Abstract
“Heterotaxy syndrome” (HS) is a general term for the improper location of the internal organs of the thoracic and abdominal cavities. The spectrum of congenital heart defects and extracardial pathology is variable. Heart lesions may include abnormalities in the position and formation of the heart, abnormalities of individual cardiac structures, and disorders of the cardiac conduction system. The intracardial anatomy differs in each group. Studies show that with left-atrial isomerism, associated heart malformations are less severe than with right isomerism. There are almost always two ventricles, the most common are isolated septal defects, a common or partial open atrioventricular canal, partial abnormal drainage of the pulmonary veins, possibly double divergence of the main arteries from the right ventricle, L-transposition of the main arteries, atresia or stenosis of the pulmonary artery are observed much less frequently. In isomerism, heart lesions include both structural disorders and cardiac conduction disorders. The material of the article is based on the analysis of publications in recent years in international medical databases.
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About the authors
- Tatiana V. Rogova, Dr. Med. Sci., Professor, Head of the Department; ORCID
- Aminat S. Magandalieva, Pediatric Cardiologist; ORCID
- Margarita V. Gordeeva, Cand. Med. Sci., Senior Researcher, Pathologist; ORCID
