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Adult CHD Muscat 2012
Adult Congenital Heart Diseases
Surgical Considerations
Samuel Weinstein MD MBA Director, Pediatric Cardiothoracic Surgery
Director, Adult Congenital Cardiac Surgery Children’s Hospital at Montefiore
Montefiore Medical Center
New York, NY
Adult CHD Muscat 2012
•*Estimated Numbers
•Warnes CA et al. J Am Coll Cardiol 2001; 37: 1170-1175
•Williams RG et al. J Am Coll Cardiol 2006; 47: 701-707
Incidence: 4 to 50 per 1,000 live births
Overall CHD mortality 3-4%
5% per year
Adult CHD Muscat 2012
Challenges for a growing population
• Who is going to take care of these patients
• Where is the best setting for this care
• Patients have a poor understanding of their heart
disease and are lost to follow-up
• Transition to adult medicine can be challenging
• Complex CHD is not “cured”
• Really a chronic effects of disease
Adult CHD Muscat 2012
• Chronic volume overload
•Left to right shunt
•Right to left shunt
• Pressure overload
•valvular disease
•subvalvular disease (LVOTO)
• systemic hypertension
• Cyanosis
• Myocardial damage due to CPB (mulitple)
• Atrial and ventricular arrhythmias
• Pulmonary Hypertension
• Coronary artery disease
• Obesity, IDDM, Pulmonary, etc.
Chronic Sequelae of ACHD/CHF
Adult CHD Muscat 2012
? Interventionalist
? Intensivist
?Anesthesiologist
? ICU/floor nursing
? Perfusion
Adult CHD Muscat 2012
Post-Operative CHF in adolescents and adults
Norozi et al, Am J Cardiology 2006;15: 1238
Adult CHD Muscat 2012
Operative Strategy
• Percutaneous vs hybrid vs. open repair
• Open Heart Surgery
– Ventricular Venting/Collateral Flow
– Cardioplegia
– Temperature
– Cannulation
Adult CHD Muscat 2012
SVC
TV
ASD 2D and 3D echocardiography
Adult CHD Muscat 2012
Ostium
Secundum
ASD
Septum
Primum
Coronary
Sinus
Pericardial
Patch
Closure
ASD Closure
CS
Adult CHD Muscat 2012
ASD device closure
Adult CHD Muscat 2012
ASD Device in Left Atrium
Adult CHD Muscat 2012
RMPV
ASD
Right
Atrium
PFO
SVC
RUPV
RMPV
Sinus Venosus ASD
Adult CHD Muscat 2012
Reoperations
Incidence in Acquired Adult Cardiac Surgery 10-20%
Incidence in Adult Congenital Cardiac Surgery >75%
Adult CHD Muscat 2012
Reoperations
Adult CHD Muscat 2012
Peripheral
Cannulation
Axillary Artery Fem-Fem w/distal limb
Femoral Artery
Superior Vena
Cava via IJ for
IVC occlusion
Adult CHD Muscat 2012
Scar on the inside, too!
Adult CHD Muscat 2012
Tetralogy of Fallot
Adult CHD Muscat 2012
Dilated RVOT
Wide Open PI in
Repaired TOF
Dilated Right Ventricle
Severe Volume
Overload
Adherence to back of
Sternum
Adult CHD Muscat 2012
Pulmonary Valve Replacement
Main PA
Main PA
Contegra
Right Ventricle
Graft in Situ
Beating Heart PVR
Adult CHD Muscat 2012
Percutaneous PVR
Adult CHD Muscat 2012
Percutaneous PVR
Adult CHD Muscat 2012
Coarctation of the Aorta
Adult CHD Muscat 2012
Coarctation of the Aorta
Adult CHD Muscat 2012
Repaired Coarctation Survival
Cohen et al. Circulation 1989; 80: 840-5
72% survival at 30 years
Essential Hypertension
Present as adults
Re-coarctation
Primary Presentation
Adult CHD Muscat 2012
Coarctation of the Aorta
Adult CHD Muscat 2012
Descending Aorta
Coarct
Isthmus
Left Subclavian Artery
Adult Coarct Repair Collaterals
Mobilization
Cardiopulmonary Bypass
Collateral
Adult CHD Muscat 2012
Coarctation of the Aorta
Percutaneous Stent Placement
Coarct Segment
Adult CHD Muscat 2012
Fontan Revision and Cryoablation
Classic Fontan Sequelae
– Right Atrial Dilation
• Stasis of Flow
• Decreased Cardiac Output
– Atrial Arrhythmias
• A Flutter
• A Fibrillation
– Pulmonary Vein Stenosis
– AVM’s
Adult CHD Muscat 2012
Fontan Revision and Cryoablation
Adult CHD Muscat 2012
Fontan Revision and
Cryoablation
Adult CHD Muscat 2012
Conclusions
• Complicated subset of patients
• Require multidisciplinary input
• No one correct way
• Consider all surgical and interventional options
• Not just bigger patients
– Co Morbidities
– Non childhood illnesses (CAD, Pulmonary, Renal, etc)
• With careful planning and in the appropriate
setting ACHD procedures can significantly
improve quality and quantity of life
Adult CHD Muscat 2012
Thank You
• Comments?
