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Tal Geva, MD Department of Cardiology Children’s Hospital Boston

Food and Drug Administration Pediatric Advisory Subcommittee Meeting February 3, 2004. Overview of Progress in Pediatric Cardiology. Tal Geva, MD Department of Cardiology Children’s Hospital Boston. Outline. Scope of congenital heart disease (CHD) Trends in CHD outcomes

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Tal Geva, MD Department of Cardiology Children’s Hospital Boston

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  1. Food and Drug Administration Pediatric Advisory Subcommittee Meeting February 3, 2004 Overview of Progress in Pediatric Cardiology Tal Geva, MD Department of Cardiology Children’s Hospital Boston

  2. Outline • Scope of congenital heart disease (CHD) • Trends in CHD outcomes • Trends in management • Trends in imaging of pediatric and adult CHD • Gaps in knowledge Tal Geva 2/04

  3. Scope of Congenital Heart Disease • AHA*: 8 per 1,000 live births (~40,000/year), ~106 Americans currently living with CHD • Hoffman and Kaplan†(review of 62 studies published since 1955): • – 4 to 50 per 1,000 live births • – Incidence depends primarily on number of small VSDs included in series • – Moderate and severe CHD: 6 per 1,000 without BAV and 19 per 1,000 with BAV * www.americanheart.org/presenter.jhtml?identifier=1477 † JACC2002;39:1890-900 Tal Geva 2/04

  4. Types of Congenital Heart Disease (median incidence per 106 live births; excluding non-stenotic BAV and silent PDA) * Excluding tiny VSDs Source: JACC2002;39:1890-900 Tal Geva 2/04

  5. Outcomes of CHD: Mortality • 1995-97: CHD contributed to 5822 deaths/year Source: Boneva et al. (CDC) Circulation 2001;103:2376-81 Tal Geva 2/04

  6. Outcomes of CHD: Mortality • 51% of deaths in infants; 7% in 1-4 years • ~19% higher mortality in blacks compared with whites Source: Boneva et al. (CDC) Circulation 2001;103:2376-81 Tal Geva 2/04

  7. Children’s Hospital Boston: CICU Mortality Tal Geva 2/04

  8. Despite an overall  in mortality, some pockets of resistance persist… Source: Sadr et al. Am J Cardiol 2000;86:577-9 Tal Geva 2/04

  9. Outcomes of CHD: Morbidity • The majority of therapeutic interventions for CHD do not lead to “cure” • Residual anatomic abnormalities • Residual hemodynamic abnormalities • Neurodevelopmental abnormalities • Social and insurability issues Tal Geva 2/04

  10. As survival of patients with CHD improved, attention shifted from getting patients out of the hospital to improving their functional, psychological, and social outcomes Tal Geva 2/04

  11. Neurodevelopmental Outcomes D-TGA Circulatory arrest v. low-flow CPB trial Wypij et al. J Thorac Cardiovsc Surg 2003;126:1397 Tal Geva 2/04

  12. Neurodevelopmental Outcomes S/p Fontan Source: Goldberg et al. J Ped 2000;137:646 Tal Geva 2/04

  13. Neurodevelopmental Outcomes Tal Geva 2/04

  14. Dunbar-Masterson et al. Circulation 2001;104:1138 Tal Geva 2/04

  15. Outcomes of CHD: Morbidity • Residual anatomic abnormalities • Residual hemodynamic abnormalities • Neurodevelopmental abnormalities • Social and insurability issues Tal Geva 2/04

  16. Even when we think treatment leads to cure… 22 year-old woman, s/p coarctation repair in infancy Tal Geva 2/04

  17. Residual hemodynamic burden is common S/p TOF repair Tal Geva 2/04

  18. Trends in Management of CHD Tal Geva 2/04

  19. Many variables account for the dramatic progress in outcomes of CHD • Better understanding of anatomy, embryology, genetics, pathophysiology, and natural history • Improved diagnosis • Support technology (e.g., cardiorespiratory support and monitoring technology in the OR and CICU, ECMO, mechanical assist devices) • Pharmacotherapy (e.g., pressors, ACE inhibitors, -blockers, NO, Sildenofil, Bosentan) • Surgical techniques • Transcatheter therapy Tal Geva 2/04

  20. Many variables account for the dramatic progress in outcomes of CHD • Better understanding of anatomy, embryology, genetics, pathophysiology, natural history • Improved diagnosis • Support technology (e.g., cardiorespiratory support and monitoring technology in the OR and CICU, ECMO, mechanical assist devices) • Pharmacotherapy (e.g., pressors, ACE inhibitors, -blockers, NO, Sildenofil, Bosentan) • Surgical techniques • Transcatheter therapy Tal Geva 2/04

  21. Trends in Surgical Management of CHD — Staged palliative approach with emphasis on Rx. of symptoms Early anatomic repair with emphasis on restoration of normal physiology • — Improved protection of vital organs • Circulatory arrest v. low-flow bypass • Improved myocardial protection • Improved O2 delivery: pH stat v.  stat • — Minimally invasive surgery • Video-assisted thoracoscopic surgery • Robotic surgery Tal Geva 2/04

  22. Coarctation repair by robotic surgery Tal Geva 2/04

  23. Many variables account for the dramatic progress in outcomes of CHD • Better understanding of anatomy, embryology, genetics, pathophysiology, natural history • Improved diagnosis • Support technology (e.g., cardiorespiratory support and monitoring technology in the OR and CICU, ECMO, mechanical assist devices) • Pharmacotherapy (e.g., pressors, ACE inhibitors,  blockers, NO, Sildenofil, Bosentan) • Surgical techniques • Transcatheter therapy Tal Geva 2/04

