Мультидисциплінарний менеджмент хворих із гіпертрофічною кардіоміопатією
##plugins.themes.bootstrap3.article.main##
Анотація
Стаття містить огляд літературних наукових джерел даних MEDLINE на платформі PubMed, Web of Science, Scopus стосовно гіпертрофічної кардіоміопатії з акцентом на лікувальні аспекти. Відповідно до рекомендацій провідних експертів подано сучасне визначення гіпертрофічної кардіоміопатії з описом класифікаційних структурних та функціональних серцевих аномалій, асоційованих симптомів і узагальнених факторів ризику раптової серцевої смерті хворих. Представлено ранжування пацієнтів з огляду на обструкцію виносного тракту лівого шлуночка та клінічну маніфестацію. Згідно з міжнародними настановами наведено основні заходи лікування хворих залежно від гемодинамічних та клінічних характеристик із застосуванням фармакотерапії та інвазивних методів. Надано патофізіологічну аргументацію та клінічне обґрунтування показань до призначення препаратів з метою корекції ускладнень. В історичному контексті окреслено застосування септальної міектомії й алкогольної септальної абляції для усунення морфологічних аномалій серця та покращання клінічного статусу пацієнтів. Наведено результати власного досвіду хірургічного лікування хворих із гіпертрофічною кардіоміопатією.
##plugins.themes.bootstrap3.article.details##
Ключові слова:
Посилання
Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC guidelines for the management of cardiomyopathies. Eur Heart J. 2023:44(37):3503-626. https://doi.org/10.1093/eurheartj/ehad194
Brock R. Functional obstruction of the left ventricle; acquired aortic subvalvar stenosis. Guys Hosp Rep. 1957;106:221-38. https://pubmed.ncbi.nlm.nih.gov/13480570/
Braunwald E, Lambrew CT, Rockoff SD, Ross J, Morrow AG. Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based upon an analysis of 64 patients. Circulation. 1964;30 Suppl 4:3-119. https://doi.org/10.1161/01.cir.29.5s4.iv-3
Cohen J, Effat H, Goodwin JF, Oakley CM, Steiner RE. Hypertrophic obstructive cardiomyopathy. Br Heart J. 1964;26:16-32 https://doi.org/10.1136/hrt.26.1.16
Garfinkel AC, Seidman JG, Seidman CE. Genetic pathogenesis of hypertrophic and dilated cardiomyopathy. Heart Fail Clin. 2018;14(2):139-46. https://doi.org/10.1016/j.hfc. 2017.12.004
Gersh B, Maron B, Bonow R, Dearani J, Fifer M, Link S, et al. 2011 ACCF/AHA Guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American college of cardiology foundation/American heart association task force of practice guidelines. J Am Coll Cardiol. 2011 Dec 13;58(25):2703-38. https://doi.org/10.1016/j.jacc.2011.10.825
Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35:2733-79. https://doi.org/10.1093/eurheartj/ehu284
Maron BJ, Gardin JM, Flack JM, et al. Prevalence of hypertrophic cardiomyopathy in a general population of young adults: echocardiographic analysis of 4111 subjects in the CARDIA study: coronary artery risk development in (young) adults. Circulation. 1995; 92:785-9. https://doi.org/10.1161/01.cir.92.4.785
Semsarian C, Ingles J, Maron MS, Maron BJ. New perspectives on the prevalence of hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015;65:1249-54. https://doi.org/10.1016/j.jacc.2015.01.019
Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, ESC Scientific Document Group. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015;36:2793-867. https://doi.org/10.1093/eurheartj/ehv316
Skinner JR, Winbo A, Abrams D, Vohra J, Wilde AA. Channelopathies that lead to sudden cardiac death: clinical and genetic aspects. Heart Lung Circ. 2019;28:22-30. https://doi.org/10.1016/j.hlc.2018.09.007
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor 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 Am Coll Cardiol. 2020;76:e159-e240. https://doi.org/10.1016/j.jacc.2020.08.045
Gray B, Ackerman MJ, Semsarian C, Behr ER. Evaluation After Sudden Death in the Young: A Global Approach. Circ Arrhythm Electrophysiol. 2019 Aug;12(8):e007453. https://doi.org/10.1161/CIRCEP.119.007453
