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CARDIAC DEFECTS: DIAGNOSIS AND TREATMENT

16 h Annual VCFSEF Meeting July 3-5 2009 Rome. CARDIAC DEFECTS: DIAGNOSIS AND TREATMENT. Bruno Marino Dipartimento di Pediatria Università di Roma “La Sapienza” bruno.marino@uniroma1.it. VCFS European Collaborative Study 1997 558 patients. J Med Genet 1997 34: 798-804 .

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CARDIAC DEFECTS: DIAGNOSIS AND TREATMENT

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  1. 16h Annual VCFSEF Meeting July 3-5 2009 Rome CARDIAC DEFECTS: DIAGNOSIS AND TREATMENT Bruno Marino Dipartimento di Pediatria Università di Roma “La Sapienza” bruno.marino@uniroma1.it

  2. VCFS European Collaborative Study 1997 558 patients J Med Genet 1997 34: 798-804

  3. VCFS: Conotruncal Heart Defects • Tetralogy of Fallot • Pulmonary Atresia • Truncus Arteriosus • Interrupted Aortic Arch - Ventricular Septal Defect - Aortic Arch Anomalies L. VanMierop, Am J Cardiol 1986 K. Momma, Am J Cardiol 1995 B. Marino, Am J Cardiol 1996 E. Goldmuntz, JACC 1998 • W.B. Strong, J Pediatr 1968 • R.M. Freedom, Circulation 1972 • Kinouchi, Pediatr Jpn 1976 • D. Young, Am J Cardiol 1980

  4. VCFS – Tetralogy of Fallot Absent Pulmonary Valve Hypoplasia/Absence of the Infundibular Septum Hypoplasia/Discontinuity of the Pulmonary Arteries Aorto-Pulmonary Collateral Right/Double/Cervical Aortic Arch K. Momma, Am J Cardiol 1995 B. Marino, Am J Cardiol 1996

  5. VCFS – Pulmonary Atresia Hypoplasia/Discontinuity of the Pulmonary Arteries Major Aorto-Pulmonary Collateral Right/Double/Cervical Aortic Arch M. Chessa, Heart 1998 M. Hofbak, Heart 1998 K. Momma, JACC 1996 M.C. Digilio, Am J Cardiol 1996

  6. VCFS – Truncus Arteriosus Hypoplasia/Discontinuity of the Pulmonary Arteries Stenosis of the Truncal Valve Right/Cervical/Interrupted Aortic Arch K. Momma, JACC 1997 B. Marino, Prog Ped Cardiol 2002

  7. VCFS – Interrupted Aortic Arch Type B (Between left carotid and left subclavian arteries) Right/Cervical Aortic Arch Hypoplasia/Absence of the Infundibular Septum B. Marino, Am J Cardiol 1999 K. Momma Cardiol Young 1999

  8. VCFS – Ventricular Septal Defect Perimembranous Septal Defect Subarterial Septal Defect Right//Double/Cervical Aortic Arch • Toscano, Eur J Pediatr 2002 • D.B. McElhinney Pediatrics 2003

  9. VCFS – Additional Cardiovascular Defects Aortic Arch Pulmonary Arteries Subclavian Arteries Aorto-Pulmonary Collaterals Infundibular Septum Pulmonary Valve

  10. VCFS – Diagnostic tool 2D, Echocardiography Angiocardiography MRI

  11. VCFS – Cardiac Surgery Issues concerning extracardiac anomalies Vascular ring K Momma, JACC 1996;27:198 Airways obstruction Laringeal web McElhinney, IJPO 2002;66:23 Broncospasm M Ackarman, JTCVS 2001;122:169 Hypertension Vasomotor instability V Shashi, AJMG 2003;1211:231 Hypotension bacterial Infections A Carotti, JTCVS 2003;126:1666 fungal Esophageal and GI anomalies Renal defects Hypocalcemia

  12. VCFS – Cardiac Surgery Specific Cardiac Patterns Specific Extracardiac Anomalies • Infections: antibacterial and antifungal prophylaxis (48h Teicoplanin + Amikacin) • Immunology: transfusion with irradiated blood products • Respiratory and Metabolic management Diagnosis: Echo, Angio MRI Surgery: Specific Techniques Specific Protocols of Diagnosis and Treatment

  13. VCFS - Surgery for Tetralogy of Fallot 306 patients G. Michielon Ann Thorac Surg 2006

  14. PA+VSD IAA Influence of chromosome 22q11.2 microdeletion on surgical outcome after treatment of tetralogy of Fallot with pulmonary atresia Carotti A. Marino B. Di Donato RM. JTCVS 2003;126:1666 Dev Disabil Res Rev 2008;14:35 VCFS – Surgery for Higher perioperative risk?

  15. IMPACT OF Del22q11, TRISOMY 21 AND OTHER GENETIC SYNDROMES ON SURGICAL OUTCOME OF CONOTRUNCAL HEART DEFECTS Michielon G, Marino B, Oricchio G, Digilio MC, Iorio F, Filippelli S, Placidi S and Di Donato R. JTCVS 2009; in press 778 patients – 91 del22q11 (11%) • In all patients with CTHD del22q11 is not a risk factor for death after surgical repair. • Postoperative long-term survival in patients with del22q11 is comparable with non-syndromic CTHDs.

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