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Tara Karamlou, Jeffrey A. Poynter, Henry L. Walters III, Jonathan Rhodes,

Long-term Functional Health Status and Exercise Test Variables for Patients with Pulmonary Atresia with Intact Ventricular Septum: A Congenital Heart Surgeons’ Society Study. Tara Karamlou, Jeffrey A. Poynter, Henry L. Walters III, Jonathan Rhodes,

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Tara Karamlou, Jeffrey A. Poynter, Henry L. Walters III, Jonathan Rhodes,

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  1. Long-term Functional Health Status and Exercise Test Variables for Patients with Pulmonary Atresia with Intact Ventricular Septum: A Congenital Heart 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

  2. Disclosures • Study was funded by a generous grant from the Children’s Heart Foundation

  3. Case • Female neonate • PAIVS • TV Z-score -2 • RV sinusoids • No RV-dependent circulation • What would you do? • UV, 1.5 V, BV

  4. Repair, TV, and Survival Biventricular Univentricular % Death 1.5 V Tricuspid Valve Z-Score

  5. Choice of repair strategy and TV Z score …how are they related to long-term functional health status and exercise capacity?

  6. Outline • 1987 – 1997 • N=448 neonates • PAIVS • Prospective evaluation: • Perceived functional health status • Exercise capacity

  7. Participants • 271 survivors from initial cohort • 106 participants (39%) • UV (N=25) • 1.5V (N=14) • BV (N=63)

  8. How do they feel?

  9. Physical Functioning *

  10. Psychosocial and Emotional Domains NormalsPAIVS * * * * * Domain Score

  11. Repair 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

  12. How well can they exercise?

  13. Tricuspid Valve Z-Score 3 2 1 0 -1 -2 -3 -4 -5 UV BV 1.5V

  14. Peak VO2 and TV Size Peak Oxygen Consumption (% predicted)

  15. 110 100 90 80 70 60 50 40 30 Peak VO2,TV Size, and Repair Pathway Peak Oxygen Consumption (% predicted) BV 1.5V UV -5 -4 -3 -2 -1 0 1 Initial Tricuspid Valve Z-Score

  16. Case • Female neonate • PAIVS • TV Z-score -2 • RV sinusoids • No RV-dependent circulation • What would you do? • UV, 1.5 V, BV

  17. …19 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

  18. Conclusions

  19. Late 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

  20. Late Exercise Capacity • Reduced 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

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