Abstract
Complete inversion of internal organs (situs inversus totalis, SIT) is a rare condition that is characterized by a mirror arrangement of all organs of the chest and abdominal cavity; its prevalence in the population is approximately 1 in 10,000 newborns. In the group of patients with SIT, multiple congenital malformations are rarely observed, therefore, on average, representatives of this population have a normal life expectancy. The frequency of congenital heart defects in these patients is 3–5%. The most common congenital heart defects in SIT are atrioventricular discordance and transposition of the main arteries, as well as cases of combination with double outlet of vessels from the right or left ventricle, Fallot’s tetralogy, ventricular septal defect, anomalies of the coronary arteries, etc. Syndrome of left hypoplasia of the heart is a common defect, accounting 2–9% of cases of congenital heart defects and is the cause of 23% of neonatal deaths from congenital heart defects, taking the sad first place in terms of negative prognosis for life, recovery and treatment difficulties.
We present the first case of successful staged surgical treatment of a patient with hypoplasia syndrome of the left heart and SIT.
References
- Van Praagh S., Vlad P., Keith J.D. Diagnosis of the anatomic types of congenital dextrocardia. Am. J. Cardiol. 1965; 15: 234–47. DOI: 10.1016/0002- 9149(64)90159-6
- Applegate K.E., Goske M.J., Pierce G., Murphy D. Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 1999; 19 (4): 837–52. DOI: 10.1148/radiographics.19.4.g99jl31837
- Dayapala A., Kumar V. Congenital cyanotic heart disease with situs inversus totalis. Am. J. Forensic. Med. Pathol. 2009; 30 (2): 171–4. DOI: 10.1097/paf.0b013e3181875a79
- Hoffman J., Kaplan S. The incidence of congenital heart disease. J. Am. Coll. Cardiol. 2002; 39 (12): 1890–900. DOI: 10.1016/s0735-1097(02)01886-7
- Fruitman D.S. Hypoplastic left heart syndrome: prognosis and management options. Pediatr. Child. Health. 2000; 5 (4): 219–25. DOI: 10.1093/pch/5.4.219
- Oppido G., Napoleone C. P., Martano S., Gargiulo G. Hypoplastic left heart syndrome in situs inversus totalis. Eur. J. Cardiothorac. Surg. 2004; 26 (5): 1052–4. DOI: 10.1016/j.ejcts.2004.08.013
- Pontailler M., Gaudin R., Raisky O. The modified Norwood procedure at Necker Hospital: stage I and II for hypoplastic left heart syndrome. Oper. Tech. Thorac. Cardiovasc. Surg. 2022; 27 (3): 313–26. DOI:10.1053/j.optechstcvs.2022.04.009
- Norwood W.I., Kirklin J.K., Sanders S.P. Hypoplastic left heart syndrome: experience with palliative surgery. Am. J. Cardiol. 1980; 45: 87–91. DOI: 10.1016/0002-9149(80)90224-6 9.
- Metcalf M.K., Rychik J. Outcomes in hypoplastic left heart syndrome. Pediatr. Clin. North. Am. 2020: 67 (5): 945–62. DOI:10.1016/j.pcl.2020.06.008
- Goldberg D.J., Zak V., Goldstein B.H., Schumacher K.R., Rhodes J., Penny D.J. et al.; Pediatric Heart Network Investigators. Results of the FUEL trial. Circulation. 2020; 141 (8): 641–51. DOI: 10.1161/CIRCULATIONAHA.119.044352
About the authors
- Dmitriy V. Adkin, Cand. Med. Sci., Pediatric Cardiologist; ORCID
- Aleksey E. Chernogrivov, Dr. Med. Sci., Chief Research Associate, Cardiovascular Surgeon; ORCID
- Aleksandr A. Esayan, Cand. Med. Sci., Senior Research Associate, Cardiovascular Surgeon; ORCID
- Elizaveta S. Dorokhina, Cardiovascular Surgeon; ORCID
