Advances in the Management of Hypertrophic Cardiomyopathy Leading to Low Disease-Related Mortality in 2023

Abstract

Hypertrophic cardiomyopathy (HCM) is a relatively common often inherited heart disease encumbered throughout much of its almost 60-year history by the expectation of an unfavorable outcome with shortened longevity. However, it is notable that in 2023, most patients affected with HCM can now achieve normal or extended life expectancy without major disability because of a comprehensive constellation of management strategies that have evolved largely over the last 20 years. Distinct adverse disease pathways dictate high-benefit low-risk personalized treatments, without reliance on genomics and sarcomere mutations, including: primary prevention implantable defibrillators for sudden cardiac death prevention, surgical myectomy and percutaneous alcohol septal ablation to reverse heart failure symptoms, anticoagulation to prevent embolic stroke associated with concomitant atrial fibrillation, external defibrillation and hypothermia for out-of-hospital cardiac arrest, and heart transplant in a small patient subgroup with end-stage disease. Large cohort studies using these contemporary management strategies achieved remarkably low HCM-related mortality (0.5%/year) across all age groups, which is lower than in the other cardiac or noncardiac risks of living, and largely confined to nonobstructive patients with progressive heart failure, including those awaiting heart transplant. Hypertrophic cardiomyopathy (HCM) is a relatively common often inherited heart disease encumbered throughout much of its almost 60-year history by the expectation of an unfavorable outcome with shortened longevity. However, it is notable that in 2023, most patients affected with HCM can now achieve normal or extended life expectancy without major disability because of a comprehensive constellation of management strategies that have evolved largely over the last 20 years. Distinct adverse disease pathways dictate high-benefit low-risk personalized treatments, without reliance on genomics and sarcomere mutations, including: primary prevention implantable defibrillators for sudden cardiac death prevention, surgical myectomy and percutaneous alcohol septal ablation to reverse heart failure symptoms, anticoagulation to prevent embolic stroke associated with concomitant atrial fibrillation, external defibrillation and hypothermia for out-of-hospital cardiac arrest, and heart transplant in a small patient subgroup with end-stage disease. Large cohort studies using these contemporary management strategies achieved remarkably low HCM-related mortality (0.5%/year) across all age groups, which is lower than in the other cardiac or noncardiac risks of living, and largely confined to nonobstructive patients with progressive heart failure, including those awaiting heart transplant. Almost since its initial clinical recognition >60 years ago,1Braunwald E, Lambrew E, Rockoff D, Rockoff D, Ross J Jr., Morrow AG. Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based upon an analysis of 64 patients. Circulation 1964;29:IV-3–IV-119.Google Scholar,2Maron BJ Maron MS Maurer MS Rowin EJ Maron BA Galiè N. Cardiovascular diseases that have emerged from the darkness.J Am Heart Assoc. 2021; 10e021095Google Scholar hypertrophic cardiomyopathy (HCM) was regarded as a rare heterogeneous debilitating disease with a grim prognosis and reduced life expectancy, and the most common cause of sudden cardiac death (SCD) in the young. That perception persisted for decades until the last 20 to 25 years when contemporary treatment options emerged.2Maron BJ Maron MS Maurer MS Rowin EJ Maron BA Galiè N. Cardiovascular diseases that have emerged from the darkness.J Am Heart Assoc. 2021; 10e021095Google Scholar, 3Maron BJ Rowin EJ Casey SA Maron MS. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice.JAMA Cardiol. 2016; 1: 98-105Google Scholar, 4Maron BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google Scholar, 5Maron BJ Desai MY Nishimura RA Spirito P Rakowski H Towbin JA Dearani JA Rowin EJ Maron MS Sherrid MV. Management of hypertrophic cardiomyopathy: JACC State-of-the-Art Review.J Am Coll Cardiol. 2022; 79: 390-414Google Scholar, 6Maron BJ. Clinical course and management of hypertrophic cardiomyopathy.N Engl J Med. 2018; 379: 655-668Google Scholar, 7Maron BJ Spirito P Shen WK Haas TS Formisano F Link MS Epstein AE Almquist AK Daubert JP Lawrenz T Boriani G Estes NA Favale S Piccininno M Winters SL Santini M Betocchi S Arribas F Sherrid MV Buja G Semsarian C Bruzzi P. