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Valve Sparing Root Replacement in Congenital Heart Disease
AATS 2012Congenital Skills
Christian Pizarro, MD
Chief, Cardiothoracic Surgery
The Nemours Cardiac Center
Alfred I. duPont Hospital for Children Aortic program
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AATS 2012
No disclosures
Congenital Skills
Aortic program
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Root aneurysm / CHD• Increasing recognition during follow up of patients
undergoing interventions for conotruncal anomalies
• Surgical indication not well defined due to unknown natural history
• risk of rupture or dissection
Congenital Skills AATS 2012
• risk of rupture or dissection
• Multiple challenges
– Technically complex
– Multiple redo
– Challenging physiology
– Surgical risk?
Aortic program
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Congenital Skills AATS 2012
JACC 2003;42:533-40
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Congenital Skills AATS 2012
Schwartz, M. L. Circulation 2004;110:II-128-II-132
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MechanismCongenital Skills AATS 2012
Aortic program
differential expression of cell adhesion and ECM
structural proteins between BAV and TAV
Developmental defects in cellular-cellular interactions between SMC and
adhesion molecules may provide unique molecular pathways for BAV and
TAV associated TAA
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Preoperative Imaging
Echocardiogram
CT angiogram
MRI/MRA
Congenital Skills AATS 2012
Trend of growth
Associated valvar issues
Involvement of adjacent
structures
Aortic program
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Case selection
Congenital Skills AATS 2012
Aortic program
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Valve selection
• Competency
• Mechanism of
regurgitation
Congenital Skills AATS 2012
regurgitation
• Anatomy / Integrity
• Annular dilatation
• Associated lesions
• Ventricular function
Aortic program
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Diagnosis
HLHS 4
BAV/Coa 6
Congenital Skills AATS 2012
TGA 3
DORV 1
TOF 1
Aortic program
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Procedures
VSARR 7
Bentall 4
Congenital Skills AATS 2012
Bentall 4
Tailoring 2
Ross/Tailoring 2
Aortic program
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Associated procedures
• Arch repair 4
• LPA plasty 2
• Konno 2
Congenital Skills AATS 2012
• Konno 2
• MPA replacement 1
• PV replacement 1
• Ventricular aneurysm repair 1
Aortic program
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Clinical dataAge (yrs) 11 (5-18)
Ao root z-score 6.2 ( 4.4 - 13)
Asc Ao z-score 6.4 ( 5.4 – 9.5)
AXC (min) 105 (62-172)
Congenital Skills AATS 2012
AXC (min) 105 (62-172)
CPB (min) 194 (103-274)
ICU stay (days) 2 (1 – 4)
Hospital stay (days) 7 ( 5 – 12)
No operative mortality
At a median follow up of 4.6 (0.7- 5.7) yrs
all patients remain free of aortic insufficiency
Aortic program
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Perfusion
• Moderate hypothermia unless DHCA used
• DHCA in 5/15 (median 22 min [14-45])
• Pulmonary venous cannulation in Fontan (maintain ventilation)
Congenital Skills AATS 2012
• Pulmonary venous cannulation in Fontan (maintain ventilation)
• Crystalloid cardioplegia
• Cell saver
• Fresh whole blood (median 400 cc[300-550])
Aortic program
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HLHS
• Single ventricle physiology
• Multiple reoperations
• Myocardial preservation
• Younger patients
• Compression of the Fontan
Congenital Skills AATS 2012
• Compression of the Fontan circuit
• Simpler coronary reimplantation
• Sub pulmonary conus
• Preservation of low PVR– CPB time
– Blood products
Aortic program
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AATS 2012Congenital Skills
Aortic program
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Transposition of the great arteries
• Posterior location of the aorta– Dissection
– Hemostasis
• Difficult assessment of the valve sparing procedure
Congenital Skills AATS 2012
valve sparing procedure
• Coronary anatomy (imaging)
• Pulmonary artery reconstruction
• Older patients
Aortic program
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Congenital Skills AATS 2012
Sections of aorta show accumulation of myxoid material (extracellular ground substance) within the media.
Aortic program
[H&E, original magnification 40X (left) and 100X (right)]
Elastin stain of aorta shows disruption and loss of
elastic fibers within the media.
[Elastin stain, original magnification 100X]
Aorta stained with trichrome stain (left) and smooth muscle actin immunohistochemistry (right)
shows expanded zones of extracellular ground substance and disruption and loss of smooth muscle
cells within the media. [Trichrome stain (left), SMA (right), original magnifications 100X]
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Summary• Aortic root dilatation after repair of CHD is becoming
increasingly recognized.
• Preliminary data suggest a common histological lesion
• In the absence of natural history data and understanding of the
surgical risks the indication and timing of surgery remains
difficult.
Congenital Skills AATS 2012
difficult.
• Indications for surgery are usually influenced by coexisting
issues (valve incompetence, coarctation, pulmonary artery
obstruction).
• Despite the high complexity of these repairs surgical and
functional outcomes are excellent.
Aortic program
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Summary• Developmental defects in cellular-cellular interactions between
SMC and adhesion molecules may provide unique molecular
pathways for BAV and TAV associated TAA.
• Is the histological lesion similar to aneurysmal dx in the adult?
• Is there a common mechanism? Or just the common expression?expression?
• Can we anticipate who will develop aneursymal disease following repair of CHD?
• Is this related to the presence of a BV?
• Does the mechanism vary depending on the presence of bicuspid semilunar valve?
Aortic program