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?

Tricuspid Valve Z-ScoreTricuspid Valve Z-Score

UVUV BV BV 1.5V 1.5V

3

-5

2

-4

-2-3

-101

Peak VO2 and TV SizePeak VO2 and TV Size

-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

CaseCase

Female neonate• PAIVS

• TV Z-score -2

• RV sinusoids

• No RV-dependent circulation

What would you do?• UV, 1.5 V, BV

……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

ConclusionsConclusions

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

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