  24. Transcatheter Management of CHD • Valve and vessel stenosis • — balloon dilation • — stents • — radiofrequency energy • Occlusion procedures • — ASD, VSD, PDA, collaterals, fistulae • — variety of occluding devices and coils • Arrhythmia therapy (ablation) • Fetal intervention Tal Geva 2/04

  25. Cardiac Catheterization Laboratory Annual Case Volume Tal Geva 2/04

  26. Many variables account for the dramatic progress in outcomes of CHD • Better understanding of anatomy, embryology, genetics, pathophysiology, natural history • Improved diagnosis • Support technology (e.g., cardiorespiratory support and monitoring technology in the OR and CICU, ECMO, mechanical assist devices) • Pharmacotherapy (e.g., pressors, ACE inhibitors,  blockers, NO, Sildenofil, Bosentan) • Surgical techniques • Transcatheter therapy Tal Geva 2/04

  27. 1940 1950 1960 1970 1980 1990 2000 Evolution of CHD Imaging Cath X-rays Diagnostic Interventional Nuclear Radioactive tracers Thallium SPECT 99mTc PET Radium 2D Color 3D TDI BM-mode Echo Ultrasound CT X-rays 5 min/slice 400 msec/slice MRI Magnetic fields and RF Anatomy Function Tal Geva 2/04

  28. Imaging Procedures Children’s Hospital Boston, 2003 Tal Geva 2/04

  29. The excellent overall survival of patients with CHD and the associated high rate of residual anatomic and functional cardiovascular impairments result in a rapidly growing population of individuals with a life-long need for surveillance that includes cardiac imaging Tal Geva 2/04

  30. Echocardiography Laboratory Annual Case Volume Tal Geva 2/04

  31. Cardiovascular MRI Program Annual Case Volume Tal Geva 2/04

  32. Safety Issues in Pediatric Cardiac Imaging • Sedation • Inherent risks of invasive dx. procedures • Ionizing radiation exposure • Contrast agents • Radiopharmaceuticals • Auditory trauma • Pharmacological testing • Improper use of imaging technology, including an unfavorable risk/benefit ratio Tal Geva 2/04

  33. Safety Issues in Pediatric Cardiac Imaging • Sedation • Inherent risks of invasive dx. procedures • Ionizing radiation exposure (cath, CT) • Contrast agents (cath, echo, CT, MRI) • Radiopharmaceuticals (nuclear medicine) • Auditory trauma (MRI) • Pharmacological testing (cath, echo, MRI, nuclear) • Proper use of imaging technology, including a favorable risk/benefit ratio Tal Geva 2/04

  34. Tal Geva 2/04

  35. Estimated Lifetime Attributable Risk of Fatal Cancer in Pediatric CT % Risk Age at CT Examination Source: Brenner. Pediatr Radiol 2002; 32: 228 Tal Geva 2/04

  36. Brenner et al, 2003* “Above doses of 50-100mSv (protracted exposure) or 10-50 mSv (acute exposure), direct epidemiologic evidence from human populations demonstrate the exposure to ionizing radiation increases the risk of some cancer.” www.pnas.org/cgi/doi/10.1073/pnas.2235592100 Tal Geva 2/04

  37. Cancer Following Cardiac Cath in Childhood Modan et al. Int J of Epidemiology 2002;29:424 • 674 children; cath between 1950-1970 • 28.6% had >1 cath; mean age at cath 8.96 • Mean age at f/u 37.5 years • Expected number of malignancies = 4.75 • Observed number of malignancies = 11.0 • Standardized incidence ratio = 2.3 (95% CI 1.2-4.1) • Of the 11 malignancies, 4 were lymphomas and 3 were melanomas Tal Geva 2/04

  38. Summary • Advances in diagnosis and management of CHD have led to a dramatic decline in mortality (<3%) • Rapidly expanding population of patients with CHD (currently 1-2 million and growing) • Patients rarely cured; frequent anatomic and hemodynamic abnormalities requiring surveillance (e.g., imaging) •  use of transcatheter and minimally-invasive surgical interventions that rely on image-guidance Tal Geva 2/04

  39. Summary • Consequently, the number of cardiovascular imaging procedures in patients with CHD will continue to increase • Urgent need for research in pediatric cardiac imaging: – safety and efficacy of radiopharmaceuticals – cost-risk/benefit analysis of imaging strategies – minimizing exposure to ionizing radiation Tal Geva 2/04

  40. Thank You Tal Geva 2/04

  41. Nuclear Studies at CHB in 2003 N= 515 Shunt and EF (n= 3; 0.5%) Myocardial perfusion (n= 92; 17.9%) Lung perfusion (n= 420; 82%) Tal Geva 2/04

  42. CAVO-PULMONARY CONNECTION: SEPTAL DEFECT: Fenestrated Fontan 5.5% ASD Repair 10.4% BDG 6.2% VSD Repair 8.6% CAVC Repair 4.2% 11.7% 23.2% SYSTEMIC OUTFLOW: Arterial switch operation 5.2% Coarctation repair 3.5% 27.8% LVOTO 13.6% Norwood procedure 5.5% 9.4% 18.2% OTHER 8.4% Heart Tx 1.0% PULMONARY OUTFLOW: 2.0% Tetralogy of Fallot repair 7.9% 7.7% Conduit placement / revision 4.5% Other RVOT reconstruction 5.8% Pacemaker/ AICD PDA Children’s Hospital Boston 2003

  43. Tal Geva 2/04

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