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy.JAMA. 2007; 298: 405-412Google Scholar, 8Maron BJ Rowin EJ Maron MS. Hypertrophic cardiomyopathy: is a “cure” coming…or is it already here?.Am J Med. 2020; 133: 886-888Google Scholar Therefore, the difficult-to-treat and high-mortality reputation of HCM has become less entrenched in the consciousness of the practicing community and patient population. Therefore, we take this opportunity to review and underscore the substantial progress made with respect to the modern management of HCM, with regard to life expectancy, survival, and mortality. The life-saving potential of the implantable cardioverter-defibrillator (ICD)12Maron BJ Estes NAM Rowin EJ Maron MS Reynolds MR. Development of the implantable cardioverter-defibrillator: JACC Historical Breakthroughs in Perspective.J Am Coll Cardiol. 2023; 82: 353-373Google Scholar was translated to HCM and other inherited diseases in 2000, by virtue of a seminal New England Journal of Medicine report10Maron BJ Shen WK Link MS Epstein AE Almquist AK Daubert JP Bardy GH Favale S Rea RF Boriani G Estes NA Spirito P. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.N Engl J Med. 2000; 342: 365-373Google Scholar that opened the possibility of effective device therapy for diseases such as HCM that were not initially foreseen by the original inventors of the ICD.12Maron BJ Estes NAM Rowin EJ Maron MS Reynolds MR. Development of the implantable cardioverter-defibrillator: JACC Historical Breakthroughs in Perspective.J Am Coll Cardiol. 2023; 82: 353-373Google Scholar Subsequently, the effective use of the ICD for patients with HCM has spread well beyond the United States into many countries (n ≥30; B.J. Maron personal communication), underscoring the principle that ICDs automatically and reliably abort life-threatening ventricular tachyarrhythmias (ventricular tachycardia/ventricular fibrillation) and restore sinus rhythm in patients with HCM of all ages, including children, adolescents, and young adults, often with the most aggressive clinical course.13Maron BJ Rowin EJ Maron MS. Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: twenty years with the implantable cardioverter-defibrillator.Heart Rhythm. 2021; 18: 1012-1023Google Scholar This ICD benefit has been demonstrated in retrospective registries and meta-analyses (e.g., 3% to 4%/year for primary prevention and 10%/year for secondary prevention after cardiac arrest) and also in prospective HCM cohort analyses in which SCD was exceedingly rare, occurring primarily in nonimplanted patients who refused ICDs.13Maron BJ Rowin EJ Maron MS. Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: twenty years with the implantable cardioverter-defibrillator.Heart Rhythm. 2021; 18: 1012-1023Google Scholar, 14Maron BJ Casey SA Olivotto I Sherrid MV Semsarian C Autore C Ahmed A Boriani G Francia P Winters SL Giudici M Koulova A Garberich R Rowin EJ Sears SF Maron MS Spirito P. Clinical course and quality of life in high-risk patients with hypertrophic cardiomyopathy and implantable cardioverter-defibrillators.Circ Arrhythm Electrophysiol. 2018; 11e005820Google Scholar, 15Maron MS Rowin EJ Wessler BS Mooney PJ Fatima A Patel P Koethe BC Romashko M Link MS Maron BJ. Enhanced American College of Cardiology/American Heart Association strategy for pre-vention of sudden cardiac death in high-risk patients with hyper-trophic cardiomyopathy.JAMA Cardiol. 2019; 4: 644-657Google Scholar After ICD therapy, patients with HCM are free of transformation to disabling heart failure or sudden arrhythmic death,14Maron BJ Casey SA Olivotto I Sherrid MV Semsarian C Autore C Ahmed A Boriani G Francia P Winters SL Giudici M Koulova A Garberich R Rowin EJ Sears SF Maron MS Spirito P. Clinical course and quality of life in high-risk patients with hypertrophic cardiomyopathy and implantable cardioverter-defibrillators.Circ Arrhythm Electrophysiol. 