pjb pbl non sianotik
TRANSCRIPT
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PENYAKIT JANTUNG BAWAAN NON-SIANOTIK
PENYAKIT JANTUNG BAWAAN NON-SIANOTIK
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Tujuan pembelajaranTujuan pembelajaran
• Umum : mampu mengidentifikasi / mengelola PJB dengan pendekatan
dokter keluarga
• Khusus : mampu menjelaskan • Epidemiologi• Etiologi / faktor risiko• Patofisiologi• Pemeriksaan penunjang• Prinsip diagnosis / Diagnosis banding• Penatalaksanaan / rujukan• Komplikasi / prognosis
• Umum : mampu mengidentifikasi / mengelola PJB dengan pendekatan
dokter keluarga
• Khusus : mampu menjelaskan • Epidemiologi• Etiologi / faktor risiko• Patofisiologi• Pemeriksaan penunjang• Prinsip diagnosis / Diagnosis banding• Penatalaksanaan / rujukan• Komplikasi / prognosis
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Acyanotic defects of CHDAcyanotic defects of CHD
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EpidemiologiEpidemiologi
• PJB 0,8-1% dari bayi lahir hidup
• 75% merupakan PJB non-sianotik
PJB non-sianotik
• VSD : 20% dari semua PJB
• PDA : 7% dari semua PJB
• ASD : 8% dari semua PJB
• PJB 0,8-1% dari bayi lahir hidup
• 75% merupakan PJB non-sianotik
PJB non-sianotik
• VSD : 20% dari semua PJB
• PDA : 7% dari semua PJB
• ASD : 8% dari semua PJB
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Etiologi / Faktor risikoEtiologi / Faktor risiko
• Sebagian besar kasus tidak diketahui
• Obat-obatan• Penyakit ibu• Pajanan sinar X• Genetik / sindrom tertentu• Multifaktorial
• Sebagian besar kasus tidak diketahui
• Obat-obatan• Penyakit ibu• Pajanan sinar X• Genetik / sindrom tertentu• Multifaktorial
Etiologi ???
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Chromosomal aberrationsChromosomal aberrations
• Trisomy 13 syndrome (Patau’s syndrome) : 25% CHD : VSD, PDA, ASD
• Trisomy 18 ( Edward’s syndrome) : 90% CHD : VSD, PDA, dextrocardia
• Trisomy 21 ( Down syndrome) : 50% CHD : ECD , VSD
• Turner’s syndrome (XO) : 35% CHD : CoA, AS, ASD
• Klinefelter’s variant (XXXXY) : 15% CHD : PDA , ASD
• Trisomy 13 syndrome (Patau’s syndrome) : 25% CHD : VSD, PDA, ASD
• Trisomy 18 ( Edward’s syndrome) : 90% CHD : VSD, PDA, dextrocardia
• Trisomy 21 ( Down syndrome) : 50% CHD : ECD , VSD
• Turner’s syndrome (XO) : 35% CHD : CoA, AS, ASD
• Klinefelter’s variant (XXXXY) : 15% CHD : PDA , ASD
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Hemodinamik PJBHemodinamik PJB
• Kelebihan beban volume• Obstruksi aliran ke ventrikel• Obstruksi aliran keluar ventrikel• Gangguan kontraksi dan
relaksasi ventrikel
• Kelebihan beban volume• Obstruksi aliran ke ventrikel• Obstruksi aliran keluar ventrikel• Gangguan kontraksi dan
relaksasi ventrikel
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Beban volume berlebihanBeban volume berlebihan
• Shunt dari kiri-kanan– Beban volume di ventrikel– Sirkulasi berlebihan ke pulmonal– Penyempitan arteriole paru– Peningkatan tahanan aliran darah
paru
• Shunt dari kiri-kanan– Beban volume di ventrikel– Sirkulasi berlebihan ke pulmonal– Penyempitan arteriole paru– Peningkatan tahanan aliran darah
paru
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Cont ……Cont ……
• Shunt kiri-kanan :– Tingkat atrium
•DSA tipe sinus venosus / PAPVD
– Tingkat ventrikel : VSD– Tingkat pb darah besar
•PDA•Trunkus arteriosus•AP window
• Shunt kiri-kanan :– Tingkat atrium
•DSA tipe sinus venosus / PAPVD
– Tingkat ventrikel : VSD– Tingkat pb darah besar
•PDA•Trunkus arteriosus•AP window
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LA LV
RV RA
PA AO
Systemic
Lungs
Qp > Qs
Atrial septal defect
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LA LV
RV RA
PA AO
Systemic
Lungs
Qp > Qs
Ventricular Septal defect
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Cont….Cont….
