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dinding pemisah antara dua atrium

jantung.
➔ Adanya lubang di septum
Overview menyebabkan sejumlah darah di
atrium kiri berpindah ke atrium
- US: 1,000,000 adults with congenital
kanan. Akibatnya, darah kaya oksigen
heart disease
yang seharusnya dipompa ke seluruh
- ± 50% of all patients born with a
tubuh malah kembali ke paru-paru
congenital heart disease needs life long,
dan bercampur dengan darah yang
specialised care (Meberg, Otterstadt et al)
miskin oksigen.
Etiology

- Genetic
➔ single gene mutation
➔ syndromes Noonan Leopard, Ells van
Creveld, Kartagener
- environmental
➔ Maternal rubella(PDA, PS, ASD),
➔ Thalidomide and isotretionin early
during gestation (cardiac
malformation nonspesific),
➔ Chronic maternal alcohol abuse(VSD)
- Multifactorial induce Embriological
Malformation
- Multifactorial induce Embriological - Prevalence :
Malformation ➔ 1/1500 live births
➔ 5-10% of all CHD
➔ Female > Male
- Keywords:
➔ Acyanotic
➔ Plethora
➔ Shunt in the atrium
- Types

- PAPVR = PARTIAL ANOMALOUS


PULMONARY VENOUS RETURN ➔ Secundum defect (50-70%)
- PVOD = PULMONARY VASCULAR ◼ the site of fossa ovalis
OBSTRUCTIVE DISEASE (EISENMENGER’S ➔ Primum defect (15-30%)
SYNDROME) ◼ associated with other endocardial
- ECD = ENDOCARDIAL CUSHION DEFECT cushion defects
- PTA = persistent truncus arteriosus ➔ Sinus Venosus (10%)
- TA=Tricuspid atresia ◼ 2 type (superior and inferior vena
- HLHS=HYPOPLASTIC LEFT HEART caval type)
SYNDROME ◼ large, associated with anomalous
- TAVPR=TOTAL ANOMALOUS pulmonary venous drainage
PULMONARY VENOUS RETURN ➔ Coronary sinus (rare)
◼ associated with unroofed coronary
Atrial Septal Defect
sinus
- Atrial septal defect (ASD) adalah kelainan - clinical manifestation
bawaan berupa lubang atau celah pada ➔ Symptom
◼ Most ASDs are asymptomatic →
until adult
◼ DOE
◼ Fatigue
◼ Recurrent lower RTI
◼ Palpitation due to atrial
tachyarrythmias
➔ Physical Examination
◼ RV heaving
◼ Fixed splitting S2
◼ Systolic murmur at upper LSB
◼ Middiastolic murmur at lower LSB
➔ Diagnostic Studies
◼ Chest radiographs (RAH, RVH,
- Prevalence
Prominent PA with increased
➔ Most common CHD 15-20%
pulmonary vascular)
- Types of VSD:
◼ ECG (RVH, RAH, RBBB)
➔ Perimembraneous (70%)
◼ Echo (RVH, RAH, ASD)
➔ Inlet (5-8%)
◼ Cardiac Catheterization to confirm
➔ Outlet (infundibular) (5-7%)
ASD, assess pulmonary vascular
➔ Muscular defect (5-20%) marginal,
resistance, and diagnose
central, or apical
concurrent CAD in adult
- Keywords:
- Therapy
➔ Acyanotic
➔ Percutaneous Closure
➔ Plethora
◼ only for secundum (contra in
➔ Shunt in the ventricle
others)
- Clinical manifestations
◼ adequate superior/inferior rim
➔ Symptom
around ASD
◼ If small >> VSDs remain
◼ no R-L shunting
asymptomatic
➔ Surgical Closure
◼ If large >> CHF (tachypnea, FD, FTT,
◼ Large defect (>10mm) unless
frequent lower RTI)
pulmonary vasculer disease
◼ VSD + PVD + reversed shunt >>
◼ Good prognosis:
dyspnea, cyanosis
➢ closure age < 25, PA pressure
◼ Baterial endocarditis
<40
➢ If >25 or PA>40, decreased
survival due to CHF, stroke, and
afib

Ventricular Septal Defect

- A ventricular septal defect (VSD) is a hole


in the heart that's present at birth
(congenital heart defect). The hole is
between the lower heart chambers (right
and left ventricles). It allows oxygen-rich
blood to move back into the lungs
instead of being pumped to the rest of
the body.
seluruh tubuh melalui ductus
arteriosus.
➔ Ductus arteriosus adalah pembuluh
darah yang menghubungkan aorta
(pembuluh yang mengalirkan darah
kaya oksigen dari jantung ke seluruh
tubuh) dan arteri pulmonal
(pembuluh yang mengalirkan darah
dari jantung ke paru-paru).

