2 . 2023

Hypertrophic cardiomyopathy at the circulatory decompensation stage: transplantation or reverse remodeling

Abstract

Progressive course of the hypertrophic cardiomyopathy (HCM) is characterized by a variety of non-random directions. The combined action of both genetic factors and diverse epigenetic influences underlies the individual development of clinical events. Here we present a unique case of the successful surgical treatment of a patient with decompensated obstructive HCM, who has already been examined under the heart-lung complex transplantation program. This clinical observation allows us to speak about the real possibility of successful reverse remodeling of the hypertrophic cardiomyopathy even at the dilated stage. It leads to a regredient decrease in intracardiac anatomy and function disorders, stabilization of systemic circulation and provide a chance to avoid or postpone for a long time the need for the heart or heart-lung transplantation.

Keywords:гипертрофическая кардиомиопатия (ГКМП); обструктивная ГКМП; дилатационная фаза ГКМП; обструкция притока; митральная дисплазия; MYH7; органосохраняющая хирургия; трансплантация сердца; диастолическая дисфункция

Funding. This study was supported by research project FURG-2023-009.

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

For citation: Dzemeshkevich S.L., Gramovich V.V., Sinitsyn V.E., Stukalova O.V., Mershina E.A., Ryzhkova E.V., Motreva A.P., Kalmykova O.V., Martyanova Yu.B., Pasyuga V.V., Zaklyazminskaya E.V. Hypertrophic cardiomyopathy at the circulatory decompensation stage: transplantation or reverse remodeling. Clinical and Experimental Surgery. Petrovsky Journal. 2023; 11 (2): 101–10. DOI: https://doi.org/10.33029/2308-1198-2023-11-2-101-110 (in Russian)

References

1. Semsarian Ch., Jngles J., Maron M.S., Maron B.S. New perspectives of hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015; 65 (12): 1249–54. DOI: https://doi.org/10.1016/j.jacc.2015.01.019

2. Biagini E., Coccolo F., Ferlito M., Perugini E., Rocchi G., Bacchi-Reggiani L., et al. Dilated-hypokinetic evolution of hypertrophic cardiomyopathy: prevalence, incidence, risk factors, and prognostic implications in pediatric and adult patients. J Am Coll Cardiol. 2005; 46 (8): 1543–50. DOI: https://doi.org/10.1016/j.jacc.2005.04.062

3. Sato A., Sakamoto N., Ando K., Kaneshiro T., Uekita H., Sugimoto K., et al. Dilated phase of hypertrophic cardiomyopathy caused by two different sarcomere mutations, treated with surgical left ventricular reconstruction and cardiac resynchronization therapy with a defibrillator. Intern Med (Tokyo, Japan). 2012; 51 (18): 2559–64. DOI: https://doi.org/10.2169/internalmedicine.51.7684

4. Dzemeshkevich S.L., Motreva A.P., Areshidze D.A., Kozlova M.A., Chernikov V.P., Mershina E.A., et al. Left ventricular remodeling and mitral reconstruction in patient with hypertrophic cardiomyopathy and inflow obstruction. Clinical and Experimental Surgery. Petrovsky Journal. 2023; 11 (1): 129–37. DOI: https://doi.org/10.33029/2308-1198-2023-11-1-129-137 (in Russian)

5. Kelly M.A., Caleshu C., Morales A., Buchan J., Wolf Z., Harrison S.M., et al. Adaptation and validation of the ACMG/AMP variant classification framework for MYH7-associated inherited cardiomyopathies: recommendations by ClinGen’s Inherited Cardiomyopathy Expert Panel. Genet Med. 2018; 20 (3): 351–9. DOI: https://doi.org/10.1038/gim.2017.218

6. Ommen S.R., Mital S., Burke M.A., Day S.M., Deswal A., Elliott P., et al. 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Thorac Cardiovasc Surg. 2021; 162 (1): e23–106. DOI: https://doi.org/10.1016/j.jtcvs.2021.04.001

