4 . 2023

Multi-stage surgical correction of critical subaortic stenosis in an infant

Abstract

Hypertrophic cardiomyopathy in most cases is a genetically determined disease that can manifest both in early childhood and in adulthood.

Anomaly of subvalvular mitral apparatus may be involved in the genesis of LVOT obstruction.

In case of persistent resistance to drug therapy, in order to free the patient from the symptoms of subaortic obstruction, septal reduction therapy may be considered.

Surgery in infants and children is associated with high risks.

The aim of this article is to represent a clinical case of а successful multistage surgical correction of critical subaortic stenosis in an infant.

Keywords:hypertrophic cardiomyopathy; subaortic stenosis; septal myectomy; anomaly of subvalvular mitral apparatus; anomalous insertion of mitral valve chordae

Funding. The study had no sponsor support.

Conflict of interest. The authors declare no conflict of interest.

For citation: Abramyan M.A., Talalaeva Е.A., Pursanov M.G., Bedin A.V., Kurako M.M., Shamrin Yu.N., Miroshnichenko V.P., Chagirev V.N., Khasanova K.A. Multi-stage surgical correction of critical subaortic stenosis in an infant. Clinical and Experimental Surgery. Petrovsky Journal. 2023; 11 (4): 121–7. DOI: https://doi.org/10.33029/2308-1198-2023-11-4-121-127 (in Russian)

References

1. Sellers R.D., Lillehei C.W., Edwards J.E. Subaortic stenosis caused by anomalies of the atrioventricular valves. J Thorac Cardiovasc Surg. 1964; 48: 289–302. PMID: 14205063.

2. Jue K.L., Edwards J.E. Anomalous attachment of mitral valve causing subaortic atresia. Observations in a case with other cardiac anomalies and multiple spleens. Circulation. 1967; 35 (5): 928–32. DOI: https://doi.org/10.1161/01.cir.35.5.928 PMID: 4225595.

3. Arbelo E., Protonotarios A., Gimeno J.R., Arbustini E., Barriales-Villa R., Basso C., et al.; ESC Scientific Document Group. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023; 44 (37): 3503–626. DOI: https://doi.org/10.1093/eurheartj/ehad194 PMID: 37622657.

4. Lipshultz S.E., Law Y.M., Asante-Korang A., Austin E.D., Dipchand A.I., Everitt M.D., et al. Cardiomyopathy in children: classification and diagnosis: a scientific statement from the American Heart Association. Circulation. 2019; 140 (1): e9–68. DOI: https://doi.org/10.1161/CIR.0000000000000682 Epub 2019 May 28. PMID: 31132865.

5. Borisov K.V., Bockeria L.A., Sinyov A.F. Surgical treatment of hypertrophic obstructive cardiomyopathy in pediatric patients with severe hypertrophy. Artif Organs. 2008; 32 (11): 856–63. DOI: https://doi.org/10.1111/j.1525-1594.2008.00642.x PMID: 18959678.

6. Minakata K., Dearani J.A., Nishimura R.A., Maron B.J., Danielson G.K. Extended septal myectomy for hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles or chordae. J Thorac Cardiovasc Surg. 2004; 127 (2): 481–9. DOI: https://doi.org/10.1016/j.jtcvs.2003.09.040 PMID: 14762358.

7. Xu H., Yan J., Wang Q., Li D., Guo H., Li S., et al. Extended septal myectomy for hypertrophic obstructive cardiomyopathy in children and adolescents. Pediatr Cardiol. 2016; 37 (6): 1091–7. DOI: https://doi.org/10.1007/s00246-016-1396-5 Epub 2016 May 9. PMID: 27160105.

8. Schleihauf J., Cleuziou J., Pabst von Ohain J., Meierhofer C., Stern H., Shehu N., et al. Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants. Eur J Cardiothorac Surg. 2018; 53 (3): 538–44. DOI: https://doi.org/10.1093/ejcts/ezx369 PMID: 29088344.

9. Bansal N., Barach P., Amdani S.M., Lipshultz S.E. When is early septal myectomy in children with hypertrophic cardiomyopathy justified? Transl Pediatr. 2018; 7 (4): 362–6. DOI: https://doi.org/10.21037/tp.2018.09.08 PMID: 30460188; PMCID: PMC6212380.

10. Schleihauf J., Cleuziou J., Wolf C.M. Surgical septal myectomy for obstructive hypertrophic cardiomyopathy during infancy. Transl Pediatr. 2019; 8 (1): 90–1. DOI: https://doi.org/10.21037/tp.2018.12.0

11. Karamlou T., Gurofsky R., Bojcevski A., Williams W.G., Caldarone C.A., Van Arsdell G.S., et al. Prevalence and associated risk factors for intervention in 313 children with subaortic stenosis. Ann Thorac Surg. 2007; 84 (3): 900–6. DOI: https://doi.org/10.1016/j.athoracsur.2007.03.059 PMID: 17720397.

12. Colan S.D., Lipshultz S.E., Lowe A.M., Sleeper L.A., Messere J., Cox G.F., et al. Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation. 2007; 115 (6): 773–81. DOI: https://doi.org/10.1161/CIRCULATIONAHA.106.621185 Epub 2007 Jan 29. PMID: 17261650.

13. Ujuanbi A.S., Onyeka C.A., Yeibake W.S., Oremodu T., Kunle-Olowu O.E., et al. Pathological left ventricular hypertrophy and outflow tract obstruction in an infant of a diabetic mother: a case report. J Cardiol Cardiovasc Med. 2020; 5: 47–50.

14. Ningning Z., Liang C., Jing Z. Septal myectomy for severe neonatal hypertrophic cardiomyopathy caused by PTPN11 gene mutation: a case report. Biomed J Sci Tech Res. 2019; 21 (1): 003544.

15. Bockeria L.A., Gudkova R.G. Cardiovascular surgery 2013. Diseases and congenital abnormalities of the circulatory system. Moscow: NTsSSKh im. A.N. Bakuleva RAMN, 2014: 49. (in Russian)

16. Henaine R., Roubertie F., Vergnat M., et al. Valve replacement in children: a challenge for a whole life. Arch Cardiovasc Dis. 2012; 105: 517–28. DOI: https://doi.org/10.1016/j.acvd.2012.02.013

17. Bockeria L.A., Borisov K.V., Sinev A.F., Zlochevskaya E.V. Surgical treatment of hypertrophic obstructive cardiomyopathy in children. Detskie bolezni serdtsa i sosudov [Children’s Heart and Vascular Diseases]. 2005; (1): 23–8. (in Russian)

18. Maron B.J. Surgery for hypertrophic obstructive cardiomyopathy. Circulation. 2005; 111: 2016–8.

19. Cui H., Schaff H.V., Nishimura R.A., et al. Conduction abnormalities and long-term mortality following septal myectomy in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2019; 74 (5): 645–55. DOI: https://doi.org/10.1016/j.jacc.2019.05.053

20. Vanderlaan R.D., Woo A., Ralph-Edwards A. Isolated septal myectomy for hypertrophic obstructive cardiomyopathy: an update on the Toronto General Hospital experience. Ann Cardiothorac Surg. 2017; 6: 364–8.

21. Alsoufi B. Management of the single ventricle and potentially obstructive systemic ventricular outflow tract. J Saudi Heart Assoc. 2013; 25 (3): 191–202. DOI: https://doi.org/10.1016/j.jsha.2013.05.003