Tal Geva, MD Tal Geva, MD Department of Department of Cardiology Cardiology Children’s Hospital Children’s Hospital Boston Boston Overview of Progress in Overview of Progress in Pediatric Cardiology Pediatric Cardiology Food and Drug Administration Food and Drug Administration Pediatric Advisory Subcommittee Meeting Pediatric Advisory Subcommittee Meeting February 3, 2004 February 3, 2004
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Tal Geva, MDTal Geva, MDDepartment of CardiologyDepartment of CardiologyChildren’s Hospital BostonChildren’s Hospital Boston
Overview of Progress in Overview of Progress in Pediatric CardiologyPediatric Cardiology
Food and Drug AdministrationFood and Drug AdministrationPediatric Advisory Subcommittee MeetingPediatric Advisory Subcommittee Meeting
February 3, 2004February 3, 2004
OutlineOutline
• Scope of congenital heart disease (CHD)Scope of congenital heart disease (CHD)
• Trends in CHD outcomesTrends in CHD outcomes
• Trends in managementTrends in management
• Trends in imaging of pediatric and adult CHDTrends in imaging of pediatric and adult CHD
• Gaps in knowledgeGaps in knowledge
Tal Geva 2/04
Scope of Congenital Heart DiseaseScope of Congenital Heart Disease
• AHA*: 8 per 1,000 live births (~40,000/year), AHA*: 8 per 1,000 live births (~40,000/year),
~10~1066 Americans currently living with CHD Americans currently living with CHD
• Hoffman and KaplanHoffman and Kaplan†† (review of 62 studies (review of 62 studies
published since 1955)published since 1955)::– – 4 to 50 per 1,000 live births4 to 50 per 1,000 live births– – Incidence depends primarily on number Incidence depends primarily on number
of small VSDs included in seriesof small VSDs included in series
– – Moderate and severe CHD: 6 per 1,000 Moderate and severe CHD: 6 per 1,000 without BAV and 19 per 1,000 with BAVwithout BAV and 19 per 1,000 with BAV
* www.americanheart.org/presenter.jhtml?identifier=1477* www.americanheart.org/presenter.jhtml?identifier=1477† † JACCJACC 2002;39:1890-9002002;39:1890-900 Tal Geva 2/04
Types of Congenital Heart DiseaseTypes of Congenital Heart Disease(median incidence per 10(median incidence per 1066 live births; excluding non-stenotic BAV and silent PDA) live births; excluding non-stenotic BAV and silent PDA)
• Social and insurability issuesSocial and insurability issues
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As survival of patients with CHD improved, As survival of patients with CHD improved, attention shifted from getting patients out of attention shifted from getting patients out of the hospital to improving their functional, the hospital to improving their functional, psychological, and social outcomes psychological, and social outcomes
• Social and insurability issuesSocial and insurability issues
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Even when we think treatment leads to cure…Even when we think treatment leads to cure…
22 year-old woman, s/p coarctation repair in infancy22 year-old woman, s/p coarctation repair in infancy
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Residual hemodynamic burden is common
S/p TOF repair
QuickTime™ and aSorenson Video 3 decompressorare needed to see this picture.
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Trends in Management of CHDTrends in Management of CHD
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Many variables account for the dramatic Many variables account for the dramatic progress in outcomes of CHDprogress in outcomes of CHD
• Better understanding of anatomy, embryology, Better understanding of anatomy, embryology, genetics, pathophysiology, and natural genetics, pathophysiology, and natural
historyhistory
• Improved diagnosisImproved diagnosis
• Support technology (e.g., cardiorespiratory Support technology (e.g., cardiorespiratory support and monitoring technology in the OR support and monitoring technology in the OR
and CICU, ECMO, mechanical assist and CICU, ECMO, mechanical assist devices)devices)
• Pharmacotherapy (e.g., pressors, ACE Pharmacotherapy (e.g., pressors, ACE inhibitors, inhibitors, -blockers, NO, Sildenofil, -blockers, NO, Sildenofil,
Bosentan)Bosentan)
• Surgical techniquesSurgical techniques
• Transcatheter therapyTranscatheter therapy
Tal Geva 2/04
Many variables account for the dramatic Many variables account for the dramatic progress in outcomes of CHDprogress in outcomes of CHD
• Better understanding of anatomy, embryology, Better understanding of anatomy, embryology, genetics, pathophysiology, natural historygenetics, pathophysiology, natural history
• Improved diagnosisImproved diagnosis
• Support technology (e.g., cardiorespiratory Support technology (e.g., cardiorespiratory support and monitoring technology in the OR support and monitoring technology in the OR
and CICU, ECMO, mechanical assist and CICU, ECMO, mechanical assist devices)devices)
• Pharmacotherapy (e.g., pressors, ACE Pharmacotherapy (e.g., pressors, ACE inhibitors, inhibitors, -blockers, NO, Sildenofil, -blockers, NO, Sildenofil,
Bosentan)Bosentan)
• Surgical techniquesSurgical techniques
• Transcatheter therapyTranscatheter therapy
Tal Geva 2/04
Trends in Surgical Management of CHDTrends in Surgical Management of CHD
— — Staged palliative Staged palliative approach with approach with emphasis on Rx. of emphasis on Rx. of symptomssymptoms
Early anatomic repair Early anatomic repair with emphasis on with emphasis on restoration of normal restoration of normal physiologyphysiology
— — Improved protection of vital organsImproved protection of vital organs• Circulatory arrest v. low-flow bypassCirculatory arrest v. low-flow bypass• Improved myocardial protectionImproved myocardial protection• Improved OImproved O22 delivery: pH stat v. delivery: pH stat v. stat stat
Coarctation repair by Coarctation repair by robotic surgeryrobotic surgeryQuickTime™ and a
YUV420 codec decompressorare needed to see this picture.