Marston NA, Han L, Olivotto I, Day SM, Ashley EA, Michels M, Pereira AC, Ingles J, Semsarian C, Jacoby D. Clinical characteristics and outcomes in childhood-onset hypertrophic cardiomyopathy. Eur Heart J. 2021;42(20):1988-96. https://doi.org/10.1093/eurheartj/ehab148
Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006. Circulation. 2009 Mar;119(8):1085-92. https://doi.org/10.1161/CIRCULATIONAHA.108.804617
Marian AJ, Braunwald E. Hypertrophic cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, and therapy. Circ Res. 2017;121:749-770. https://doi.org/10.1161/CIRCRESAHA.117.311059
Maron BJ, Desai MY, Nishimura RA, Spirito P, Rakowski H, Towbin JA, et al. Management of hypertrophic cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol. 2022;79(4):390-414. https://doi.org/10.1016/j.jacc.2021.11.021
Dybro AM, Rasmussen TB, Nielsen RR, et al. Randomized trial of metoprolol in patients with obstructive hypertropic cardiomyopathy. J Am Coll Cardiol. 2021;78:2505-17. https://doi.org/10.1016/j.jacc.2021.07.065
Sherrid MV, Shetty A, Winson G, et al. Treatment of obstructive hypertrophic cardiomyopathy symptoms and gradient resistant to first-line therapy with β-blockade or verapamil. Circ Heart Fail. 2013;6:694-702 https://doi.org/10.1161/CIRCHEARTFAILURE.112.000122
Coppini R, Ferrantini C, Pioner JM, et al. Electrophysiological and contractile effects of disopyramide in patients with obstructive hypertrophic cardiomyopathy: a translational study. JACC Basic Transl Sci. 2019;4:795-813. https://doi.org/10.1016/j.jacbts.2019.06.004
Adler A, Fourey D, Weissler-Snir A, et al. Safety of outpatient initiation of disopyramide for obstructive hypertrophic cardiomyopathy patients. J Am Heart Assoc. 2017;6:e005152. https://doi.org/10.1161/JAHA.116.005152
Autore C, Francia P, Tini G, Musumeci B. Old and new therapeutic solutions in the treatment of hypertrophic cardiomyopathy. Eur Heart J Suppl. 2023;25(Suppl B):B12-B15. https://doi.org/10.1093/eurheartjsupp/suad060
Zampieri M, Argirò A, Marchi A, et al. Mavacamten, a novel therapeutic strategy for obstructive hypertrophic cardiomyopathy. Curr Cardiol Rep. 2021;23:79. https://doi.org/10.1007/s11886-021-01508-0
Spertus JA, Fine JT, Elliott P, et al. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORERHCM): health status analysis of a randomised, double-blind, placebocontrolled, phase 3 trial. Lancet. 2021;397:2467-75. https://doi.org/10.1016/S0140-6736(21)00763-7
Chase Cole J, Benvie SF, DeLosSantos M. Mavacamten: A Novel Agent for Hypertrophic Cardiomyopathy. Clin Ther. 2024 Apr;46(4):368-73. https://doi.org/10.1016/j.clinthera.2024.02.007
Abood Z, Jan MF, Ashraf M, Kroboth S, Sanders H, Schweitzer M, Misicka A, Ollerman E, Jahangir A, Galazka P, Tajik AJ. Mavacamten in real-life practice: Initial experience at a hypertrophic cardiomyopathy centre. ESC Heart Fail. 2025 Feb;12(1):672-6. https://doi.org/10.1002/ehf2.14882
Chuang C, Collibee S, Ashcraft L, et al. Discovery of Aficamten (CK-274), a next generation cardiac myosin inhibitor for the treatment of hypertrophic cardiomyopathy. J Med Chem. 2021;64:14142-52. https://doi.org/10.1021/acs.jmedchem.1c01290
Ommen SR, Ho CY, Asif IM, Balaji S, Burke MA, Day SM, Dearani JA, Epps KC, Evanovich L, Ferrari VA, et al. 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association / American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation. 2024;149:e1239-e1311 https://doi.org/10.1161/CIR.0000000000001250
Morrow A.G., Brockenbrough E.S. Surgical treatment of idiopathic hypertrophic subaortic stenosis: technic and hemodynamic results of subaortic ventriculomyotomy. Ann Surg. 1961;154:181-9. https://doi.org/10.1097/00000658-196108000-00003
