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Published: 2023-06-20
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DOI: https://doi.org/10.52852/tcncyh.v167i6.1716
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Vol. 167 No. 6 (2023)
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Các bài báo
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Hằng, N. T. T., Trường, N. L. T., Mai, N. T., Quang, L. H., Anh, N. T. H., & Vân, Đặng T. H. (2023). 26. Clinical syndrome, echocardiography and surgical outcomes of tetralogy of fallot with absent pulmonary valve syndrome at the National Children’s Hospital. Journal of Medical Research, 167(6), 221-229. https://doi.org/10.52852/tcncyh.v167i6.1716

26. Clinical syndrome, echocardiography and surgical outcomes of tetralogy of fallot with absent pulmonary valve syndrome at the National Children’s Hospital

Nguyen Thi Thuy Hang, Nguyen Ly Thinh Truong, Nguyen Tuan Mai, Le Hong Quang, Nguyen Thi Hai Anh, Dang Thi Hai Van

Main Article Content

Abstract

This is a review of 27 patients with tetralogy of Fallot with absent pulmonary valve syndrome (TOFAPV) who  underwent surgical treatment between 2015 and 2023 at National Children’s hospital. The median age at surgery was 33 days (IQR 24-120 days) and the mean weight were 4,4 ± 1,4 kg (range, 1,9 to 8,5 kg). The patient presented with symptoms of preoperative congestive heart failure (70,4%), respiratory distress (85,2%) and preoperative mechanical ventilatory support (25,9%). After 1 month of surgery, no patient showed heart failure and severe respiratory failure. Wheezing symptoms decreased from 63% (pre-operation) to 13% (post-operation) (p<0.05). On echocardiography, 96,3% patients had right or left pulmonary artery (PA) dilatation. Mean preoperative right PA zscore was 6,16 ± 1,92 SD and left PA zscore was 5,60 ± 1,76 SD . Mean postoperative right and left PA zscore were 1,0 ± 1,58 và 1,48± 1,32, respectively. The proportion of moderate and severe pulmonary regurgitation (PR) decreased from 100% (pre-operation) to 40,7% (post-operation). Moderate and severe pulmonary stenosis rate decreased from 96,3% (pre-operation) to 8,7% (post-operation). The average follow-up time was 34 ± 27,2 months (2- 82 months). Operative mortality was 14,8%. Overall survival at 5 years was 80%. The 2 years and 5 years freedom from reoperations were 92,6% and 75,8%, respectively.

Article Details

Keywords

Tetralogy of Fallot with absent pulmonary valve syndrome, echocardiography

References

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