Long-term Functional Health Status and Long-term Functional Health Status and Exercise Test Variables for Patients Exercise Test Variables for Patients with Pulmonary Atresia with Intact with Pulmonary Atresia with Intact Ventricular Septum: A Congenital Heart Ventricular Septum: A Congenital Heart Surgeons’ Society Study Surgeons’ Society Study Tara 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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Long-term Functional Health Status and Exercise Test Variables for Patients with Pulmonary Atresia with Intact Ventricular Septum: A Congenital Heart Surgeons.
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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
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
CaseCase
Female neonate• PAIVS
• TV Z-score -2
• RV sinusoids
• No RV-dependent circulation
What would you do?• UV, 1.5 V, BV
Repair, TV, and SurvivalRepair, TV, and Survival
UniventricularBiventricular
Death
1.5 V
%
Tricuspid Valve Z-Score
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?
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
ParticipantsParticipants
271 survivors from initial cohort
106 participants (39%)• UV (N=25)
• 1.5V (N=14)
• BV (N=63)
How do they feel?How do they feel?
Physical FunctioningPhysical Functioning*
Do
mai
n S
core
*
Normals PAIVS
* *
* *
Psychosocial and Emotional DomainsPsychosocial and Emotional Domains
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
How well can they exercise?How well can they exercise?