➔ X-ray:
◼ Cardiomegaly of varying degrees
◼ Pulmonary Vascular marking
increase
◼ Echocardiography
➔ Physical examination
◼ RV heaving
◼ Wide splitting S2, accentuated P2
◼ Harsh holosystolic murmur at LSB
◼ Small defect >> louder murmur >>
- Prevalence:
great turbulence
➔ 5-10% of all CHD, Female:Male 1:3
◼ Systolic thrill at LSB
- Keywords:
◼ Middiastolic rumble at apex
➔ Acyanotic
◼ If PVD, murmur diminishes and
➔ Plethora
cyanosis may be present
➔ Shunt between PA and Descending
➔ Diagnostic Studies
Aorta
◼ Chest radiographs (LAH, LVH, RVH,
- Clinical manifestations
Prominent PA with increased
➔ History: asymptomatic if the ductus is
pulmonary vascular)
small, may cause lower respiratory
◼ ECG (LAH, LVH, RAH)
infection, atelektasis, CHF
◼ Echo (LAH, LVH, RAH, VSD)
➔ Physical Examination: tachycardia,
◼ Cardiac Catheterization (02 sat in
Continuous murmur at left
RV >> RA)
infraclavicular area, maybe cyanosis
➔ Treatment
only in the lower half of body, systolic
◼ By age 2 at least 50% small and
thrill at ULSB
moderate VSDs undergo partial or
➔ ECG: LVH (small-mod PDA), LVH+RVH
complete spontaneous closure
(large PDA)
◼ Surgical is recommended in first
➔ X-ray: cardiomegaly varying degrees,
few months for CHF or PVD
pulmonary vasc marking increased
children, if no PVD, can be
➔ Echocardiography
corrected later
- Treatment
Patent Ductus Arteriosus ➔ Spontaneous closure during 1st month
(rare)
- Patent ductus arteriosus (PDA) adalah ➔ In the absence of other CHD >> should
kondisi ketika pembuluh darah yang
be occluded (surgical or ligation)
menghubungkan aorta dan arteri paru
➔ For neonatus and premature with
tetap terbuka setelah bayi lahir. PDA
CHF >> prostaglandin synthesis
merupakan jenis kelainan jantung
bawaan yang biasanya dialami oleh bayi inhibitor (indomethacin)
prematur. Tetralogy of Fallot
➔ Selama di dalam rahim, bayi belum
membutuhkan paru-paru untuk - Tetralogy of Fallot adalah salah satu
bernapas karena sudah mendapatkan penyakit jantung bawaan pada bayi baru
oksigen dari ari-ari (plasenta). Oleh lahir. Penyakit jantung ini terdiri dari empat
sebab itu, sebagian besar darah yang jenis kelainan struktur pada jantung bayi.
akan menuju paru-paru dialihkan ke
➔ Tetralogy of Fallot menyebabkan darah
yang dialirkan ke seluruh tubuh bayi
tidak mengandung cukup oksigen
➔ Tetralogy of Fallot (ToF) terjadi ketika
bayi masih di dalam kandungan,
tepatnya ketika jantungnya sedang
berkembang
➔ Kelainan struktur jantung pada ToF
menyebabkan darah yang kaya
oksigen bercampur dengan darah
yang kekurangan oksigen. Akibatnya,
darah yang mengalir ke seluruh tubuh
tidak mengandung oksigen yang
cukup dan bayi pun kekurangan
oksigen.

➔ is a palliative surgical procedure to


treat patients with cyanotic heart
diseases characterized by decreased
pulmonary artery flow.
➔ The BT shunt aims to supply the
pulmonary artery with blood flow
sufficient to relieve cyanosis without
inducing pulmonary over-circulation.
The classic BT shunt procedure was
performed through a lateral
➔ Berikut ini adalah empat kelainan thoracotomy, dividing the subclavian
jantung yang terjadi pada ToF: artery and anastomosing it to the
◼ Ventricular septal defect (VSD), pulmonary artery in an end-to-side
yaitu lubang di dinding yang manner. The original technique has
memisahkan ventrikel kanan dan been modified extensively and has
kiri
evolved to the mBTT shunt, which
◼ Pulmonary valve stenosis, yaitu
utilizes an interposition
penyempitan pada katup
pembuluh darah yang ke paru- polytetrafluoroethylene (PTFE) graft to
paru (katup pulmonal) sehingga establish a systemic-pulmonary shunt
darah dari jantung yang ke paru- without sacrificing the subclavian
paru berkurang artery or any of the brachiocephalic
◼ Posisi aorta tidak normal, yakni tributaries
bergeser ke kanan mengikuti VSD
yang terbentuk (Dextroposition of DENTAL PROCEDURE
Aorta)
- Elimination of focal infection before
◼ Right ventricular hypertrophy atau
correction of CHD
penebalan pada otot ventrikel
kanan jantung akibat jantung - In highest risk population, prophylaxis of
bekerja terlalu keras untuk IE should be considered before dental
memompa darah procedure
- Clinical manifestations
➔ History: cyanosis at birth/shortly
thereafter, Dyspneu,Squatting, hipoxic
spells develop later
➔ Physical Examination:
◼ Cyanosis, tachypnea, clubbing
◼ ESM at the upper, S2 single
➔ ECG: RAD (cyanotic TOF), RVH, RAH
➔ X-ray: oligemi, heart size N/<, Boot
shaped heart

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