Functional health status in an inception cohort of adult survivors with tetralogy of Fallot
Edward J. Hickey, Gruschen Veldtman, Timothy Bradley, Aungkana Gengsakul, Gary Webb, William G. Williams, Cedric Manlhiot, Brian W. McCrindle; The Hospital for Sick Children, Toronto, ON, Canada
Comment on this Abstract
Objective: We have recently demonstrated the late hazard for death to be very low (<0.5% per year) in adult survivors with tetralogy of Fallot. Therefore efforts to assess and improve quality of late survivorship will be more important than efforts to further improve late survival. We therefore aimed to determine the long-term functional health status in the growing population of adult survivors with tetralogy of Fallot (TOF).
Methods: Of 1693 patients diagnosed with TOF at our institution and born prior to 1984, current known vital status for 1333 yielded 840 (63%) adult survivors to age 18 years. Current cross-sectional follow-up was achieved by interview (n=707), or chart review (n=133). SF-36 health status questionnaire was completed by 396 of these survivors and compared with age- and gender-matched norms.
Results: Cardiorespiratory symptomatology was denied in more than half (55%). Chest pain (15%) was associated with late pulmonary valve replacement (PVR). Exertional dyspnoea (23%) was associated with older age at follow-up, associated cardiovascular anomalies and PVR. Palpitations (32%) were more common in older patients at the time of follow-up, women and following open surgical re-intervention.
SF-36 scores were significantly below normal for physical domains, particularly physical functioning and general health (table). However, vitality was the only psycho-social domain that was significantly abnormal.
Older age at follow-up and associated cardiovascular lesions were consistent independent predictors of worse physical scores compared to age-matched norms.
Conclusion: Despite a low late hazard for death, characterization of functional health status from an inception cohort of adult survivors with TOF shows a high prevalence of symptomatology (45%) with decrements in physical functioning.
Older patients exhibit lower scores (relative to age-matched norms), which may reflect late deterioration with advancing age or cohort effects related to historical management. Interestingly, psycho-social well-being was comparable to norms, despite the burden of re-operations and impaired physical capacity.
SF36 functional health scores of survivors (N=396)
| Scale | Z score | z-score SEM | P value | Predictors |
| Physical functioning | Physical | -0.59 | .07 | <.001 | Older age at follow-upAssociated lesionsPalliative shunt |
| Role physical | Physical | -0.15 | .05 | <.01 | Older age at follow-upAssociated lesionsRe-operation |
| Body pain | Physical | +0.20 | .05 | <.001 | Older age at follow-upAssociated lesions |
| General health | Physical | -0.84 | .07 | <.001 | Associated lesions |
| Vitality | Mental | -0.26 | .06 | <.001 | Palliative shunt |
| Social functioning | Mental | -0.06 | .05 | .27 | - |
| Role emotion | Mental | -0.01 | .05 | .86 | - |
| Mental healthy | Mental | -0.10 | .06 | .09 | - |
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