Download - penyakit jantung bawaan.pdf
![Page 1: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/1.jpg)
PENYAKIT JANTUNG BAWAAN
Ganesja M harimurti Departemen kardiologi dan kedokteran Vaskular FKUI/ Pusat Jantung Nasional Harapan Kita
![Page 2: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/2.jpg)
Bentuk kelainan saat lahir yang paling sering Penyebab utama kematian pada tahun pertama kelahiran 8/1000 kelahiran hidup
VSD 30-50%, PDA 10%, ASD 7%. PS 7% Coarctation 6%, AS 5% Tetralogy 5%, TGA 5% AV canal defects 3%
Gejala klinis minimal s/d berat dan memerlukan penanganan segera
Tidak muncul gejala sampai tahunan
Lilly. Pathophysiology of Heart Disease. Congenital Heart Disease. 2007.
![Page 3: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/3.jpg)
Etiologi PJB
GENETIK Syndromes:
Noonan, Leopard, Ellis van Creveld,
Kartagener, Alcapa, Alagille, DiGeorge,
Down, Scimitar, Holt-Oram, Turner, William,
Shone complex
ENVIROMENTAL Maternal rubella (PDA, PV, ASD)
Thalidomide and Isotretinoin (cardiac malformation)
Lithium (TV)
Maternal alcohol abuse (VSD)
DM, hipertensi
Braunwald. Heart Disease. Congenital Heart Disease. 2008.
![Page 4: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/4.jpg)
Alur diagnostik
Anamnesa
riwayat penyaki
t
Pemeriksaan fisik
EKG Foto torak
Ekokardiografi
Kateterisasi
jantung
![Page 5: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/5.jpg)
Anamnesa
Aliran ke paru meningkat:
-batuk panas berulang
-gangguan menyusui
-gangguan tumbuh kembang
-tidak ada riwayat spel sianotik
![Page 6: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/6.jpg)
Anamnesa
Aliran ke paru berkurang: jarang batuk panas
gangguan menyusui
gangguan tumbuh kembang
riwayat spel sianotik
sering jongkok
![Page 7: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/7.jpg)
SPEL HIPOKSIA
SINDROMA SERANGAN hiperpnoe paroksismal
gelisah (irritable)
menangis berkepanjangan (prolonged crying)
biru bertambah (increasing cyanosis)
lemas (lethargy)
kesadaran menurun
kejang-kejang
![Page 8: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/8.jpg)
awal kehidupan (3 bulan – 3 tahun) sering timbul saat bangun tidur di pagi hari pulih spontan dalam waktu < 15 – 30 menit sering berulang - komplikasi serius - Serebral
- KEMATIAN
EMERGENSI
HARUS CEPAT DIKENAL
![Page 9: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/9.jpg)
SPEL HIPOKSIA MEKANISME
HEMOGLOBIN
SPASME
INFUND. RV
TAHANAN VASKULAR SISTEMIK
- menangis
- BAB
- aktifitas fisik
- demam
R - L SHUNT
pO2
pCO2
pH
HIPERPNOE
ALIRAN BALIK
VENA SISTEMIK
TAKIKARDIA
HIPOVOLEMIA
?
- dehidrasi
- diare
aliran ke paru
![Page 10: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/10.jpg)
Tata laksana
knee-chest position
systemic vascular resistance meningkat venous return berkurang
oxygen
sedation : - morphine sulfate 0.1 - 0.2 mg/kg - SC / IM
- diazepam 0.1 - 0.2 mg/kg - IV / rectal
Mengurangi hypernea
correct acidosis : sodium bicarbonate 1 meq/kg - IV
![Page 11: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/11.jpg)
propranolol : 0.02 - 0.1 mg/kg/dose - IV
mengurangi heart rate relaksasi spasme infindibulum RV
vasokonstriktor : phenylephrine 0.02 mg/kg - IV
Meningkatkan SVR
ketamin : 1 - 2 mg/kg - IV
-Meningkatkan SVR
-Sedatif
![Page 12: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/12.jpg)
Pemeriksaan fisik
Gangguan pertumbuhan
Sianosis bibir, kuku jari tangan dan kaki Perabaan dada, nilai aktifitas jantung
yang meningkat kanan / kiri
![Page 13: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/13.jpg)
Auskultasi
Deskripsi bunyi jantung I & II bj I tunggal/ganda, intensitas
Pada normal, bj I tunggal
ganda : kelainan ktp trikuspid (Ebstein)
bj II ganda/tunggal, intensitas
bila ganda split N atau menetap
bila tunggal, intensitas N PS/PA
intensitas meningkat malposisi arteri besar
![Page 14: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/14.jpg)
Elektrokardiografi
Irama jantung Situs solitus/inversus/ambiguous (gelombang P) Aksis QRS kiri : VSD, truncus arteriosus (+LVH) kanan: ASD, TOF superior kanan: ASD primum RVH / LVH / BVH Letak Vki, Vka (morfologi QRS prec lead)
