Multifaces of hypertrophic cardiomyopathy: a case of transformation of hypertrophic phenotype into dilated

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Abstract

The article presents a clinical case of a rather rare course of hypertrophic cardiomyopathy with the transformation of a hypertrophic phenotype into a dilated phenotype against the background of the “burned-out phase” phenomenon, ventricular and supraventricular rhythm disturbances, and multiple genetic mutations. Timely started disease-modifying therapy (quadruple therapy) for chronic heart failure led to reverse positive remodeling of the left chambers of the heart.

About the authors

Tatiana N. Novikova

North-Western State Medical University named after I.I. Mechnikov

Author for correspondence.
Email: novikova-tn@mail.ru
ORCID iD: 0000-0003-4655-0297
SPIN-code: 3401-0329

MD, Cand. Sci. (Med.), Assistant Professor of the Department of Hospital Therapy and Cardiology named after M.S. Kushakovskiy

Russian Federation, Saint Petersburg

Alina E. Andreeva

North-Western State Medical University named after I.I. Mechnikov

Email: Doc.alina@mail.ru
ORCID iD: 0009-0004-1212-5406
SPIN-code: 7378-1194

Head of hospital department of functional diagnostics, Assistant Professor

Russian Federation, Saint Petersburg

Fatima I. Bitakova

North-Western State Medical University named after I.I. Mechnikov

Email: Fatima.Bitakova@szgmu.ru
ORCID iD: 0000-0001-6637-8266
SPIN-code: 8624-7193

MD, Cand. Sci. (Med.), Assistant Professor

Russian Federation, Saint Petersburg

Vladimir I. Novikov

North-Western State Medical University named after I.I. Mechnikov

Email: novikov-vi@mail.ru
ORCID iD: 0000-0002-2493-6300
SPIN-code: 6909-3377

MD, Dr Sci. (Med.), Professor

Russian Federation, Saint Petersburg

Kristina A. Gladysheva

North-Western State Medical University named after I.I. Mechnikov

Email: kirklandgladysheva@mail.ru
ORCID iD: 0009-0001-2262-2770

6th year student

Russian Federation, Saint Petersburg

Polina V. Petrova

North-Western State Medical University named after I.I. Mechnikov

Email: apolly2017@yandex.ru
ORCID iD: 0000-0002-2658-9920

clinical resident

Russian Federation, Saint Petersburg

Polina A. Stalnova

North-Western State Medical University named after I.I. Mechnikov

Email: polya161001@gmail.com
ORCID iD: 0009-0009-5835-8359

6th year student

Russian Federation, Saint Petersburg

Nadezhda A. Tokareva

North-Western State Medical University named after I.I. Mechnikov

Email: tokareva.nad9@mail.ru
ORCID iD: 0009-0001-2663-5640

clinical resident

Russian Federation, Saint Petersburg

References

  1. Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(37):3503–3626. doi: 10.1093/eurheartj/ehad194
  2. Raphael CE, Cooper R, Parker KH, et al. 2016 Mechanisms of myocardial ischemia in hypertrophic cardiomyopathy: insights from wave intensity analysis and magnetic resonance. J Am Coll Cardiol. 2016;68(15):1651–1660. doi: 10.1016/j.jacc.2016.07.751
  3. Novikov VI, Novikova TN. Cardiomyopathies. 2nd ed., rev. and supplement. Moscow: MEDpress-Inform; 2024. 144 p. (In Russ.)
  4. Brinkley DM, Wells OS, Stevenson LW. Avoiding burnout from hypertrophic cardiomyopathy. J Am Coll Cardiol. 2020;75(24): 3044–3047. doi: 10.1016/j.jacc.2020.05.009
  5. Potter E, Marwick TH. Assessment of left ventricular function by echocardiography: the case for routinely adding global longitudinal strain to ejection fraction. JACC Cardiovasc Imaging. 2018;11(2(Pt 1)):260–274. doi: 10.1016/j.jcmg.2017.11.017
  6. Pathan F, D’Elia N, Nolan MT, et al. Normal ranges of left atrial strain by speckle-tracking echocardiography: a systematic review and meta-analysis. J Am Soc Echocardiogr. 2017;30(1):59–70.e8. doi: 10.1016/j.echo.2016.09.007
  7. McDonagh TA, Metra M, Adamo M, et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: Developed by the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) With the special contribution of the Heart Failure Association (HFA) of the ESC. Euro Heart J. 2021;42(36):3599–3726. doi: 10.1093/eurheartj/ehab368
  8. Tereshchenko SN, Galyavich AS, Uskach TM, et al. 2020 Clinical practice guidelines for Chronic heart failure. Russian Journal of Cardiology. 2020;25(11):311–374. EDN: LJGGQV doi: 10.15829/1560-4071-2020-4083
  9. Gabrusenko SA, Gudkova AYa, Koziolova NA, et al. 2020 Clinical practice guidelines for Hypertrophic cardiomyopathy. Russian Journal of Cardiology. 2021;26(5):269–334. EDN: MXDYLE doi: 10.15829/1560-4071-2021-4541
  10. Adamczak DM, Rogala A, Antoniak M, Oko-Sarnowska Z. New predictors of burned-out phase in hypertrophic cardiomyopathy. Eur Heart J Cardiovasc Imaging. 2020;21(Supplement_1). doi: 10.1093/ehjci/jez319.828
  11. Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022;43(40):3997–4126. doi: 10.1093/eurheartj/ehac262
  12. Novikova TN, Bitakova FI, Ignatieva VS, et al. European guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 2022: cardiomyopathy. What’s new? Cardiac Arrhythmias. 2023;3(3):41–62. EDN: DHKBZF doi: 10.17816/cardar56

Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1. Patient’s ECG in May 2022 (recording speed is 50 mm/s)

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3. Fig. 2. Bull’s eye format of peak global longitudinal strain of the left ventricular myocardium. Explanation in the text

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4. Fig. 3. Assessment of left atrial function in 2022 in 2D-Strain mode. Explanation in the text

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5. Fig. 4. Assessment of left atrial function over time in February 2024 in 2D-Strain mode. Explanation in the text

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6. Fig. 5. Fragment of ECG monitoring. Episode of non-sustained ventricular tachycardia

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