Download - Penyakit jantung katup'13
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SIRKULASI JANIN
Dr. Sevina Marisya, Mked(ped), SpA
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Fetal circulation* Differs from adult circulation in several ways* Almost all differences are attributable to the fundamental defference in the site of gas exchange Adult: lungs Fetus: placenta
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Course of Fetal CirculationThere are 4 shunts in fetal circulation:• Placenta• Ductus venosus• Foramen ovale• Ductus Arteriosus
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Some important aspects of fetal circulation:1. The placenta receives the largest amount of
combined ventricular output(55%) and has the lowest vascular resistance in the fetus
2. SVC drains the upper part of the body, IVC drains the lower part of the body and placenta. O2 saturation in the IVC(70%) is higher than in the SVC(40%)
3. Most of SVC blood goes to the RV. One third of the IVC blood is directed by the crista dividens to the LA through the foramen ovale, the remaining two third enters the RV and PA.
4. Less oxygenated blood in the PA flows through the widely open ductus arteriosus to the descending aorta and then to the placenta for oxygenation.
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Changes in Circulation after Birth
The primary change in circulation
after birth is a shift of blood flow
for gas exchange from the placenta to the lungs.
The placental circulation disappears, and the pulmonary circulation is established.
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1. Interruption of the umbilical cord result in the following: a. Systemic vascular resistance >> as a result of the removal of the very low resistance placenta b. Closure of the ductus venosus as a result of lack of blood return from the placenta2. Lung expansion results in the following: a. Pulmonary vasc resistance <<, pulmonary blood flow >> and fall in PA pressure b. Functional closure of foramen ovale as a result of increased pressure in the LA c. Closure of patent ductus arteriosus (PDA) as a result of increased arterial oxygen saturation.
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Thank you
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PENYAKIT JANTUNG BAWAAN
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Structures of the heart
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Normal Heart
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• Penyakit jantung bawaan (PJB):– Non-sianotik terbesar
• Defek Septum Ventrikel (DSV) : 30%• Defek Septum Atrium (DSA)• Duktus Arteriosus Persisten (DAP)• Stenosis Pulmonal
– Sianotik• TOF (Tetralogi of Fallot)• Atresia Pulmonal• TGA (Tranposisi Great Artery)• Single Ventrikel
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Defek Septum Ventrikel• Insiden
30 % dari PJB
• Anatomi Defek subarteri : di bawah katup aorta dan pulmonal Defek Perimembran: below aortic valve at pars membranous septum Defek Muskular
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• Menurut besarnya diklasifikasikan:– DSV kecil : < 5 mm2/m 2 luas permukaan
tubuh– DSV sedang : 5-10 mm2/m 2 luas
permukaan tubuh– DSV besar : defek lebih dari ½ diameter
aorta atau > 10 mm2/m 2 luas permukaan tubuh
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VSD
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Ventricular Septal Defect
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LA LV
RV RA
PA AO
Systemic
Lungs
Qp > Qs
Ventricular Septal defect
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RA
RV
RA LALA
RV LVLV
Ventricular septal defect
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Defek Septum Ventrikel
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Defek Septum Ventrikel
• Clinical findings
Day 1st after birth: murmur (-)
After 2-6 weeks : murmur (+)
Murmur : pansystolic grade 3/6 or higher
at LSB 3
Small muscular defect: early systolic murmur
Significant defect: Mid diastolic murmur at apex
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Small VSD
Large VSD
Ventricular Septal Defect
Murmur: pansystolic grade 3/6 or higher at LSB 3
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Ventricular Septal Defect
KardiomegaliKonus pulmonalis menonjolVaskularisasi paru meningkatApex down ward
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Defek septum Ventrikel
Diagnosis Differential
PDA with PH Tetralogy Fallot non cyanotic Inoscent murmur
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Defek septum ventrikel
Management:
Definitive : VSD closure Surgery Transcatheter closure
Gagal Jantung : Digoksin 0.01 mg/kg/hari dibagi 2 dosisInfeksi Sal. Nafas: antibiotik
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DSV
Heart failure (+) Heart failure (-)
Anti failure
Fail Success
PAB
Evaluate in 6 mths
Surgical closure/Transcatheter closure
Aortic valve prolaps
Infundibular stenosis
PH SmallerSpontaneousclosure
Cath
PVD(-) PVD(+) Cath
Cath
Reactive Non-reactive
Conservative
FR>1.5FR<1.5
