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Health related quality of life in adults after late repair of tetralogy of fallot: experience from a low-middle income country

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Abstract

Purpose

Without neonatal screening in low middle-income countries like Pakistan, Tetralogy of Fallot (TOF) is a congenital heart disease which frequently remains untreated beyond infancy. The purpose of this study is to determine and assess outcomes and health related quality of life (HRQOL) in patients who undergo complete repair of TOF as adults.

Methods

56 patients who underwent complete TOF repair after 16 years of age were included. Patient data was collected via retrospective chart review, and a semi structured interview along with Short-Form 36 (SF-36) questionnaire were used to assess HRQOL.

Results

66.1% of patients were male with the mean age at surgery of 22.3 ± 6.00. All patients had a post-operative NYHA Classification of I or II, 94.6% had an ejection fraction of ≥ 50% and 28.6% showed small residual lesions in follow-up echocardiograms. 32.1% of patients suffered post-operative morbidity. For the quantitative assessment using SF-36 scores, patients showed good scores of median 95 (65–100). A major cause of delay to treatment was lack of consensus between treatments offered by doctors in different parts of Pakistan. There was a pattern of ‘inability to fit in’ among patients who had had late TOF repair, despite self- reported improved HRQOL.

Conclusion

Our results indicate that even with a delayed diagnosis, surgical repair of TOF produces good functional results. However, these patients face significant psychosocial issues. While early diagnosis remains the ultimate goal, patients undergoing late repair should be managed in more holistic manner with attention to psychological impact of the disease as well.

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Funding

This study was partially funded by the Department of Surgery at the Aga Khan University Hospital.

Author information

Author notes

  1. Maha Inam and Laila Akbar Ladak have contributed equally to this work.

Authors and Affiliations

  1. Medical College, Aga Khan University Hospital, Karachi, Pakistan

    Maha Inam & Maarij Malik

  2. School of Nursing and Midwifery, Aga Khan University Hospital, Karachi, Pakistan

    Laila Akbar Ladak

  3. Department of Surgery, Howard University College of Medicine, Washington, DC, USA

    Mahin Janjua

  4. Department of Pediatrics, Aga Khan University Hospital, Karachi, Pakistan

    Fatima Ali

  5. Department of Cardiac Surgery, Rawalpindi Institute of Cardiology, Rawalpindi, Pakistan

    Mahim Akmal Malik

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  1. Maha Inam
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  2. Laila Akbar Ladak
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  4. Maarij Malik
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  5. Fatima Ali
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Contributions

MI: conducted the data collection, data analysis and drafted and revised the manuscript. LAL: contributed to the study design, conducted the data analysis and provided critical feedback on the manuscript drafts. MJ: conducted the data collection, data analysis and drafted the manuscript. MM: conducted the data collection and drafted the manuscript. FA: conducted the data collection, mentored in data analysis and provided critical feedback on the manuscript drafts. MM: conceived the study, contributed to the study design, mentored in data analysis, provided critical feedback on the manuscript drafts and revised the manuscript. All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work. MI and LAL: have shared co-first authorship.

Corresponding author

Correspondence to Mahim Akmal Malik.

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Competing interests

The authors have no relevant financial or non-financial interests to disclose.

Ethical approval

Approval was obtained for this study from the Ethical Review Committee at the Aga Khan University Hospital, Karachi, Pakistan (2019-0872-2829).

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Inam, M., Ladak, L.A., Janjua, M. et al. Health related quality of life in adults after late repair of tetralogy of fallot: experience from a low-middle income country. Qual Life Res 32, 3063–3074 (2023). https://doi.org/10.1007/s11136-023-03453-7

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