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Tetralogi fallot

Presentasi Kasus. Tetralogi fallot. Davrina Rianda Modul Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Indonesia. Tetralogi Fallot. ILUSTRASI KASUS. Tetralogi Fallot. ANAMNESIS. Identitas. Nama : An. DNH Nama orangtua : Ny . TR NRM : 387-54-53 TL : 28 Maret 2013

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Tetralogi fallot

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  1. PresentasiKasus Tetralogifallot Davrina Rianda ModulIlmuKesehatanAnak FakultasKedokteran Universitas Indonesia

  2. TetralogiFallot ILUSTRASI KASUS

  3. TetralogiFallot ANAMNESIS

  4. Identitas • Nama : An. DNH • Namaorangtua : Ny. TR • NRM : 387-54-53 • TL : 28 Maret 2013 • Usia : 11 bulan 14 hari • J.Kelamin : Perempuan • Alamat : Pekanbaru • Agama : Islam • Suku : Jawa • Tgl.Masuk : 11/3/14 • Tgl. Pemeriksaan: 12/3/14 Caretaker : Ibu (Ny. TR) • UsiaIbu: 41 tahun • Pendidikan : SMP • Pekerjaan : IbuRumahTangga • Kebangsaan : Indonesia • Pembayaran : Jamkesda

  5. KeluhanUtama Birusaatberaktivitassejakusia 1 bulan (10 bulan SMRS) (Rujukandari RS Pekanbaruuntukkateterisasi)

  6. RiwayatPenyakitSekarang

  7. RiwayatPenyakitSekarang

  8. RiwayatPenyakitSekarang

  9. RiwayatNutrisi

  10. RiwayatImunisasi • Pasienbelumpernahdiimunisasi. Ibupasienmengatakantakutmemberikanimunisasipadapasien, karenatakutmunculdemamsetelahpemberianimunisasi. RiwayatSosial • Pasiensaatinitinggalbersamakedua orang tuadankelimakakaknya. • Pasienmerupakananakkelimadari lima bersaudara.

  11. TetralogiFallot PEMERIKSAAN FISIK

  12. PemeriksaanFisik (12 Maret 2014)

  13. PemeriksaanFisik (12 Maret 2014)

  14. PemeriksaanFisik (12 Maret 2014)

  15. PemeriksaanFisik (12 Maret 2014)

  16. Diagnosis kerja

  17. Birusaatberaktivitassejakusia 1 bulan Sianosis: TipeSentral

  18. SianosisSentral

  19. Etiologi: Kardiak SianosisSentral: Kardiak • Altman, Carolyn A. Congenital heart disease (CHD) in the newborn: Presentation and screening for crticial CHD. DiaksespadaSenin, 17 Maret 2014. Diunduhdari: http://www.uptodate.com/contents/congenital-heart-disease-chd-in-the-newborn-presentation-and-screening-for-critical-chd • Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. PediatrClin N Am. 2004; 51: 999-1021.

  20. Etiologi: Kardiak SianosisSentral: Kardiak Lee JY. Clinical presentations of critical cardiac defects in the newborn: Decision making and initial management. Korean J Pediatr. 2010 Jun;53(6):669-679.

  21. SianosisSentral – Kardiak: ToF Balderas. Cyanotic Heart Disease.

  22. Diagnosis: Tetralogi of Fallot SianosisSentral – Kardiak: ToF Ebstein Anomaly Tetralogy of Fallot Toronto Notes 2012.

  23. 3:10.000 kelahiran Tetralogifallot *Minimal memenuhi2 dari 4 kriteria • Bailliard F, Anderson RH. Tetralogy of fallot. Orphanet Journal of rare Disease. 2009; 4(2): 1-10. • Bhimji S. Tetralogy of fallot. DiaksespadaSenin, 17 Maret 2014. Diunduhdari: http://emedicine.medscape.com/article/2035949-overview • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  24. Webb GD, Smallhorn JF, Therrien J, Redigton AN. Congenital Disease dalamBraunwald’s Heart Disease, 9th ed. Philadephia: Elsevier Saunders; 2012. [ebook].

  25. Etiologi • Bailliard F, Anderson RH. Tetralogy of fallot. Orphanet Journal of rare Disease. 2009; 4(2): 1-10.

  26. GambaranTetralogiFallot Bailliard F, Anderson RH. Tetralogy of fallot. Orphanet Journal of rare Disease. 2009; 4(2): 1-10. Webb GD, Smallhorn JF, Therrien J, Redigton AN. Congenital Disease dalamBraunwald’s Heart Disease, 9th ed. Philadephia: Elsevier Saunders; 2012. [ebook].