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Ventricular septal defect
VSD before occlusion
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Ventricular septal defect
VSD during deploying the device
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Ventricular septal defect
VSD after occludedusing ASO
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Defek Septum Atrium (DSA)
• Insiden : + 10 % : ratio = 1,5 to 2 : 1• Anatomi :
DSA Sekundum: Defect on foramen ovale DSA Sinus venosus: Defect at SVC and RA junction DSA primum: Defect at ostium primum
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ASD
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Atrial Septal Defect
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Atrial Septal Defect
Diagram of ASD
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LA LV
RV RA
PA AO
Systemic
Lungs
Qp > Qs
Atrial septal defect
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RA
RV
LA
LV
RA
RV
LA
LV
Atrial septal Defect
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Klinis- Asymptomatic- Auskultasi: - Bunyi jantung I normal atau
mengeras - Bising ejeksi sistolik di daerah
pulmonum - Bising diastolik daerah
trikuspid
Defek Septum Atrium
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Atrial Septal Defect
Auscultation :1st HS N or loud
widely split and fixed 2nd HS
Ejection Sistolic Murmur
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ECG : RBBB right ventricular hypertrophy
Atrial Septal Defect
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Pembesaran atrium kananKonus pulmonum menonjolVaskularisasi paru meningkat sesuai besarnya pirau
Atrial Septal DefectChest X-Ray
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Defek Septum Atrium
Diagnosis Differential
Primary Atrial Septal DefectECG : LAD
Partial Anomalous Pulmonary Vein Drainage
Pulmonary Stenosis Innocent Murmur
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Defek Septum Atrium
ManagementSurgery : Preschool age 4-5 thnRecent treatment: transcatheter closure using ASO (Amplatzer septal occluder)
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ASD
Small Shunt Large Shunt
Observation
EvaluationAt age 5-8 yrs
Cath
FR<1.5 FR>1.5
Conservative
Infants Children/Adults
Heart Failure (-)
Heart Failure (+)
Age >1yrsW >10kg
Transcatheter closure (Secundum ASD) /Surgical Closure(others)
Conservative
Anti failure
FailSuccess
PH (-) PH (+)
PVD (-)
PVD (+)
Hyperoxia
Reac-tive
Nonreactive
SurgicalClosure
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Atrial septal defect
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Atrial septal defect
ASD before occlusion
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During balloon sizing
Atrial septal defect
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Atrial septal defect
ASD after occluded using ASO
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PENYAKIT JANTUNG BAWAAN SIANOTIK
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Tetralogy Fallot
Insiden5-8% dari PJBAnatomiCause: Left-anterior deviation of infundibular septumSindroma consist of 4 items:
VSD pulmonal stenosis aortic over-riding RVH
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Tetralogy Fallot
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Tetralogy Fallot
Hemodynamic acyanotic Hemodynamic cyanotic
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Tetralogy Fallot
• Diagnosis
Klinis:- sianosis PS- jari tabuh setelah 6 bln- sianotik spell : sesak mendadak, nafas cepat dalam, lemas, kejang, koma- Squatting (sering jongkok)- Single 2nd HS, ejection systolic murmur
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Tetralogy Fallot
Single 2nd HS, ejection systolic murmur
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Tetralogi Fallot
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Foto thoraks: - Boot-shaped- Apeks jantung
terangkat- Konus
pulmonalis cekung
- Vaskularisasi paru berkurang
Tetralogy Fallot
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Tetralogy Fallot
ECG : RADEchocardiography : to confirm diagnosis
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Tetralogy Fallot
• Diagnosis Differential Pulmonary Atresia Double outlet right ventricle and pulmonary stenosis Transposisi of great arteri and pulmonary stenosis
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• Management Sianotic spell:
- knee-chest position- O2 sungkup 5-8l/i- Morfin sulfat 0.1-0.2 mg/kg/subkutan- Sodium bikarbonat 1 mEq/kg/iv
- Propanolol 0.1 mg/kg/iv cegah dehidrasi dan rumatan propanolol
Bedah:- Paliative treatment: Blalock-Taussig
shunt - Definitive: total correction
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Tetralogy of Fallot
< 1 yr > 1 yr
spell (+) spell (-)propranolol
failed succeed
BTS
total correction
cath
small PA good sized PA
• clinically• ECG
• CXR• echo
age 1 yr
cath BTS/
PDA Stent
evaluation
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Tetralogy Fallot
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Tetralogy Fallot
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