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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Presentation transcript:

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

Disclosures Study was funded by a generous grant from the Childrens Heart Foundation

Case 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 Survival Univentricular Biventricular Death 1.5 V % Tricuspid Valve Z-Score

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

Outline 1987 – 1997 N=448 neonates PAIVS Prospective evaluation: Perceived functional health statusPerceived functional health status Exercise capacityExercise capacity

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

How do they feel?

Physical Functioning *

Domain Score * Normals PAIVS * * * * Psychosocial and Emotional Domains

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

How well can they exercise?

Tricuspid Valve Z-Score UV BV 1.5V

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

Initial Tricuspid Valve Z-Score Peak Oxygen Consumption (% predicted) 1.5V UV BV Peak VO2,TV Size, and Repair Pathway

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

…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

Conclusions

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

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