• Shunt kanan-kiri : jika tahanan arteriole paru
> tahanan sirkulasi sistemik sianosis ( Eisenmenger sindrome )
• Shunt kanan-kiri : jika tahanan arteriole paru
> tahanan sirkulasi sistemik sianosis ( Eisenmenger sindrome )
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Lesi ObstruktifLesi Obstruktif
• Lesi obstruktif dengan defek ki-ka
• Lesi obstruktif tanpa defek
shunts tergantung beratnya defek
• Contoh : obs aliran masuk ventrikel– Stenosis mitral / trikuspidal– Cor triatrium– Anomali Ebstein
• Lesi obstruktif dengan defek ki-ka
• Lesi obstruktif tanpa defek
shunts tergantung beratnya defek
• Contoh : obs aliran masuk ventrikel– Stenosis mitral / trikuspidal– Cor triatrium– Anomali Ebstein
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Cont….Cont….
• Obstruksi saluran keluar ventrikel :– Stenosis aorta / pulmonal– Hipertensi sistemik / pulmonal– Koarktasio Ao/P
• Gangguan kontraksi ventrikel– Kardiomiopati
• Obstruksi saluran keluar ventrikel :– Stenosis aorta / pulmonal– Hipertensi sistemik / pulmonal– Koarktasio Ao/P
• Gangguan kontraksi ventrikel– Kardiomiopati
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Manifestasi klinisManifestasi klinis
• Tergantung jenis PJB
• Sianotik / non-sianotik
• Gangguan tumbuh kembang
• ISPA berulang
• Cepat lelah
• Sesak
• Gagal jantung
• Tergantung jenis PJB
• Sianotik / non-sianotik
• Gangguan tumbuh kembang
• ISPA berulang
• Cepat lelah
• Sesak
• Gagal jantung
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Pemeriksaan penunjangPemeriksaan penunjang
• Hematology / AGD
• Foto toraks
• Elektrokardiografi ( EKG )
• Ekokardiografi
• Kateterisasi
• Hematology / AGD
• Foto toraks
• Elektrokardiografi ( EKG )
• Ekokardiografi
• Kateterisasi
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PA and Lateral chest x-rayPA and Lateral chest x-ray
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Ventricular Septal Defect
CardiomegalyApex down wardProminence pulmonary artery segmentIncreased pulmonary vascularmarking
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Normal ECGNormal ECG
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Normal color flow image
4-chamber
Color Doppler Techniques & Evaluation
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Ventricle septal defect
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Kateterisasi PDAKateterisasi PDA
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Kateterisasi ToF-PAKateterisasi ToF-PA
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Diagnosis Diagnosis
Tahapan diagnosis PJB :– Evaluasi klinis : riwayat penyakit /
anamnesis dan pemeriksaan fisik– Pemeriksaan penunjang sederhana :
EKG , foto toraks, darah rutin– Ekokardiografi : M-mode , 2-dimensi,
doppler (color flow mapping)– Kateterisasi jantung : hemodinamik
dan angiografi
Tahapan diagnosis PJB :– Evaluasi klinis : riwayat penyakit /
anamnesis dan pemeriksaan fisik– Pemeriksaan penunjang sederhana :
EKG , foto toraks, darah rutin– Ekokardiografi : M-mode , 2-dimensi,
doppler (color flow mapping)– Kateterisasi jantung : hemodinamik
dan angiografi
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Cont…Cont…
• Foto toraks :– Kardiomegali ( LVH / RVH )– Vaskularisasi paru– Cardiac silhouette
• EKG :– Posisi jantung– Hipertrofi / Dilatasi– dll
• Foto toraks :– Kardiomegali ( LVH / RVH )– Vaskularisasi paru– Cardiac silhouette
• EKG :– Posisi jantung– Hipertrofi / Dilatasi– dll
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Clinical findings
Asymptomatic
A relatively slender body build is typical
Auscultation :
Normal 1st HS or loud Widely split and fixed 2nd HS
Ejection systolic murmur
Clinical findings
Asymptomatic
A relatively slender body build is typical
Auscultation :
Normal 1st HS or loud Widely split and fixed 2nd HS
Ejection systolic murmur
Atrial septal DefectAtrial septal Defect
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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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Atrial Septal Defect
Diagram of ASD
Sinus venosus defect
Secundum ASD
Primum ASD
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Atrial Septal Defects
(View from right side)
Atrial Septal Defects
(View from right side)
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Right atrial enlargementProminence the MPA segmentIncreased pulmonary vascular marking
