long-term functional health status and exercise test variables for patients with pulmonary atresia...
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Long-term Functional Health Status and Exercise Long-term Functional Health Status and Exercise Test Variables for Patients with Pulmonary Test Variables for Patients with Pulmonary Atresia with Intact Ventricular Septum: A Atresia with Intact Ventricular Septum: A
Congenital Heart Surgeons’ Society StudyCongenital Heart Surgeons’ Society StudyTara Karamlou,
Jeffrey A. Poynter, Henry L. Walters III, Jonathan Rhodes,
Igor Bondarenko, Sara K. Pasquali, Stephanie M. Fuller,
Linda Lambert,
Eugene H. Blackstone, Marshall L. Jacobs, Kim Duncan, Christopher A. Caldarone,
William G. Williams, Brian W. McCrindle
AATS Annual Meeting April 30, 2012
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DisclosuresDisclosures
Study was funded by a generous Study was funded by a generous grant from the Children’s Heart grant from the Children’s Heart FoundationFoundation
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CaseCase
Female neonate• PAIVS
• TV Z-score -2
• RV sinusoids
• No RV-dependent circulation
What would you do?• UV, 1.5 V, BV
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Repair, TV, and SurvivalRepair, TV, and Survival
UniventricularBiventricular
Death
1.5 V
%
Tricuspid Valve Z-Score
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Choice of repair strategy and TV Z Choice of repair strategy and TV Z score …how are they related to score …how are they related to long-term functional health status long-term functional health status and exercise capacity?and exercise capacity?
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OutlineOutline
1987 – 19971987 – 1997
N=448 neonatesN=448 neonates
PAIVSPAIVS
Prospective evaluation:Prospective evaluation:• Perceived functional health Perceived functional health status status
• Exercise capacity Exercise capacity
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ParticipantsParticipants
271 survivors from initial cohort
106 participants (39%)• UV (N=25)
• 1.5V (N=14)
• BV (N=63)
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How do they feel?How do they feel?
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Physical FunctioningPhysical Functioning*
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Do
mai
n S
core
*
Normals PAIVS
* *
* *
Psychosocial and Emotional DomainsPsychosocial and Emotional Domains
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Repair strategy, TV, and FHSRepair strategy, TV, and FHS
Only 1 of 23 domains showed any difference
• Global general health (CHQ)
No influence of repair strategy on FHS
No influence of TV Z-score on FHS
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How well can they exercise?How well can they exercise?
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Tricuspid Valve Z-ScoreTricuspid Valve Z-Score
UVUV BV BV 1.5V 1.5V
3
-5
2
-4
-2-3
-101
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Peak VO2 and TV SizePeak VO2 and TV Size
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-5 -4 -3 -2 -1 0 1
110
100
90
80
70
60
50
40 30
Initial Tricuspid Valve Z-ScoreInitial Tricuspid Valve Z-Score
1.5V
UV
BV
Peak VO2,TV Size, and Repair PathwayPeak VO2,TV Size, and Repair Pathway
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CaseCase
Female neonate• PAIVS
• TV Z-score -2
• RV sinusoids
• No RV-dependent circulation
What would you do?• UV, 1.5 V, BV
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……19 years later19 years later
How will she feel?• Worse than normal physical
functioning
• Better than normal perceived FHS
How will she exercise?• 1V - 82%
• 1.5V – 90%
• 2V – 765
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ConclusionsConclusions
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Late FHSLate FHS
Reduced regardless of repair pathway compared to normals
Not related to the initial TV Z
Dichotomy – patients with PAIVS feel as if they are doing well despite physical impediments
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Late Exercise CapacityLate Exercise CapacityReduced regardless of repair pathway compared to normal
Borderline patients with smaller initial TV Z may be better served with 1.5 V or UV
TV Z clinically useful for predicting late aerobic capacity