• Questions?
Adult CHD Muscat 2012
Complete Transposition of the Great
Arteries (D-TGA)
• Separate
PARRALLEL
circuits that require a
communication for
survival
• PDA
• ASD
• VSD
Brickner et al. NEJM 2000; 342: 334-342
Adult CHD Muscat 2012
Arterial Switch Repair Atrial Switch Repair
Adult CHD Muscat 2012
Late Complications of Atrial Switch Roos-Hesselink et al. Eur Heart J 2004; 25: 1264-70
Morphologic RV (systemic ventricle) failure
Systemic AV valve regurgitation (tricuspid valve)
Baffle Obstruction or Leak
At 15 years, only 50% have avoided sig morbidity
Adult CHD Muscat 2012
RV LV
Ao PA
TGA post Mustard Baffle
RV
LV
Adult CHD Muscat 2012
TGA s/p Mustard, poor RV fx, pre-
transplant
Adult CHD Muscat 2012
Venous
Confluence Left Atrium
Adult CHD Muscat 2012 Boneva, R. S. et al. Circulation 2001;103:2376-2381
Age-adjusted death rate due to CHDs by year of death, United States, 1979-1997
Mortality Declined by 40% from 1979 through 1997
Survival of children born with CHD is now
approximately 85-90%
Adult CHD Muscat 2012
Low complexity ACHD
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Patent Ductus Arteriosus (PDA)
Mild Pulmonary Stenosis
Oechslin et al. Am J Card 2000; 86: 1111-1116
Moderate Complexity ACHD
Coarctation of the aorta
Ebstein’s anomaly
AV canal
Tetralogy of Fallot
High Complexity ACHD
Transposition of the great arteries
Single ventricle
Fontan procedure
Eisenmenger syndrome
Adult CHD Muscat 2012
Left PAPVR
Main PA
Aorta
Adult CHD Muscat 2012
Post-Operative CHF in adolescents and adults
Norozi et al, Am J Cardiology 2006;15: 1238
At 40 Y of age
> 2/3 Fallot with CHF
At 40 Y
25% ASD
VSD, PDA
Adult CHD Muscat 2012
1 Million
Incidence of Adult Diseases in General Population
Adult CHD Muscat 2012
Coarctation of the Aorta
Percutaneous Stent Placement
Coarct Segment
Adult CHD Muscat 2012
Atrial Septal Defect
Asymptomatic
• Murmur
• Abnormal EKG
• ECHO
Symptomatic
• Exercise intolerance
• Paradoxical embolus
• Atrial arrhythmias
Lai et al. Echocardiography in Pediatric and Congenital Heart Disease 2009
Adult CHD Muscat 2012
Case
• 26 year old male with a history of Tetralogy of
Fallot repair at age 5. Now presents with PI, RHF.
• Most common form of cyanotic
congenital heart disease
• 4 features
• Right ventricular outflow tract
obstruction
• Overriding of the aorta
• VSD
• Right ventricular hypertrophy
• Features of Repair
Relief of right ventricular
outflow tract obstruction
• VSD Closure
• Takedown of any shunts
Success 85% at 30 year F/u
Adult CHD Muscat 2012
PAPVR Repair
Left Lung
Ascending Vertical Vein
Adult CHD Muscat 2012
Age of the Patient Population
50%
Adult CHD Muscat 2012
Common Procedures in Adult CHD
• Fallot: PV replacement
• Valved conduit failure
• Aortic valve, root or LVOTO
• Primary Disease (ASD, PAPVR)
• Residual lesions (VSD, obstruction, Pulmonary Vein Stenosis)
• Fontan Revision
and Cryoablation
• Transplantation
Adult CHD Muscat 2012
Types of Congenital Heart Disease in
Montefiore ACHD Program
ASD, VSD, PDA
Coarctation
TOF, LVOTO
Ebstein’s, AVC, Conduits
Single Ventricle,
TGA,Transplantation