2018; 11e005820Google Scholar in stark contrast to at-risk patients with ischemic heart disease after appropriate device interventions. Notably, the risk stratification algorithm for selecting patients for an ICD has matured over the last 20 years, now, with a sensitivity of 95% for identifying patients in whom prophylactically implanted ICDs will prevent arrhythmic SCD, using the traditional American Heart Association/American College of Cardiology risk marker strategy.15Maron MS Rowin EJ Wessler BS Mooney PJ Fatima A Patel P Koethe BC Romashko M Link MS Maron BJ. Enhanced American College of Cardiology/American Heart Association strategy for pre-vention of sudden cardiac death in high-risk patients with hyper-trophic cardiomyopathy.JAMA Cardiol. 2019; 4: 644-657Google Scholar, 16Rowin EJ Maron BJ Haas TS Garberich RF Wang W Link MS Maron MS. Hypertrophic cardiomyopathy with left ventricular apical aneurysm: implications for risk stratification and management.J Am Coll Cardiol. 2017; 69: 761-773Google Scholar, 17Chan RH Maron BJ Olivotto I Pencina MJ Assenza GE Haas T Lesser JR Gruner C Crean AM Rakowski H Udelson JE Rowin E Lombardi M Cecchi F Tomberli B Spirito P Formisano F Biagini E Rapezzi C De Cecco CN Autore C Cook EF Hong SN Gibson CM Manning WJ Appelbaum E Maron MS. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy.Circulation. 2014; 130: 484-495Google Scholar The ICD represents a paradigm change in management, which has altered the clinical course of patients with HCM and significantly reduced disease-related mortality.9Maron MS, Rowin EJ, Maron BJ.The paradigm of sudden death prevention in hypertrophic cardiomyopathy. Am J CardiolGoogle Scholar,13Maron BJ Rowin EJ Maron MS. Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: twenty years with the implantable cardioverter-defibrillator.Heart Rhythm. 2021; 18: 1012-1023Google Scholar Progressive refractory heart failure in HCM is usually attributable to dynamic left ventricular (LV) outflow obstruction and is largely reversible with septal reduction intervention.4Maron BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google Scholar For over 60 years, surgical septal myectomy has been used effectively in several centers worldwide to permanently abolish LV outflow gradients (and secondary mitral regurgitation) (Figure 4).4Maron BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google Scholar,18Maron BJ Roberts WC. The father of septal myectomy for obstructive HCM, who also had HCM: the unbelievable story.J Am Coll Cardiol. 2016; 67: 2900-2903Google Scholar, 19Maron BJ Dearani JA Ommen SR Maron MS Schaff HV Nishimura RA Ralph-Edwards A Rakowski H Sherrid MV Swistel DG Balaram S Rastegar H Rowin EJ Smedira NG Lytle BW Desai MY Lever HM. Low operative mortality achieved with surgical septal myectomy: role of dedicated hypertrophic cardiomyopathy centers in the management of dynamic subaortic obstruction.J Am Coll Cardiol. 2015; 66: 1307-1308Google Scholar, 20Maron MS Rastegar H Dolan N Carpino P Koethe B Maron BJ Rowin EJ. Outcomes over follow-up ≥10 years after surgical myectomy for symptomatic obstructive hypertrophic cardiomyopathy.Am J Cardiol. 2022; 163: 91-97Google Scholar As a consequence, myectomy uniquely relieved advanced heart failure symptoms (e.g., predominantly exertional dyspnea), in the process, returning 90% to 95% of patients to normal activity and acceptable quality of life.4Maron BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google Scholar Surgical myectomy enhances long-term survival (and possibly reduces arrhythmic SCD risk), equivalent to that expected in the general population.4Maron BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google Scholar In those uncommon instances in which septal reduction treatment is unavailable (or gradients are incompletely treated), persistent obstruction can lead to refractory HCM-related heart failure.21Maurizi N Olivotto I Maron MS Bonacchi G Antiochos P Tomberli B Fumagalli C Poggesi C Berteotti M Girolami F Cecchi F. Lifetime clinical course of hypertrophic cardiomyopathy: outcome of the historical Florence cohort over 5 decades.JACC Adv. 2023; 2100337Google Scholar Nevertheless, the only deaths directly attributable to obstructive HCM are those few occurring in the first 30 days postoperatively (i.e., 0.5% mortality in experienced surgical centers).19Maron BJ Dearani JA Ommen SR Maron MS Schaff HV Nishimura RA Ralph-Edwards A Rakowski H Sherrid MV Swistel DG Balaram S Rastegar H Rowin EJ Smedira NG Lytle BW Desai MY Lever HM. Low operative mortality achieved with surgical septal myectomy: role of dedicated hypertrophic cardiomyopathy centers in the management of dynamic subaortic obstruction.J Am Coll Cardiol. 