  27. Embriogenesis • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book] • Apitz C, Webb GD, Redlinton AN. Tetralogy of fallot. The Lancet. 2009; 174: 1462-71.

  28. Winn KJ. The pathogenesis of tetralogy of fallot.

  29. Bailliard F, Anderson RH. Tetralogy of fallot. Orphanet Journal of rare Disease. 2009; 4(2): 1-10.

  30. KarakteristikToF • Bailliard F, Anderson RH. Tetralogy of fallot. Orphanet Journal of rare Disease. 2009; 4(2): 1-10. • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  31. Anamnesis dan PF • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  32. Hypoxic Spell hiperpneaparoksismal (respirasidalamdancepat), iritabilitasdanprolonged crying, sianosis yang bertambah, danmenurunnyaintensitas murmur jantungkarenaabsennyaaliranantegrade yang melewatijalur RVOT. • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  33. TetralogiFallot PEMERIKSAAN PENUNJANG

  34. PemeriksaanPenunjang • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  35. PemeriksaanLaboratorium Polisitemia Tidakterdapatdefisiensibesi

  36. Pemeriksaan EKG ToF • Right Axis Deviation  padaToFsianosis • QRS normal  padapasienasianosis • Right ventricular hypertrophy • Hipertrofiventrikel bilateral • Hipertrofi atrium kanan • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  37. Hasil EKG (2 Maret 2014) Sinus rhythm, QRS rate 125x/menit, QRS normal, PR interval normal, ST-T changes (-), right axis deviation (+)

  38. Foto X-Ray ToF • Ukuranjantung normal/lebihkecildari normal • Corakanvaskularpulmonalcenderungmenurun • Lapangparu yang “hitam”  atresia pulmonal • Boot shaped appearance • Pembesaran atrium kanan & arkus aorta kanan • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  39. Hasil Rontgen (2 Maret 2014) • Jaringanlunaktidakadaemfisa • Tulang costae tidakmelebar • Tidakterdapatdeviasitrakea • Sudutkostofrenikuskanandankirilancip • Corakanbronkovaskulartidakmeningkat • CTR <50% • Tidakterdapatgambaran “boot shaped”

  40. EkokardiografiRoF • Park MK. Pediatric cardiology for practicioners, 4th ed. Philadelphia: Mosby; 2002. [e-book]

  41. Bailliard F, Anderson RH. Tetralogy of fallot. Orphanet Journal of rare Disease. 2009; 4(2): 1-10.

  42. HasilEkokardiografi (14 Februari 2014) • Atrial situssolitus • AV concordance • Normal systemic and pulmonary venous drainage • Dilated RA-RV • PFO R to L shunt • TR (PG 23 mmHg) • Severe infundibulary pulmonary stenosis (PG 69 mmHg) • Large perimembranousVSD (RL shunts) • Overriding aorta ± 50% • Confluent PA’s (RPA 6 mm, LPA 3 mm) • Left aortic arch, laminar flow across • Well-contracting ventricles, no paradoxical movement seen • No pericardial effusion seen • Colateral • Kesimpulan: • Tetralogy of Fallot, smallish LPA • PFO • Colateral

  43. Kateterisasi RFA & LFA, Plebotomi± 40 cm (12 Maret 2014) • Vena innominate (+), P-LSV C (-) • RV grafi (LAO 20, CRAN 30) • Tampakpulmonal stenosis infundibulardansupravalularberat • Overriding aorta • Confluent PA ØRPA 7,09 mm; LPA 9,01 mm • LV grafi: • Tampak VSD • Good LV Size • Aortografi: • Two ostiumcorronary artery • Left aortic arch • Tidakada PDA • Minimal kolateral • Tidakadakoartasio aorta • ØAorta desendens: 11,41 mm

  44. Kateterisasi RFA & LFA, Plebotomi± 40 cm (12 Maret 2014) • StudiHemodinamik • Half Size: 6,5 • McGoon Ratio: 1,41 • Nakata Index: 277 • Waktuprosedur: 45 menit • Waktufuloroskopi: 3,4 menit • Kesimpulan: • Tetralogy of Fallot • Confluent PA • Normal Coronary Artery • No PDA • Minimal collateral

  45. TetralogiFallot DIAGNOSIS

  46. Assessment VATER • Vertebral anomalies • Anal atresia • Cardiac defects • Tracheoesophageal fistula • Renal & radial anomalies • Limb defects • Simona P, Gluffire M. Esophageal atresia in newborns: a wide spectrum from the isolated forms to a full VACTERL phenotype. Ital J Pediatr. 2013; 39(1): 1-8.

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