SURGICAL TREATMENT OF CONGENITAL SUPRAVALVULAR AORTIC STENOSIS: CLINICAL CASE
Abstract
The study aims to show the results of efficient surgical treatment of congenital supravalvular aortic stenosis in a patient with Williams-Beuren syndrome. Materials and methods. The article presents the results of
diagnosis and surgical treatment of rare clinical and diagnostic case of supravalvular aortic stenosis with William-Beuren syndrome. In the course of the study, anamnesis of the disease, the dynamics of symptoms and the results of surgical treatment were analyzed. Results. A child was diagnosed with valvular pulmonary stenosis at birth. A balloon pulmonary valvuloplasty was performed on the child at the age of 3 and 5 months. According to the
results of the fragment analysis of genomic DNA probe, microdeletions in the 7q11.23 area were found, which clinically complies with Williams-Beuren syndrome. At the age of 14 months the child was examined with increase
in velocity indicators in sinotubular junction area which worsened at 18 months. The child was hospitalized with the diagnosis of supravalvular aortic stenosis, and received ascending aorta plasty according to Doty. No
indications for re-intervention were found according to the results of the examination when the child was 4.6 years old. Surgical treatment shows efficient immediate medium-term results. Aortic root surgery with two or three sinuses makes it possible to avoid aortic restenosis in post-surgery period.
About the Authors
A. G. Brodsky
Regional Cardiology Dispensary “Diagnosis and Cardiovascular Surgery Center”, Surgut
Russian Federation
Russian Federation
Candidate of Sciences (Medicine), Cardiovascular Surgeon
E-mail: brodsky.alexey@gmail.com
A. N. Molchanov
Regional Cardiology Dispensary “Diagnosis and Cardiovascular Surgery Center”;
Surgut State University, Surgut
Russian Federation
Russian Federation
Doctor of Sciences (Medicine), Cardiovascular Surgeon
E-mail: amolchanov432@gmail.com
L. A. Tiltaeva
Regional Cardiology Dispensary “Diagnosis and Cardiovascular Surgery Center”, Surgut
Russian Federation
Russian Federation
Pediatric Cardiologist
E-mail: lora_tiltaeva@mail.ru
Yu. R. Burlachenko
Regional Cardiology Dispensary “Diagnosis and Cardiovascular Surgery Center”, Surgut
Russian Federation
Russian Federation
Radiotherapist
E-mail: zakirova-2011@list.ru
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Review
For citations:
Brodsky A.G., Molchanov A.N., Tiltaeva L.A., Burlachenko Yu.R. SURGICAL TREATMENT OF CONGENITAL SUPRAVALVULAR AORTIC STENOSIS: CLINICAL CASE. Vestnik SurGU. Meditsina. 2022;(1 (51)):52-57. (In Russ.)
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