Schaff HV, Dearani JA, Ommen SR, Sorajja P, Nishimura RA. Expanding the indications for septal myectomy in patients with hypertrophic cardiomyopathy: Results of operation in patients with latent obstruction J Thorac Carciovasc Surg 2012;143:303-9. https://doi.org/10.1016/j.jtcvs.2011.10.059
Maron BJ, Dearani JA, Smedira NG, Schaff HV, Wang S, Rastegar H, Ralph-Edwards A, Ferrazzi P, Swistel D, et al. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel. Am J Cardiol. 2022 Oct 1;180:124-39. https://doi.org/10.1016/j.amjcard.2022.06.007
Sigwart U. Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy. Lancet. 1995 Jul 22;346(8969):211-4. https://doi.org/10.1016/s0140-6736(95)91267-
Zhanga Yu, Adamo M, Zou C, Porcari A, et al. Management of hypertrophic cardiomyopathy. J Cardiovasc Med. 2024;25;6. https://doi.org/10.2459/JCM.0000000000001616
Zhu M, Reyes KRL, Bilgili G, Siegel RJ, Lee Claggett B, Medical Therapies to Improve Left Ventricular Outflow Obstruction and Diastolic Function in Hypertrophic Cardiomyopathy. Adv. 2023 Sep 25;2(8):100622. https://doi.org/1016/j.jacadv.2023.100622. eCollection 2023
Monda E, Lioncino M, Palmiero G, et al. Bisoprolol for treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy: the BASIC (bisoprolol AS therapy in hypertrophic cardiomyopathy) study. Int J Cardiol 2022; 354:22-8. https://doi.org/10.1016/j.ijcard.2022.03.013
Kitaoka H, Tsutsui H, Kubo T, Ide T, Chikamori T, et al. CS/JHFS 2018 Guideline on the Diagnosis and Treatment of Cardiomyopathies. Circ J. 2021 Aug 25;85(9):1590-689. https://doi.org/10.1253/circj.CJ-20-0910
Hamada M, Ikeda S, Ohshima K, et al. Impact of chronic use of cibenzoline on left ventricular pressure gradient and left ventricular remodeling in patients with hypertrophic obstructive cardiomyopathy. J Cardiol. 2016;67:279-86. https://doi.org/10.1016/j.jjcc.2015.05.014
Gasior T. Advances in Cardiac Imaging and Genetic Testing for Diagnosis and Risk Stratification in Cardiomyopathies: 2024 Update J Clin Med. 2024 Nov 26;13(23):7166. J Clin Med. 2024 Nov 26;13(23):7166. https://doi.org/10.3390/jcm13237166.10.3390/jcm13237166.
Spoladore R, Fragasso G, Pannone L, Slavich M, Margonato A. Pharmacotherapy for the treatment of obstructive hypertrophic cardiomyopathy. Expert Opin Pharmacother. 2020;21(2):233-242. https://doi.org/10.1080/14656566.2019.1702023
McDonagh TA, Metra M, Adamo M, Gardner RS, Baumbach A, Böhm M, et al. 2023 Focused Update of the 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J. 2023;44:3627-39. J Clin Med. 2024 Nov 26;13(23):7166. https://doi.org/10.3390/jcm13237166.10.1093/ eurheartj/ehad195
Olivotto I, Oreziak A, Barriales-Villa R, et al. EXPLORER-HCM study investigators. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet 2020;396:759-69. https://doi.org/10.1016/S0140-6736(21)00763-7
Rudenko KV, Lazoryshynets VV, Trembovetska OM, Nevmerzhytska LO, Tregubova MO, Danchenko PA. The Results of Surgical Treatment of Patients with Hypertrophic Obstructive Cardiomyopathy as a Risk Factor of Sudden Cardiac Death. World of Medicine and Biology. 2020;72 (2): 109-14. Ukrainian. https://doi.org/10.26724/2079-8334-2020-2-72-109-114
Jallad P, Soliani M, Marins PHA, Fernandes F, Arteaga-Fernandez E et al. Brazilian Multicenter Registry of Alcohol Septal Ablation for Patients with Symptomatic Hypertrophic Obstructive Cardiomyopathy – BRASA Registry. Arq Bras Cardiol. 2025 May;122(6):e20240626. https://doi.org/10.36660/abc.20240626
Yokoyama Y, Shimoda T, Shimada YJ, Shimamura J, Akita K, Yasuda R, et al. Alcohol septal ablation versus surgical septal myectomy of obstructive hypertrophic cardiomyopathy: systematic review and meta-analysis. Eur J Cardiothorac Surg. 2023 Mar 1;63(3):ezad043. https://doi.org/10.1093/ejcts/ezad043
Rudenko KV, Lazoryshynets VV, Nevmerzhytska LO, Tregubova MO, Danchenko PA. Septal Myectomy with Mitral Valve Surgery in Patients After Alcohol Septal blation. Interact Cardiovasc Thorac Surg. 2022 May 2;34(5):723-30. https://doi.org/10.1093/icvts/ivac010
