardiac catheterization, a procedure


called a balloon atrial septostomy may be performed to
improve mixing oxygenoxygen-rich (red) blood and oxygenoxygenpoor (blue) blood.
A special catheter with a balloon in the tip is used to
create or enlarge an opening in the atrial septum
(wall between the left and right atria).
The catheter is guided through the foramen ovale (a
small opening present in the atrial septum that
closes shortly after birth) and into the left atrium.
The balloon is inflated.
The catheter is quickly pulled back through the hole,
into the right atrium, enlarging the hole, allowing
blood to mix between the atria.
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initial treatment include the following:


A cardiac catheterization procedure can be
used as a diagnostic procedure, as well as
initial treatment procedure for some heart
defects. A cardiac catheterization procedure will
usually be performed to evaluate the defect(s)
and the amount of blood that is mixing.

An intravenous (IV) medication called


prostaglandin E1 is given to keep the ductus
arteriosus from closing.

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Atrio Septal Defect (ASD)


Ggn septum antara atrium yg
mengikuti pirau darah dari atrium
kiri kanan
Murmur, infeksi pernafasan, dyspnea,
atrial disritmia, obstruksi pulmonal,
pembentukan emboli, gagal jantung

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Ventrikel Septal Defect (VSD)


Terdapatnya hubungan antara
ventrikel kiri & kanan darah
mengalir ventrikel kiri kanan
Murmur sistolik, resiko endokarditis
bakteri, overloading ventrikel kanan,
obstruksi paru, gagal jantung

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Stenosis Aorta
Penyempitan pada katup aorta
menyebabkan darah tertahan di ventrikel
kiri, CO menurun, Hipertropi ventrikel kiri
& kongesti vaskular paru
Penurunan CO, Distress pernafasan,
hipotensi, gagal ventrikel kiri, murmur,
takikardi, nafsu makan menurun, nyeri
dada, hipotensi postural, & intoleransi
aktifitas
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Stenosis Pulmonal

Tetralogi of Fallot (TOF)


Empat kondisi spesifik abnormal
jantung yag terjadi secara simultan
yaitu : pembesaran spesifik VSD,
Obstruksi aliran ventrikel kanan,
letak aorta yang menjorok kekanan,
& hipertropi ventrikel

Penyempitan pada katup arteri


pulmonal
pulmonal
tahanan aliran darah
darah
hipertropi ventrikel kanan dan
penurunan aliran darah paru
Sianosis ringan, distress pernafasan,
dyspnea, kelemahan, kardiomegali,
gagal jantung akut
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Sianosis akut, hipoksia, anoksia,


clubbing, & gagal tumbuh
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Transposition of Great
Arteri/Vessel (TGA/TGV)

Lesi Kardiak (TAPVR, TA, TGA, HLHS


Percampuran darah anoksigen & teroksigen
Penurunan Aliran Pulmonal

Aliran darah Pulmonal berlebih

Peningkatan Jumlah darah


Di kanan
Peningkatan aliran darah paru
Membantu aliran sistemik

Penurunan CO

Stenosis serta Pulmonal & COA

Obstruksi aliran darah


Peningkatan tekanan di vertikel dan arteri
Besar penurunan tekanan pada daerah setelah
obtruksi
Tahanan pada vertikel dan penurunan CO
MK Penurunan CO

Stimulasi Baroreseptor
Sisitem Saraf simpatis

Stimulasi serat kolinergik

Obstruksi vena paru, sianosis setelah


menangis, CHF, kardiomegali, suara
jantung S1 keras

Kelainan jantung bawaan

Aliran darah dan jantung kiri


kekanan

Penurunan Restitensi Vaskular Pulmonal

Letak aorta & arteri pulmonalis terbalik


biasanya disertai adanya defect
untuk bertahan hidup seperti
foramen ovale, VSD atau PDA

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Keringat banyak terutama


di kepala MK Gangguan
keseimbangan cairan

Peningkatan nadi dan kekuatan


Kontraksi jantung
Takikardia
MK : Resti gangguan perfusi miokardial
Cemas
Nyeri
Intoleransi aktifitas

Penikatan tonus vaskuler dan vasokontiksi


Peningkatan venous retunn
Peningkatan tahanan vaskuler pulmonal
Peningkatan tahanan vaskuler sitemik
Peningkatan after load
Penurunan aliran darah ke ekstremitas bawah
MK : Gangguan perfusi jaringan
Gangguan pertukaran gas
Pola nafas tidak efektif

Penurunan aliran darah ginjal


MK : Resti gangguan keseimbangan
Cairan dan elektrolit
Produksi renin aldosteron
ADH
Anuria
Retensi Na dan H,O
Edema
Peningkatan volume darah
Peningkatan preload

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Pelebaran vena pulmonal dan sitemik

Pemeriksaan Penunjang
Echocardiografi mengetahui adanya
atrium/ventrikel kanan & kiri serta
mengetahui penebalan katup pulmonal
Elektrokardiografi melihat pembesaran
di ventrikel & atrium
Chestradiografi
Chestradiografi
menentukan penurunan
aliran darah pulmonal & ukuran jantung
Katerisasi Jantung Melihat abnormalitas
pembesaran septum atrial & peningkatan
saturasi oksigen pada jantung kanan

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