Atrial Septal DefectChest X-Ray
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Ventricular Septal DefectVentricular Septal Defect
• 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
• 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 DefectVentricular septal Defect
Diagnosis Differential
PDA with PH Tetralogy Fallot non cyanotic Inoscent murmur
Diagnosis Differential
PDA with PH Tetralogy Fallot non cyanotic Inoscent murmur
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Patent Ductus ArteriosusPatent Ductus Arteriosus
• Clinical findings
Small defect: Symptom (-) Growth and development normal
Significant defect:Decreased exercise tolerantWeigh gained not good
Specific case: pulsus seler at 4th extremities and continuous murmur
• Clinical findings
Small defect: Symptom (-) Growth and development normal
Significant defect:Decreased exercise tolerantWeigh gained not good
Specific case: pulsus seler at 4th extremities and continuous murmur
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Patent Ductus Arteriosus
Auscultation : continuosus murmur at upper LSB 2
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Diagnosis DifferentialAP-window
Arterio-venous fistulae
Management premature : indomethacin
PDA closure : surgery
transcatheter closure
Diagnosis DifferentialAP-window
Arterio-venous fistulae
Management premature : indomethacin
PDA closure : surgery
transcatheter closure
Patent Ductus ArteriosusPatent Ductus Arteriosus
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Indomethacin Indomethacin
• Hari I : 0,2 mg/kgbb/hari
• Hari II – VII : 0,1 mg/kgbb/hari
– evaluasi dengan ekokardiografi– efektif pada bayi prematur
• Hari I : 0,2 mg/kgbb/hari
• Hari II – VII : 0,1 mg/kgbb/hari
– evaluasi dengan ekokardiografi– efektif pada bayi prematur
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Tatalaksana Tatalaksana
• Tergantung jenis kelainan PJB
• Medikamentosa– Mengurangi preload / afterload– Inotropik– Mengurangi serangan hipoksia :
propranolol– Penutupan duktus : indometasin /
ibuprofen– Mempertahankan duktus :
prostaglandin E1
• Tergantung jenis kelainan PJB
• Medikamentosa– Mengurangi preload / afterload– Inotropik– Mengurangi serangan hipoksia :
propranolol– Penutupan duktus : indometasin /
ibuprofen– Mempertahankan duktus :
prostaglandin E1
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Cont….Cont….
• Intervensi– Bedah :
• paliatif : BT-shunts , PA Banding
• Korektif : Biventrikular repair, one and half vent repair, dll
– Non-Bedah• Amplatzer• Ballon• dll
• Intervensi– Bedah :
• paliatif : BT-shunts , PA Banding
• Korektif : Biventrikular repair, one and half vent repair, dll
– Non-Bedah• Amplatzer• Ballon• dll
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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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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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PDA
Neonates/Infants Children/Adults
Heart failure (+) Heart failure (-)
Premature Full term
Anti failureIndometacin
Success Fail
Spontaneous closure
Anti failure
SuccessFail
Surgical ligation
Transcatheter closure
PH (-) PH (+)
LR RL
Hyperoxia
Reactive Nonreactive
Conservative
Age >12wksW >4kg
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Amplatzer Occlusion of Atrial Septal DefectAmplatzer Occlusion of Atrial Septal Defect
Clockwise from above: Transcatheter delivery of Amplatzer device, which is positioned across the atrial septal defect
Left: Amplatzer device in place
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Complications / prognosis Complications / prognosis
• Blok jantung / RBBB• Residual shunts • Bacterial endocarditis• Pulmonary hypertension• bleeding problems / polycythemic• Delayed growth and development• Congestive Heart Failure
• Blok jantung / RBBB• Residual shunts • Bacterial endocarditis• Pulmonary hypertension• bleeding problems / polycythemic• Delayed growth and development• Congestive Heart Failure
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Non-surgical closure using the amplatzer
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Intervensi non-bedahIntervensi non-bedah
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Your attention