2015; 66: 1307-1308Google Scholar Heart transplant is the only definitive option available to relieve heart failure symptoms and preserve life in the small subgroup of patients with nonobstructive HCM and advanced end-stage disease refractory to pharmacologic management.22Maron MS Kalsmith BM Udelson JE Li W after cardiac in patients with hypertrophic Heart EJ Maron BJ P MS A F Maron MS of advanced for survival to heart transplant in patients with hypertrophic cardiomyopathy.Am J Cardiol. 2018; Scholar and quality of life to many patients with In the United of the survival after heart transplant for patients with HCM are and and years, and to that of patients for other end-stage MS Kalsmith BM Udelson JE Li W after cardiac in patients with hypertrophic Heart Scholar This transplant benefit is because of the age and lower of patients with is an and treatment to septal myectomy that also represents a to heart failure and mortality. is most for patients with who are not surgical or in patients to P. septal ablation for obstructive hypertrophic cardiomyopathy: a of Am Coll Cardiol. 2017; SR S A P J JA P C M Link MS Maron MS Schaff HV Semsarian C P. for the and treatment of patients with hypertrophic cardiomyopathy: a of the American College of Cardiology/American Heart Association on Clinical Am Coll Cardiol. 2020; Scholar (Figure is associated with low it is less than myectomy for outflow and heart failure on the of the to relieve outflow gradients (and in to of or The to which the can over the A therapy with has been to HCM with a with the of LV outflow gradients by the of LV and I A R A P S A M W J J D Li W M Wang A D for treatment of symptomatic obstructive hypertrophic cardiomyopathy a 2020; Scholar the of heart failure symptoms, or with surgical myectomy or BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google MS Ommen and for obstructive hypertrophic cardiomyopathy: in 2021; BJ Maron MS Sherrid MV Rowin EJ. role of in the of surgical myectomy for symptomatic obstructive hypertrophic Am Heart Assoc. 2022; Scholar Therefore, over the can as a therapy option in patients who not or for treatments, encumbered by a risk for and for atrial MS Ommen and for obstructive hypertrophic cardiomyopathy: in 2021; BJ Maron MS Sherrid MV Rowin EJ. role of in the of surgical myectomy for symptomatic obstructive hypertrophic Am Heart Assoc. 2022; Scholar A is with a pharmacologic relatively and MS A Olivotto I S Wang A P SF F A C A H R CM SL S A T and of in patients with obstructive hypertrophic Am Coll Cardiol. 2023; Scholar The are to the quality of life by is that these have the potential to significantly the already low HCM-related mortality that is based on BJ Rowin EJ Casey SA Maron MS. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice.JAMA Cardiol. 2016; 1: 98-105Google Scholar, 4Maron BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google Scholar, 5Maron BJ Desai MY Nishimura RA Spirito P Rakowski H Towbin JA Dearani JA Rowin EJ Maron MS Sherrid MV. Management of hypertrophic cardiomyopathy: JACC State-of-the-Art Review.J Am Coll Cardiol. 2022; 79: 390-414Google Scholar, 6Maron BJ. Clinical course and management of hypertrophic cardiomyopathy.N Engl J Med. 2018; 379: 655-668Google Scholar over years, has demonstrated the to obstruction and symptoms without MV I WJ S Casey S Maron BJ. of the and of in obstructive hypertrophic Am Coll Cardiol. Scholar is is the most common in HCM, occurring in of including associated with EJ Link MS Maron MS Maron BJ. contemporary management of atrial in hypertrophic cardiomyopathy.Circulation. 2023; Scholar (Figure EJ Link MS Maron MS Maron BJ. contemporary management of atrial in hypertrophic cardiomyopathy.Circulation. 2023; I Cecchi F Casey SA A Maron BJ. of atrial on the clinical course of hypertrophic cardiomyopathy.Circulation. has been a major of embolic stroke in HCM, in with the of with than is in HCM clinical that after the first This strategy has reduced stroke to only of patients with and with of stroke confined to the initial or in patients who Notably, with contemporary treatments, the HCM mortality attributable to is now only which is not from the risk by patients with HCM without EJ Link MS Maron MS Maron BJ. contemporary management of atrial in hypertrophic cardiomyopathy.Circulation. 2023; Scholar In the history of HCM was by a disease-related mortality of BJ Rowin EJ Casey SA Maron MS. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice.JAMA Cardiol. 2016; 1: 98-105Google Scholar This has been reduced to for 95% BJ Rowin EJ Casey SA Maron MS. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice.JAMA Cardiol. 2016; 1: 98-105Google M A M A A Winters SL SR Maron BJ Rowin E Maron MS. and of the of sudden cardiac death in hypertrophic Electrophysiol. 2022; BJ Maron MS Rowin EJ. on the risks of with hypertrophic cardiomyopathy.Circulation. 2017; Scholar which is lower than other noncardiac diseases (e.g., or cardiac (e.g., heart failure and that the risks of BJ Maron MS Rowin EJ. on the risks of with hypertrophic cardiomyopathy.Circulation. 2017; Scholar BJ Shen WK Link MS Epstein AE Almquist AK Daubert JP Bardy GH Favale S Rea RF Boriani G Estes NA Spirito P. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.N Engl J Med. 2000; 342: 365-373Google M A M A A Winters SL SR Maron BJ Rowin E Maron MS. and of the of sudden cardiac death in hypertrophic Electrophysiol. 2022; Scholar and of SCD to HCM spanning clinical since the decades after of the ICD to HCM in BJ Shen WK Link MS Epstein AE Almquist AK Daubert JP Bardy GH Favale S Rea RF Boriani G Estes NA Spirito P. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.N Engl J Med. 2000; 342: 365-373Google Scholar and with the are also in and in and M A M A A Winters SL SR Maron BJ Rowin E Maron MS. and of the of sudden cardiac death in hypertrophic Electrophysiol. 2022; Large and mortality for diseases that the risks of The is for HCM in the contemporary management end-stage Large septal BJ Dearani JA Smedira NG Schaff HV Wang S Rastegar H Ralph-Edwards A Ferrazzi P Swistel D Shemin RJ Quintana E Bannon PG Shekar PS Desai M Roberts WC Lever HM Adler A Rakowski H Spirito P Nishimura RA Ommen SR Sherrid MV Rowin EJ Maron MS. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC Expert Panel.Am J Cardiol. 2022; 180: 124-139Google Scholar in myectomy from major American HCM surgical centers with alcohol septal ablation have alcohol ablation have a and much less free from mortality after myectomy is to an age and general population. mortality for myectomy is much lower than for other common including and of HCM-related survival free from mortality in HCM patient surgical myectomy (n with obstruction (n and nonobstructive (n MV mitral Large outcome of patients with HCM and demonstrated by HCM 20 years This in clinical course from the of treatment EJ Link MS Maron MS Maron BJ. contemporary management of atrial in hypertrophic cardiomyopathy.Circulation. 2023; Scholar with Olivotto I Cecchi F Casey SA A Maron BJ. of atrial on the clinical course of hypertrophic cardiomyopathy.Circulation. Scholar in Large This low HCM mortality is across all age groups, including patients who are the risk for the small of deaths directly attributable to HCM in the subgroup of nonobstructive patients with progressive refractory heart failure, in the end-stage with and awaiting or after heart BJ Desai MY Nishimura RA Spirito P Rakowski H Towbin JA Dearani JA Rowin EJ Maron MS Sherrid MV. Management of hypertrophic cardiomyopathy: JACC State-of-the-Art Review.J Am Coll Cardiol. 2022; 79: 390-414Google BJ. Clinical course and management of hypertrophic cardiomyopathy.N Engl J Med. 2018; 379: 655-668Google EJ Maron BJ Patel Koethe B S Maron MS. Outcomes in patients with hypertrophic cardiomyopathy and left ventricular Am Coll Cardiol. 2020; Scholar The primary for HCM management is and of the personalized treatment strategies that have reduced HCM mortality. The to of these management to HCM can and other as well as the to effectively such as surgical heart and BJ Rowin EJ Maron MS. of hypertrophic Heart 2018; Scholar Nevertheless, progress in these is with the of HCM centers in the United and in and a of all patients with HCM BJ. and of hypertrophic cardiomyopathy centers in the in J Cardiol. 2015; BJ Hypertrophic cardiomyopathy in the on Heart 2014; Scholar the last several years, substantial has emerged the and clinical course of to that HCM is rare and for shortened life expectancy, this disease is now that with a potential for normal or extended and to low disease-related mortality (0.5%/year) largely confined to refractory heart failure in the of BJ Rowin EJ Casey SA Maron MS. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice.JAMA Cardiol. 2016; 1: 98-105Google Scholar

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