Dokumen tersebut membahas tentang penyakit jantung bawaan sianotik yang menyebabkan warna biru pada kulit (sianosis), seperti transposisi pembuluh darah besar (TGA), tetralogi Fallot (TOF), dan anomalitas pembuluh darah paru total. Dokumen tersebut menjelaskan epidemiologi, etiologi, patofisiologi, gejala klinis, pemeriksaan diagnostik, penatalaksanaan bedah maupun non-bedah, serta komplik
0 penilaian0% menganggap dokumen ini bermanfaat (0 suara)
302 tayangan44 halaman
Dokumen tersebut membahas tentang penyakit jantung bawaan sianotik yang menyebabkan warna biru pada kulit (sianosis), seperti transposisi pembuluh darah besar (TGA), tetralogi Fallot (TOF), dan anomalitas pembuluh darah paru total. Dokumen tersebut menjelaskan epidemiologi, etiologi, patofisiologi, gejala klinis, pemeriksaan diagnostik, penatalaksanaan bedah maupun non-bedah, serta komplik
Dokumen tersebut membahas tentang penyakit jantung bawaan sianotik yang menyebabkan warna biru pada kulit (sianosis), seperti transposisi pembuluh darah besar (TGA), tetralogi Fallot (TOF), dan anomalitas pembuluh darah paru total. Dokumen tersebut menjelaskan epidemiologi, etiologi, patofisiologi, gejala klinis, pemeriksaan diagnostik, penatalaksanaan bedah maupun non-bedah, serta komplik
Dokumen tersebut membahas tentang penyakit jantung bawaan sianotik yang menyebabkan warna biru pada kulit (sianosis), seperti transposisi pembuluh darah besar (TGA), tetralogi Fallot (TOF), dan anomalitas pembuluh darah paru total. Dokumen tersebut menjelaskan epidemiologi, etiologi, patofisiologi, gejala klinis, pemeriksaan diagnostik, penatalaksanaan bedah maupun non-bedah, serta komplik
Cont Lesi Campuran Klinis : - sianosis - gagal jantung kongestif - corakan pembuluh darah paru meningkat Jenis kelainan : TGA Trunkus arteriosus Anomali total muara VP 12
Tetralogy of Fallot
13
Pathology of TOF Leftward deviation malalignment of ventricular septal defect + aortic overriding Anterior deviation pulmonary stenosis right ventricle outflow tract obstruction right ventricular hypertrophy
14 Bove EL, Lupinetti FM. Tetralogy of Fallot. Pediatric cardiac surgery. 1994.
Pathology of TOF VSD in TOF is a perimembranous defect RV outflow tract obst is most frequenly infundibular stenosis The PA branches are usually small Right aortic arch is present in 25% of cases In about 5% abnormal coronary arteries are present 15
Clinical Manifestation of TOF
Cyanotic of the skin and mucous membranes ToF desaturation of arterial blood increased concentration of reduced hemoglobin > 5g/dL in circulation Clinical manifestation depends on the source and volume of pulmonary blood flow ductus arteriosus and or aortopulmonary collaterals
Park MK. Pathophysiology of cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002. Kulkarni A, Pettersen M. Tetralogy of Fallot with pulmonary atresia. www.emedicine.com. 16 Park MK. Cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002.
Clinical Manifestation
Newborn infant in whom the ductus
arteriosus is the sole source of pulmonary blood flow increasingly cyanotic as the DA closes Severe pulmonary stenosis or pulmonary atresia cyanotic at birth or soon after birth ToF with severe PS or pulmonary atresia duct-dependent congenital heart defect Park MK. Pathophysiology of cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002. Kulkarni A, Pettersen M. Tetralogy of Fallot with pulmonary atresia. www.emedicine.com. 17 Park MK. Cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002.
Dyspnea on exertion Squatting Hypoxic spells Failure to thrive
Transposition of Great Artery Insidence 5% of CHD Anatomy Abnormality of formation of trunkal septum that cause aorta arising from RV and PA arising from LV
Transposition of Great artery
Transposition of Great artery
Hemodynamic normal series
Hemodynamic of TGA parallel
Transposition of Great artery
Clinical aspects
More frequent in male
Birth weight usually normal normal or bigger Cyanotic vary from mild to severe Auscultation : single 2nd HS and loud Murmur vary from silent to pansystolic murmur or continuous murmur
Transposition of Great artery
Diagnosis Clinically : Suspicious if neonates presents with cyanotic with birth weight normal or bigger Murmur (-) Single 2nd HS and loud
Intervensi Bedah : paliatif : BT-shunts , PA Banding Korektif : Biventrikular repair, one and half vent repair, dll
Non-Bedah Amplatzer Ballon dll
37
Palliative surgery
38
Bedah paliatif
39
Complications
Blok jantung / RBBB
Residual shunts Bacterial endocarditis Pulmonary hypertension bleeding problems / polycythemic Delayed growth and development Congestive Heart Failure
40
Hypoxic Spell Hypoxic spells may develop
before total
repair Increasing cyanosis Decreasing intensity of the heart murmur Hyperpnoea (rapid and deep) Severe spell convulsion, cerebrovascular accident death
Park MK. Pathophysiology of cyanotic congenital heart defects. Pediatric cardiology for practitioners. 41 2002. Bove EL, Lupinetti FM. Tetralogy of Fallot. Pediatric cardiac surgery. 1994.
Prognosis Tergantung -Derajat / kompleksitas penyakit -Tingkat kesulitan opeasi -Usia saat koreksi
Total correction of TF
43
The heart is not
everything, But without a heart, Everything is nothing