Tal Geva 2/04
Tal Geva 2/04
Many variables account for the dramatic Many variables account for the dramatic progress in outcomes of CHDprogress in outcomes of CHD
• Better understanding of anatomy, embryology, Better understanding of anatomy, embryology, genetics, pathophysiology, natural historygenetics, pathophysiology, natural history
• Improved diagnosisImproved diagnosis
• Support technology (e.g., cardiorespiratory Support technology (e.g., cardiorespiratory support and monitoring technology in the OR support and monitoring technology in the OR and CICU, ECMO, mechanical assist and CICU, ECMO, mechanical assist
devices)devices)
• Pharmacotherapy (e.g., pressors, ACE Pharmacotherapy (e.g., pressors, ACE inhibitors, inhibitors, blockers, NO, Sildenofil, blockers, NO, Sildenofil,
Bosentan)Bosentan)
• Surgical techniquesSurgical techniques
• Transcatheter therapyTranscatheter therapy
Tal Geva 2/04
Transcatheter Management of CHDTranscatheter Management of CHD
• Valve and vessel stenosisValve and vessel stenosis— — balloon dilationballoon dilation— — stentsstents— — radiofrequency energyradiofrequency energy
• Occlusion proceduresOcclusion procedures— — ASD, VSD, PDA, collaterals, fistulaeASD, VSD, PDA, collaterals, fistulae— — variety of occluding devices and coilsvariety of occluding devices and coils
Many variables account for the dramatic Many variables account for the dramatic progress in outcomes of CHDprogress in outcomes of CHD
• Better understanding of anatomy, embryology, Better understanding of anatomy, embryology, genetics, pathophysiology, natural historygenetics, pathophysiology, natural history
• Improved diagnosisImproved diagnosis
• Support technology (e.g., cardiorespiratory Support technology (e.g., cardiorespiratory support and monitoring technology in the OR support and monitoring technology in the OR and CICU, ECMO, mechanical assist and CICU, ECMO, mechanical assist
devices)devices)
• Pharmacotherapy (e.g., pressors, ACE Pharmacotherapy (e.g., pressors, ACE inhibitors, inhibitors, blockers, NO, Sildenofil, blockers, NO, Sildenofil,
Bosentan)Bosentan)
• Surgical techniquesSurgical techniques
• Transcatheter therapyTranscatheter therapy
EchoEchoUltrasoundUltrasound
BBM-modeM-mode 2D2D ColorColor 3D TDI3D TDI
AnatomyAnatomy FunctionFunctionMRIMRIMagnetic fields and RFMagnetic fields and RF
The excellent overall survival of patients The excellent overall survival of patients with CHD and the associated high rate of with CHD and the associated high rate of residual anatomic and functional residual anatomic and functional cardiovascular impairments result in a cardiovascular impairments result in a rapidly growing population of individuals rapidly growing population of individuals with a life-long need for surveillance that with a life-long need for surveillance that includes cardiac imagingincludes cardiac imaging
Cancer Following Cardiac Cath in ChildhoodCancer Following Cardiac Cath in ChildhoodModan et al. Int J Modan et al. Int J ofof Epidemiology 2002;29:424 Epidemiology 2002;29:424
• 674 children; cath between 1950-1970674 children; cath between 1950-1970• 28.6% had >1 cath; mean age at cath 8.9628.6% had >1 cath; mean age at cath 8.96• Mean age at f/u 37.5 yearsMean age at f/u 37.5 years• Expected number of malignancies = 4.75Expected number of malignancies = 4.75• Observed number of malignancies = 11.0Observed number of malignancies = 11.0• Standardized incidence ratio = 2.3Standardized incidence ratio = 2.3
(95% CI (95% CI 1.2-4.1)1.2-4.1)• Of the 11 malignancies, 4 were lymphomas Of the 11 malignancies, 4 were lymphomas
and 3 were melanomasand 3 were melanomas
SummarySummary
• 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-guidanceTal Geva 2/04
SummarySummary
• 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
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Tal Geva 2/04
Thank YouThank You
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Nuclear Studies at CHB in 2003Nuclear Studies at CHB in 2003N= 515N= 515