![Page 15: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/15.jpg)
Foto torak
Situs solitus : hepar di kanan, gaster di kiri
inversus: vice versa
ambigus: hepar bilateral, gaster mid
Kardiomegali
Segmen pulmonal? Menonjolaliran meningkat
Cekung aliran berkurang
Vaskularisasi paru? N/ meningkat/ berkurang
![Page 16: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/16.jpg)
Penyakit Jantung Bawaan
Sianotik
Normal
RVH LVH
Asianotik
Plethora
CoA
MR
PS
MS
CoA(infant)
LVH RVH
VSD
PDA
ECD
ASD
PAPVR
PVOD
Oligemia Plethora
RVH LVH LVH RVH
TA Pulmonary
atresia w/
hypoplastic
RV TOF
PVOD Ebstein
anomaly
TGA+PS PTA w/
hypoplastic
PA
PTA
Single
ventr
TGA+
VSD
BVH
TGA
TAPVR
HLHS
![Page 17: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/17.jpg)
Atrial Septal Defect
Prevalensi : 1/1500 kelahiran
5-10% dari seluruh PJB laki:perempuan → 1:2
![Page 18: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/18.jpg)
Tipe ASD
Sekundum (50-70%)
fossa ovalis
Primum (15-30%) Terdapat bersama defek
endocardial cushion lain
Sinus Venosus (10%) superior and inferior vena caval besar, terdapat bersama
anomalous pulmonary venous drainage
Coronary sinus (jarang) terdapat bersama unroofed
coronary sinus
![Page 19: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/19.jpg)
Manifestasi klinis Histori: biasanya asimtomatik
Pemeriksaan fisik:
Fixed wide splitting of S2.
Flow meningkat di trikuspid
diastolic rumble di LLSB.
EKG: right axis deviation,
RVH,
RBBB dengan rsR’ pattern di
V1
X –ray: kardiomegali
(pembesaran RA dan RV),
phletora
![Page 20: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/20.jpg)
ASD
Big Shunt
Observe
PH (-)
HF (+)
PH (+) Evaluate
5-8 yo Elective
> 1 yo
Cath Controlled Failed
Immediately
PVD (+)
•Clinical
•EKG
•CXR
•Echo
Medical th/
Conservative Ligation or Amplatzer Septal Occluder Conservative
FR < 1.5 reactive Non reactive
Cath
Small Shunt
HF :heart failure
PH : Plumonary hipertension
PVD : Pulmonary Vascular Diseases
ASO tidak dapat dilakukan pada bayi < 8 Kg
FR > 1.5
Baby Adolescent
Adult
HF (-)
> 1 yo
PVD (-)
![Page 21: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/21.jpg)
VENTRICULAR SEPTAL DEFECT
Prevalensi Tersering 15-20% dari Seluruh PJB
Tipe VSD: • Perimembraneous (70%) • Inlet (5-8%) • Outlet (infundibular) (5-7%) • Muscular (5-20%) marginal, central, or apical
![Page 22: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/22.jpg)
Manifestasi klinis
X-ray: kardiomegali Phletora
B. VSD moderate
• FTT, RRTI • Holosystolic murmur • Early diastolic murmur
>>> flow di mitral valve • EKG: LVH, LAH
C. VSD besar: PVR < SVR • FTT, RRTI, CHF • S2 mengeras (P2) • Ejection systolic murmur
>>> flow di RVOT • ECG: LVH + RVH, LAH
D. large VSD : PVR > SVR • + cyanosis • S2 mengeras (P2) • Murmur (-)
shunt (-) • EKG: RVH saja
A. VSD kecil • asimtomatik • Holosystolic murmur
shunt L - R • EKG: normal
![Page 23: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/23.jpg)
VSD
HF (-)
Controlled
PVD (+)
•Clinical
•EKG
•CXR
•Echo
Medical th/
VSD Closure
Conservative
FR < 1.5 reactive Non reactive
Cath
HF (+)
HF :heart failure
PH : Plumonary hipertension
PVD : Pulmonary Vascular Diseases
Reactive : PARI < 8 u/m2
FR > 1.5
Natural History
PVD (-) PAB If weight
< 3kg
Evaluate
6 mo
Prolaps
Ao valve Stenosis
Infundibulum
Pulmonal
Hypertension Smaller
Closed
Spontaneously
Cath Cath
5 yo
Cath
PARI & FR
RV : infundibular
LV : VSD type
Ao : prolaps
![Page 24: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/24.jpg)
Terapi - Ace-inhibitor , digoxin dan
diuretik
- intake kalori tinggi
- jaga higiene oral (pencegahan IE)
![Page 25: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/25.jpg)
PATENT DUCTUS ARTERIOSUS
Prevalensi: 5-10% dari seluruh PJB, Perempuan:Laki 1:3
![Page 26: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/26.jpg)
Manifestasi klinis
Histori: asimtomatik jika PDA kecil, RRTI, CHF
Pemeriksaan fisik: Continuous murmur di