7. Schaff H.V., Brown M.L., Dearani J.A., Abel M.D., Ommen S.R., Sorajja P., et al. Apical myectomy: a new surgical technique for management of severely symptomatic patients with apical hypertrophic cardiomyopathy. J Thorac Cardiovasc Surg. 2010; 139 (3): 634–40. DOI: https://doi.org/10.1016/j.jtcvs.2009.07.079

8. Harris K.M., Spirito P., Maron M.S., Zenovich A.G., Formisano F., Lesser J.R., et al. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation. 2006; 114 (3): 216–25. DOI: https://doi.org/10.1161/CIRCULATIONAHA.105.583500

9. Dzemeshkevich S.L., Motreva A.P., Kalachanova E.P., Nikityuk T.G., Mart’yanova Yu.B., Kalmykova O.V., et al. Clinical and Experimental Surgery. Petrovsky Journal. 2021; 9 (3): 16–24. DOI: https://doi.org/10.33029/2308-1198-2021-9-3-16-24 (in Russian)

10. Bogachev-Prokophiev A.V., Afanasyev A.V., Zheleznev S.I., Pivkin A.N., Fomenko M.S., Sharifulin R.M., et al. Concomitant ablation for atrial fibrillation during septal myectomy in patients with hypertrophic obstructive cardiomyopathy. J Thorac Cardiovasc Surg. 2018; 155 (4): 1536–42.e2. DOI: https://doi.org/10.1016/j.jtcvs.2017.08.063

11. Miron A., Lafreniere-Roula M., Steve Fan C.P., Armstrong K.R., Dragulescu A., Papaz T., et al. A validated model for sudden cardiac death risk prediction in pediatric hypertrophic cardiomyopathy. Circulation. 2020; 142 (3): 217–29. DOI: https://doi.org/10.1161/CIRCULATIONAHA.120.047235

12. Yamabe T., Ginns J., Vedula V., Leb J.S., Shimada Y.J., Weiner S.D., et al. Left ventricular remodeling following septal myectomy in hypertrophic obstructive cardiomyopathy. JTCVS Open. 2022; 11: 105–15. DOI: https://doi.org/10.1016/j.xjon.2022.05.018

13. Bos J.M., Towbin J.A., Ackerman M.J. Diagnostic, prognostic, and therapeutic implications of genetic testing for hypertrophic cardiomyopathy. J Am Coll Cardiol. 2009; 54 (3): 201–11. DOI: https://doi.org/10.1016/j.jacc.2009.02.075

14. Lopes L.R., Garcia-Hernández S., Lorenzini M., Futema M., Chumakova O., Zateyshchikov D., et al. Alpha-protein kinase 3 (ALPK3) truncating variants are a cause of autosomal dominant hypertrophic cardiomyopathy. Eur Heart J. 2021; 42 (32): 3063–73. DOI: https://doi.org/10.1093/eurheartj/ehab424

15. Kubo T., Gimeno J.R., Bahl A., Steffensen U., Steffensen M., Osman E., et al. Prevalence, clinical significance, and genetic basis of hypertrophic cardiomyopathy with restrictive phenotype. J Am Coll Cardiol. 2007; 49 (25): 2419–26. DOI: https://doi.org/10.1016/j.jacc.2007.02.061

16. Dzemeshkevich S.L., Korolev S.V., Gramovich V.V., Frolova Y.V., Lugovoy A.N., Dombrovskaya A.V., et al. Modified chordal sparing mitral valve replacement as effective technique for both stenotic and insufficient mitral valves. J Cardiovasc Surg. 2022; 63 (4): 498–506. DOI: https://doi.org/10.23736/S0021-9509.22.12065-3

17. Afanas’ev A.V. Optimization of surgical treatment methods for obstructive hypertrophic cardiomyopathy: Diss. Novosibirsk, 2023: 304 p. (in Russian)