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Quality of Life is Diminished in Patients with Tetralogy of Fallot with Mild Residual Disease: A Comparison of Tetralogy of Fallot and Isolated Valvar Pulmonary Stenosis

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Abstract

The objective of this study is to compare quality of life (QOL) in patients with mild pulmonary insufficiency (PI) after Tetralogy of Fallot (TOF) repair or after balloon dilation for isolated valvar pulmonary stenosis (VPS). A cross-sectional study of patients with TOF (n = 12) and VPS (n = 19), ages 8–18 years, who underwent cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) was conducted. Patients with genetic syndromes were excluded. The groups were matched by severity and duration of PI using propensity scores. PI was greater than mild if the regurgitant fraction by CMR was >20%. Health status and QOL assessment included Child Health Questionnaire Child Self-Report (CHQ-CF87), Child Health Questionnaire Parent Report (CHQ-PF50), and Pediatric Cardiac QOL Instrument (parent and patient). QOL scores were compared between groups. Due to propensity matching, the groups had, at worst, mild PI and normal right ventricular ejection fraction on CMR. Parental perception of QOL was significantly worse in TOF as compared to VPS in the domains of general perception of health (P = 0.03), physical functioning (PF; P = 0.004), and family cohesion (P = 0.048). There were no differences in self-reported QOL between groups. There was no association between QOL and right ventricular function on CMR or percent-predicted maximal oxygen consumption on CPET in both groups. Parent-perceived QOL, in the domains of general health perception, PF, and family cohesion, is significantly reduced in patients with TOF with mild residual disease suggesting that the psychosocial impact of congenital heart disease may be significant even with successful repair and satisfactory medical status.

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Abbreviations

QOL:

Quality of life

TOF:

Tetralogy of Fallot

VPS:

Isolated valvar pulmonary stenosis

CMR:

Cardiac magnetic resonance

CPET:

Cardiopulmonary exercise test

RF:

Regurgitant fraction

PI:

Pulmonary insufficiency

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Acknowledgements

This study was supported by NHLBI HL7473 (Goldmuntz), CHOP Cardiac Center Grant (Mercer-Rosa), NIH U01HL098153 (Goldmuntz), NIH K01HL125521 (Mercer-Rosa), and NIH 5T32HL007915 (Bhatt).

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Authors and Affiliations

  1. Division of Cardiology, The Children’s Hospital of Philadelphia, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA

    Shivani M. Bhatt, Elizabeth Goldmuntz & Laura Mercer-Rosa

  2. Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Amy Cassedy

  3. Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Bradley S. Marino

Authors
  1. Shivani M. Bhatt
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  2. Elizabeth Goldmuntz
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  3. Amy Cassedy
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  4. Bradley S. Marino
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  5. Laura Mercer-Rosa
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Correspondence to Shivani M. Bhatt.

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Research Involving Human Participants and/or Animals

All procedures performed in studies involving human participants were in accordance with the Ethical Standards of the Institutional and/or National Research Committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Bhatt, S.M., Goldmuntz, E., Cassedy, A. et al. Quality of Life is Diminished in Patients with Tetralogy of Fallot with Mild Residual Disease: A Comparison of Tetralogy of Fallot and Isolated Valvar Pulmonary Stenosis. Pediatr Cardiol 38, 1645–1653 (2017). https://doi.org/10.1007/s00246-017-1709-3

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  • DOI: https://doi.org/10.1007/s00246-017-1709-3

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