Abstract
Anomalies of intrathoracic positioning and formation of the heart account for 1.5% of all congenital heart defects. Situs ambigous represents a structural alteration of the cardiovascular system, where the nature of heart formation cannot be precisely determined, despite anatomical identification of the atrial cavity and the location of the venae cavae and pulmonary veins. One characteristic syndrome associated with situs ambigous is left isomerism, which is marked by the absence of the sinus node (SN) or, if it present, often involves hypoplasia and abnormal positioning, leading to arrhythmias, particularly sick sinus syndrome (SSS). This article presents preoperative planning methods and surgical treatment approaches for SSS in a patient after correction of congenital heart defects, including anomalies of intrathoracic positioning and formation of the heart (situs ambigous, left-sided heart, left isomerism, absence of hepatic segment of the inferior vena cava, single atrium, and pulmonary venous return draining into a right-sided atrium).
References
- Golukhova E.Z., Kim A.I., Zavalikhina T.V., Nefedova I.E., Chernogrivov A.E., Avakova S.A. Analysis of medical care delivery to children with congenital heart defects in the Russian Federation and prerequisites for the creation of a registry in the modern era of digital medical information systems. Creative Cardiology. 2023; 17 (3): 315–321 (in Russ.). DOI: 10.24022/1997-3187-2023-17-3-315-321
- Baumgartner H., De Backer J., Babu-Narayan S.V., Budts W., Chessa M., Diller G.P. et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur. Heart J. 2021; 42 (6): 563–645. DOI: 10.1093/eurheartj/ehaa554
- Zinkovskiy M.F. Congenital heart defects. Ed. by A.F. Vozianov. Kyiv; 2008 (in Russ.).
- Irwin R., Greaves M., Schmitt M. Left superior vena cava revisited. Eur. Heart J. Cardiovasc. Imaging. 2012; 13 (4): 284–291. DOI: 10.1093/ehjci/jes017
- Bockeria L.A., Shatalov K.V. (Eds.) Pediatric cardiac surgery: a guide for physicians. Moscow; 2016 (in Russ.).
- Podzolkov V.P., Ivanitskiy A.V., Kisileva I.P. Diagnosis of congenital heart defects with anomalies of its intrathoracic location. Bulletin of the USSR Academy of Sciences. 1974; 6: 41–46 (in Russ.).
- Evlakhov V.I., Pugovkin A.P., Rudakova T.L., Shalkovskaya L.N. Fundamentals of heart physiology: textbook. Saint-Petersburg; 2015 (in Russ.).
- Zweerink A., van der Lingen A.C.J., Handoko M.L., van Rossum A.C., Allaart C.P. Chronotropic incompetence in chronic heart failure. Circ. Heart Fail. 2018; 11 (8): e004969. DOI: 10.1161/CIRCHEARTFAILURE.118.004969
- Medvedowsky J.L., Barnay C., Pisapia A., Coste A. La maladie du sinus – diagnostic et traitement. Schweiz Med. Wochenschr. 1981; 111 (45): 1671–1678.
- Hernández-Madrid A., Paul T., Abrams D., Aziz P.F., Blom N.A., Chenet J. et al. Arrhythmias in congenital heart disease: a position paper of the EHRA/AEPC/ESC Working Group on Grown-up Congenital Heart Disease. Europace. 2018; 20 (10): 1719–1720. DOI: 10.1093/europace/eux380
- Revishvili A.Sh., Molodykh S.V., Neminushchiy N.M., Idov E.M., Kandinskiy M.L., Kazantsev K.B. Implantation of antiarrhythmic devices in congenital structural heart and vascular anomalies (indications, technical features of the operation, new technologies). Ekaterinburg-Tver; 2020 (in Russ.).
- 2021 ESC Guidelines on Cardiac Pacing and Cardiac Resynchronization Therapy. Russian Journal of Cardiology. 2022; 27 (7): 5159 (in Russ.). DOI: 10.15829/1560-4071-2022-5159
- Mond H., Karpawich P. Pacing options in the adult patient with congenital heart disease. Malden, USA: Blackwell Publishing Inc.; 2007.
- Shenthar J., Rai M.K., Walia R., Ghanta S., Sreekumar P., Reddy S.S. Transvenous permanent pacemaker implantation in dextrocardia: technique, challenges, outcome, and a brief review of literature. Europace. 2014; 16 (9): 1327–1333. DOI: 10.1093/europace/eut346
- Avanesyan G.A., Filatov A.G., Satyukova A.S., Obrezkov O.I., Bazhin M.A., Yakhyaev Ya.B. Temporary cardiac pacing. Annals of Arrhythmology. 2023; 20 (4): 224–231 (in Russ).
- Sergeev A.V., Shafiev E.Kh., Alatsiev T.D. A case report of the succesful dual chamber pacemaker implantation through the persistent left- sided superior vena cava. Annals of Arrhythmology. 2017; 14 (3): 171–176 (in Russ.). DOI: 10.15275/annaritmol.2017.3.8
- Tsao S., Deal B., Backer C. Device management of arrhythmias after Fontan conversion. J. Thorac. Cardiovasc. Surg. 2009; 138 (4): 937–940. DOI: 10.1016/j.jtcvs.2008.11.066
- Morozov A.A., Latypov A.K., Vasichkina E.S., Lebedev D.S. A case of using combined surgical access for implantation of a permanent epicardial pacing system in a child after fontan operation. Journal of Arrhythmology. 2014; 76: 60–62 (in Russ.).
- Avanesyan G.A., Kovalev S.A., Filatov A.G. Pathophysiology of rhythm disorders after mustard or senning operations. Annals of Arrhythmology. 2023; 20 (1): 4–14 (in Russ.). DOI: 10.15275/annaritmol.2023.1.1
- Bockeria L.A., Rubtsov P.P. Heart rhythm disorders in adult patients with congenital heart defects. Annals of Arrhythmology. 2021; 18 (1): 13–19 (in Russ.). DOI: 10.15275/annaritmol.2021.1.3
- Chan N.-Y., Kwong N.-P., Cheong A.-P. Venous access and long-term pacemaker lead failure: comparing contrast-guided axillary vein puncture with subclavian puncture and cephalic cutdown. Europace. 2017; 19 (7): 1193–1197. DOI: 10.1093/europace/euw147
- Revishvili A.Sh., Artyukhina E.A., Glezer M.G., Bazayev V.A., Batalov R.E., Bockeria L.A. et al. Bradyarrhythmias and conduction disorders. Clinical Guidelines 2020. Russian Journal of Cardiology. 2021; 26 (4): 4448 (in Russ.). DOI: 10.15829/1560-4071-2021-4448
About the authors
- Anton S. Serguladze, Resident Physician; ORCID
- Georgy R. Matsonasvili, Cand. Med. Sci., Cardiovascular Surgeon; ORCID
- Teymurazi R. Matsonasvili, Cand. Med. Sci., Cardiovascular Surgeon; ORCID
- Anton V. Minaev, Dr. Med. Sci., Cardiovascular Surgeon; ORCID
- Mikhail L. Dvali, Postgraduate, Cardiovascular Surgeon; ORCID