area infraclavicular kiri EKG: LVH (PDA kecil - moderate), LVH+RVH
(PDA besar) X-ray: kardiomegali, phletora
![Page 27: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/27.jpg)
PDA
Adolescent/Adult
HF (+) PH (-) HF (-) PH (+)
Premature Mature
Medical th/
+
Indomethacin Controlled Failed
Elective After
>12 weeks
L→R L↔R
•Clinical
•EKG
•CXR
•Echo
Medical th/
Closed
spontaneously Ligation or Amplatzer Ductal Occluder Conservative
Controlled Failed
reactive Non
reactive Elective After
>12 weeks
Cath
Neonate/Baby
HF :heart failure
PH : Plumonary hipertension
Indomethacin
0,2 mg/kgbb 3x interval 12 hour
<10days
![Page 28: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/28.jpg)
Tetralogy of Fallot
• Stenosis Infundibular (obstruksi RVOT)
• VSD (non restriktif, subaortik perimembranous)
• Over-riding aorta
• RVH
Prevalensi: 10% dari seluruh PJB PJB sianotik paling sering
![Page 29: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/29.jpg)
Histori: sianosis , dyspneu,squatting, spell
Pem. fisik:
Sianosis, takipnea, clubbing finger
ESM di ULSB, S2 tunggal
EKG: RAD , RVH, RAH
X-ray: oligemi, ukuran jantung N / <, Boot shaped heart
Manifestasi klinis
![Page 30: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/30.jpg)
TOF
> 1 yo
Spell (+)
PROPANOLOL
Cath
Controlled Failed
•Clinical
•EKG
•CXR
•Echo
TOTAL CORRECTION OPERATION
Cath
< 1 yo
BTS : Blalock Taussig Shunt
Propanolol 0,5-1,5 mg/kg/dose 3-4x
CI : asthma
Spell (-)
BTS
PA/RV graphy
BTS
Cath
evaluate 6 mo
PA/RV graphy
Small PA Good size PA
Criteria for Operation
– Good PA size
– Good LV function
Cath
– PA confluence/size
– Anomaly coroner
– MAPCA
Spell :
– O2 100%
– Knee Chest Position
– Mo 0,1 mg/kgbb
– Diazepam 0,1 mg/kgbb
– BicNat 3-5 meq/kgbb
– Propanolol 0,02-0,1 mg/kg
– Fenilefrine
CI 2-5 mg/kgbb/mt
IV 0,02 mg/kg
IM 0,1 mg/kg
if not controlled
Ventilation
BT Shunt,sat <30
![Page 31: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/31.jpg)
Transposition of Great Arteries
• PA keluar dari LV, Aorta dari RV dan
terletak di anterior kanan of PA
• lokasi mixing :
Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
• tatalaksana awal dengan
prostaglandin agar duktus tetap
terbuka & balloon atrial
septostomy untuk memperbaiki
saturasi sistemik
![Page 32: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/32.jpg)
TGA tanpa VSD Duct dependent
Sianosis berat
Observasi hingga usia 10 hr PDA dapat menutup setiap saat.
Baloon atrial eptostomi bila ASD (-)
Prostaglandin
![Page 33: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/33.jpg)
TGA
VSD (+)
LVOTO (-) LVOTO (+) < 1 mo > 1 mo
> 3 mo Dynamic LVOTO
or
Can be resected
•Clinical
•EKG
•CXR
•Echo
< 3 mo
ARTERIAL SWITCH ARTERIAL SWITCH &
PERFORATED VSD RASTELLI
LV > 2/3
PAB Cath
VSD (-)
LVOTO :
left ventricular outflow tract obstruction
Cath
LV < 2/3
Cath
PARI
< 8
PARI
> 8
Can not be
resected
BTS
![Page 34: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/34.jpg)
Pulmonary Atresia
PA tanpa VSD
Duct dependent
Sangat biru
Murmur (-)
Foto toraks: gambaran
sepatu bot
PDA sewaktu-waktu dapat menutup spontan prostaglandin operasi (tanpa kateterisasi)
![Page 35: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/35.jpg)
PA dengan VSD (Fallot type)
• Bunyi jantung II tunggal tak mengeras
• Bising (-) / bising kontinu halus kolateral
• Foto torak: sepatu bot (= TOF)
![Page 36: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/36.jpg)
ASO
![Page 37: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/37.jpg)
AMVO
![Page 38: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/38.jpg)
ADO
![Page 39: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/39.jpg)
BPV
![Page 40: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/40.jpg)
Take Home Messages
Kenali gejala dan tanda PJB, terutama kegawat daruratan
Mencari faktor risiko
Edukasi saat kehamilan
Perujukan pasien
![Page 41: penyakit jantung bawaan.pdf](https://reader034.vdocuments.pub/reader034/viewer/2022052200/55cf9b12550346d033a49e53/html5/thumbnails/41.jpg)