Heart failure in patients with hypertrophic obstructive cardiomyopathy
DOI:
https://doi.org/10.58354/jvc.107E.2023.717Từ khóa:
Hypertrophic cardiomyopathy (HCM), HOCM, heart failureTóm tắt
Hypertrophic cardiomyopathy is a hereditary cardiac disease, diverse in clinical manifestations, cardiac structure and natural progression. Patients with disease are associate with a broad range of clinical presentations, from patients who are asymptomatic, accidentally discovered during routine examination, to patients with chest pain, dyspnea, syncope, and even sudden death. We are presenting the clinical case of a young male patient with hypertrophic cardiomyopathy with rather large left ventricular wall thickness about 37mm who was hospitalized with severe dyspnea accompanied by tachycardia episodes and hypotension. The patient underwent electrical cardioversion, Doppler echocardiography, stress Doppler echocardiography with treadmill, cardiac MRI, basic blood tests, coronary CT angiography and ventriculography. The results showed the patient had a very high NT-proBNP level of 16,271 pg/mL; echocardiography showed asymmetric left ventricular hypertrophy, a maximum resting LVOT gradient of 28 mmHg, increasing to 64mmHg at peak stress, preserved left ventricular systolic function and grade III left ventricular diastolic dysfunction; greatly increased left ventricular mass of 199.6 g/m2, and delayed contrast enhancement involving the subendocardium of both ventricles and transecting the left ventricular free wall on cardiac MRI. We also discuss various treatment option for this young man.
Tài liệu tham khảo
Maron BJ. Clinical Course and Management of Hypertrophic Cardiomyopathy. N Engl J Med. Aug 16 2018;379(7):655-668. doi: 10.1056/NEJMra1710575
Corrado D, Zorzi A. Declining Risk of Sudden Cardiac Death in Young Athletes. Circulation. Nov 13 2023. doi: 10.1161/CIRCULATIONAHA.123.067243
Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet. Jan 19 2013;381(9862):242-55. doi: 10.1016/S0140-6736(12)60397-3
Maron MS, Olivotto I, Betocchi S, et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med. Jan 23 2003;348(4):295-303. doi: 10.1056/NEJMoa021332
Maron BJ. Hypertrophic cardiomyopathy. Lancet. Jul 12 1997;350(9071):127-33. doi: 10.1016/S0140-6736(97)01282-8
Leggit JC, Whitaker D. Diagnosis and Management of Hypertrophic Cardiomyopathy: Updated Guidelines From the ACC/AHA. Am Fam Physician. Feb 1 2022;105(2):207-209.
Arbelo E, Protonotarios A, Gimeno JR, et al. [2023 ESC Guidelines for the management of cardiomyopathies]. G Ital Cardiol (Rome). Nov 2023;24(11):1e-127e. Linee guida ESC 2023 per il trattamento delle cardiomiopatie elaborate dalla task force per il trattamento delle cardiomiopatie della Societa Europea di Cardiologia (ESC). doi: 10.1714/4127.41209
Butzner M, Maron M, Sarocco P, et al. Clinical Diagnosis of Hypertrophic Cardiomyopathy Over Time in the United States (A Population-Based Claims Analysis). Am J Cardiol. Nov 15 2021;159:107-112. doi: 10.1016/j.amjcard.2021.08.024
Olivotto I, Oreziak A, Barriales-Villa R, et al. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet. Sep 12 2020;396(10253):759-769. doi: 10.1016/S0140-6736(20)31792-X
Rabiee Rad M, Ghasempour Dabaghi G, Habibi D. Safety and efficacy of mavacamten for treatment of hypertrophic cardiomyopathy: a systematic review and meta-analysis of randomized clinical trials. Egypt Heart J. Jan 12 2023;75(1):4. doi: 10.1186/s43044-023-00328-7
McDonagh TA, Metra M, Adamo M, et al. Corrigendum to: 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. Eur Heart J. Dec 21 2021;42(48):4901. doi: 10.1093/eurheartj/ehab670
McDonagh TA, Metra M, Adamo 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. Oct 1 2023;44(37):3627-3639. doi: 10.1093/eurheartj/ehad195