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Features and results of treatment of aortic coarctation in patients with hereditary syndromic pathology

Authors: Arakelyan V.S.1, Kidakoev R.Z.-G.1, Papitashvili V.G., 1 Bukatsello R.G. 1 2

Company: 1 Bakoulev National Medical Research Center for Cardiovascular Surgery, Moscow, Russian Federation
2 I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation

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Type:  Original articles


DOI: https://doi.org/10.24022/1997-3187-2024-18-4-447-458

For citation: Arakelyan V.S., Kidakoev R.Z-G., Papitashvili V.G., Bukatsello R.G. Features and results of treatment of aortic coarctation in patients with hereditary syndromic pathology. Creative Cardiology. 2024; 18 (4): 447–458 (in Russ.). DOI: 10.24022/1997-3187-2024-18-4-447-458

Received / Accepted:  15.10.2024 / 02.12.2024

Keywords: aortic coarctation congenital heart disease Turner syndrome surgery

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Abstract

Objective: analysis of experience for identification coarctation of the aorta of combination with hereditary diseases, in particular with Turner syndrome, determination of the frequency of occurrence, characteristics of patients, influence on the tactics and outcome of surgical intervention.

Materials and methods. The analysis of patients operated on for aortic coarctation from 2010 to 2021 was carried out. Among them, 15 (4.3%) patients had an established hereditary syndrome pathology: 12 – Turner syndrome, 1 – Loys– Dietz syndrome, 1 – Down syndrome, 1 – Aarsky–Scott syndrome. The median age was 17 (from 7 to 53) years. Aortic coarctation resection with end-to-end anastomosis was performed in 73.3%, prosthetics – in 26.7%.

Results. In all patients in the study group, a hypotensive effect was achieved in the form of stabilization of blood pressure levels with complete or partial withdrawal of antihypertensive drugs, there was no difference in systolic blood pressure figures between the upper and lower extremities. The results obtained did not differ when compared with patients without syndromic pathology. The overall complication rate was 20%: chylothorax – 2 patients, bleeding – 2 cases. Patients had a higher chance of developing complications (odds ratio (OR) 9.7%; 95% confidence interval (CI) 2.7–35.2) and the need for additional surgical measures at the intraoperative stage (OR 3.8; 95% CI 1.3–11). Nevertheless, all patients were discharged in satisfactory condition. In the long-term period, adverse events and hospitalization due to the performed reconstruction were not revealed.

Conclusion. Among patients with aortic coarctation, the frequency of combination with hereditary disease is a small percentage of cases – 4.3% (Turner syndrome – 3.4%). However, such patients require precision examination for the presence of other concomitant congenital heart defects, aneurysms (dilations) of the aorta and other arteries, and the risk of hemorrhagic complications and lymphorrhea should also be considered. A comparative analysis of the results of reconstructive interventions with patients without syndromes had no differences in the frequency of adverse events, duration of hospitalization and achievement of a hypotensive effect.

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About Authors

  • Valeriy S. Arakelyan, Dr. Med. Sci., Professor, Head of the Department, Cardiovascular Surgeon; ORCID
  • Rustam Z.-G. Kidakoev, Postgraduate, Cardiovascular Surgeon; ORCID
  • Vasiliy G. Papitashvili, Dr. Med. Sci., Head of the Department, Cardiovascular Surgeon; ORCID
  • Roman G. Bukatsello, Cand. Med. Sci., Senior Researcher, Cardiovascular Surgeon; ORCID

Chief Editor

Leo A. Bockeria, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, President of Bakoulev National Medical Research Center for Cardiovascular Surgery


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