Furman D. I., Truba Ia. P., Dziuryi I. V., Goryachev A. G., Lazoryshynets V. V.

EXPERIENCE IN SURGICAL TREATMENT OF SCIMITAR SYNDROME

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About the author:

Furman D. I., Truba Ia. P., Dziuryi I. V., Goryachev A. G., Lazoryshynets V. V.

Heading:

CLINICAL AND EXPERIMENTAL MEDICINE

Type of article:

Scientific article

Annotation:

Scimitar syndrome, also known as congenital venolobar syndrome, Galash syndrome, mirror image lung syndrome, hypoplastic lung syndrome, and bronchovascular syndrome of the vena cava, is a rare congenital heart defect. In 1956, Halatz and his colleagues first used the term “Scimitar” to describe one of the forms of abnormal connection of the pulmonary veins. Due to the rarity of the disease, there are very few published studies on clinical outcomes, and treatment strategies in different age groups have not yet reached a consensus. The aim of this study is to evaluate perioperative characteristics in patients of different ages with Scimitar syndrome, as well as immediate and long-term outcomes after radical correction. Between 2000 and 2024, the Amosov National Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine performed radical correction in 16 patients with Scimitar syndrome. Given the controversy surrounding the optimal timing of surgery and the lack of consensus among the scientific community, it was decided to divide patients into two age groups. Group I consisted of 7 (44%) patients under 18, and group II consisted of 9 (56%) patients over 18. At the time of surgery, the median age in group I was 6 [4; 11] years, and in group II, 36 [35; 39] years. There were no hospital deaths in either group after radical correction of Scimitar syndrome. In patients in the first group, the postoperative period was characterized by shorter mechanical ventilation and intensive care unit and hospital stays, as well as lower exudate volumes on the 1st, 3rd, and 5th days compared to patients in the second group. The need for sympathomimetic support did not differ significantly between the groups. An uncomplicated course of the early postoperative period was noted in 6 (37.5%) patients – 3 in each group. In the remaining 10 (62.5%) patients (4 in group I and 6 in group II), a total of 14 complications were recorded. All 15 patients under observation progressed to NYHA class I heart failure during the follow-up period. A characteristic radiographic shadow in the chest suggests Scimitar syndrome, but computed tomography is necessary to select the optimal surgical strategy. Radical surgical correction of the defect demonstrates good early and long-term outcomes in patients of different age groups.

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Bibliography:

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Publication of the article:

«Bulletin of problems biology and medicine», 2025 Issue 4, 179, 212-220 pages, index UDC 616.141-007.2-053.1-089.48

DOI:

10.29254/2077-4214-2025-4-179-212-220