Abstract
Shawn's syndrome is a rare form of syndromic congenital heart disease (about 0.6% of all congenital heart defects). Being a combined congenital heart disease, it is characterized by multilevel obstruction of the left heart, namely, the presence of a supravalvular mitral valve membrane, a parachute-like mitral valve with stenosis, subaortic stenosis and сoarctation of the aorta. Mostly common it is occurs an incomplete form of this syndrome when two or three of the above components of the defect are diagnosed. The prognosis for patients with Shone's anomaly is poor. In this article, we describe a case of successful stage-by-stage surgical correction of this defect in a child of the first year of life. The patient was diagnosed with Shawn's syndrome, including valvular and subvalvular mitral valve stenosis with an abnormality of the development of the subvalvular apparatus (shortening of the chords, papillary muscle fibrosis), valvular stenosis of the aortic valve, hypoplasia of the aortic arch. Taking into account the pronounced fibroelastosis and reduced pumping function of the left ventricle, the child underwent the first stage of surgical correction with the Damus–Kay–Stansel operation at the age of 7 days of life. Due to the progressive deformation of the distal part of the ascending aorta at the age of 3 months, the patient was re-operated: plastic surgery of the distal part of the ascending aorta was performed. At the age of 10 months, the operation to create a right-sided bidirectional cavapulmonal anastomosis was performed as planned. This case illustrates the adequacy of the chosen treatment tactics, as well as the effectiveness of stage-by-stage surgical treatment of a child of the first year of life with a severe form of Shawn's syndrome.
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About the authors
- Svetlana A. Zubkova, Junior Researcher, ORCID
- Margarita R. Tumanyan, Dr. Med. Sc., Professor, Head of Department,
ORCID
- Andrey A. Svobodov, Dr. Med. Sc., Chief Researcher, ORCID
- Elena G. Levchenko, Dr. Med. Sc., Leading Researcher, ORCID
- Gul’nara S. Netalieva, Ultrasonic Diagnostics Physician, ORCID
