Evolution of Hypertrophic Cardiomyopathy to a Contemporary Treatable Disease
2012; Lippincott Williams & Wilkins; Volume: 126; Issue: 13 Linguagem: Inglês
10.1161/circulationaha.112.123174
ISSN1524-4539
AutoresBarry J. Maron, Eugene Braunwald,
Tópico(s)Viral Infections and Immunology Research
ResumoHomeCirculationVol. 126, No. 13Evolution of Hypertrophic Cardiomyopathy to a Contemporary Treatable Disease Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessCase ReportPDF/EPUBEvolution of Hypertrophic Cardiomyopathy to a Contemporary Treatable Disease Barry J. Maron, MD and Eugene Braunwald, MD Barry J. MaronBarry J. Maron From the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M.); and The TIMI Study Group, Brigham and Women's Hospital, Boston, MA (E.B.). and Eugene BraunwaldEugene Braunwald From the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M.); and The TIMI Study Group, Brigham and Women's Hospital, Boston, MA (E.B.). Originally published25 Sep 2012https://doi.org/10.1161/CIRCULATIONAHA.112.123174Circulation. 2012;126:1640–1644Case 1: The patient is now a 56-year-old man with nonobstructive hypertrophic cardiomyopathy (HCM). At 35 years of age, his brother (aged 39) died suddenly with a new diagnosis of HCM made at autopsy. A diagnostic evaluation ensued, and nonobstructive HCM was identified with a ventricular septal thickness of 31 mm. Because of the family history of sudden death (and his marked hypertrophy), an implantable cardioverter- defibrillator (ICD) was implanted for primary prevention of sudden death. Five years later, during sleep (1:00 am), the ICD aborted an episode of ventricular fibrillation (VF) and restored sinus rhythm (Figure 1). Nine years passed without incident, and at 50 years of age, an identical VF event occurred during sleep, similarly terminated by a defibrillation shock. Now, at 56 years of age, the patient continues to be asymptomatic and active. His clinical course with HCM has been punctuated only by these 2 unpredictable sudden-death events effectively treated by an ICD prophylactically implanted for high-risk status.Download figureDownload PowerPointFigure 1. Primary prevention of sudden death with the ICD that aborted an episode of VT/VF at 41 years of age (appropriate shock #1). A, After 4 sinus beats, VT begins abruptly at 200 bpm; B, Defibrillator senses VT and begins an 8-second charge. C, VT deteriorates into VF. D, Defibrillator appropriately discharges a 20-J shock (bar) and restores sinus rhythm within 1 beat. SD indicates sudden death; ICD, implantable cardioverter defibrillator; VT, ventricular tachycardia; and VF, ventricular fibrillation. Adapted and reproduced from Maron et al,1 with permission from the publisher. Copyright © 2007, American Medical Association.Case 2: The patient is a 53-year-old female elementary school teacher with obstructive HCM. She presented at 44 years of age, asymptomatic, but with a strong family history of sudden deaths due to HCM in 2 brothers (aged 20 and 34 years, respectively). An ICD was implanted prophylactically, and 2 years later (at 46 years of age), VF was defibrillated during sleep. Over the next 6 years, 4 more appropriate interventions for ventricular tachycardia (VT)/VF followed.Ventricular septal thickness is 21 mm; left ventricular outflow obstruction is absent at rest, although provoked with exercise (gradient, 70 mm Hg). By 51 years of age, exertional dyspnea and functional limitation had progressed despite maximum medical management, with the patient disabled in New York Heart Association functional class III. Surgical septal myectomy abolished both the outflow gradient and the patient's heart failure–related symptoms. Once again, at 53 years of age, she is asymptomatic and active, having experienced 2 adverse disease pathways, ie, sudden-death risk, and heart failure due to outflow tract obstruction, both of which were amenable to effective treatment strategies.IntroductionCardiovascular medicine advances rapidly, sometimes with blinding speed, to create new and important treatment initiatives. Coronary artery disease is at the core of cardiovascular practice, arguably an epidemic in this and much of the last century. But what of less common inherited heart diseases, historically overlooked, and sometimes seemingly lost among the vast number of patients with ischemic heart disease?HCM is perhaps the most prominent among the genetic heart diseases.2,3 This is the story of how this condition, once regarded as rare and odd, but interesting (with grim prognosis) has come to be recognized as a relatively common, contemporary, and treatable clinical entity with established consensus recommendations and guidelines (Figures 1 through 3).4–7 This evolution represents an instructive message for the practicing community, and for their patients with HCM.Historical HCMHCM is now recognized as the most common cause of sudden cardiac death in the young (including trained athletes), present in at least 1 of 500 people in the general population, and probably affecting as many as 750 000 Americans.8 However, to arrive at its current maturity, HCM has experienced a sometimes rocky and unpredictable course through its first 50 years.9,10 That journey has been punctuated by a myriad of obstacles, including periodic misunderstanding and contentious debate, and an often confusing and conflicted literature. Early, but now obsolete, discourse even suggested that HCM was not a distinct disease entity, or that true mechanical obstruction to left ventricular outflow did not exist.9,10 A confusing litany of names (n=75) dominated the literature to describe the same condition, eg, among those idiopathic hypertrophic subaortic stenosis and hypertrophic obstructive cardiomyopathy now replaced by one, ie, HCM.11The early years of HCM (1960–1965) were characterized by astute and novel clinical descriptions of a previously unrecognized clinical entity, first largely at the National Institutes of Health Clinical Center. Initially, there was a notable paucity of treatment options available.2 Indeed, one of the first published articles on this disease from National Institutes of Health describing dynamic left ventricular outflow tract obstruction could only view treatment strategies in futile terms12: "At this time, we are aware of no method of management that can specifically and favorably influence the course of a patient with idiopathic ventricular hypertrophy."Although this was an accurate assessment at that time (ie, 1959) β-blockers13,14 and surgical myectomy15 were soon introduced. However, the cardiology community was slow to accept invasive treatment for HCM. For example, after an early but brief experience, myectomy was abandoned in the United Kingdom for 30 years.An unfortunate notion took hold, which surprisingly persists with some frequency today, ie, the myth that HCM is an unrelenting disease without definitive or effective treatment options. HCM acquired the stigma of a disease characterized by an unpredictable downhill course affecting most patients. Perhaps contributing to this misconception now are the highly publicized but very uncommon athletic field deaths due to HCM in young previously undiagnosed sports participants.16Diseases such as HCM are susceptible to misunderstanding. Old concepts penetrate the consciousness and become entrenched, seemingly difficult to revise despite evidence to the contrary. Relatively uncommon conditions in clinical practice, such as HCM, represent challenges to individual cardiologists in order to best serve their patients by acquiring sufficient experience and exposure to fully appreciate nuances of the broad disease spectrum and its management.Contemporary HCMThe clinical profile and treatment armamentarium of HCM has undergone a vast transformation. First, we now recognize that most genetically affected individuals are probably not identified clinically, including many who achieve normal life expectancy into their 70s, 80s, and even 90s, without disability or the necessity for major management interventions.4–7,17 Indeed, largely because of a generally heightened suspicion of the disease due in part to dissemination of advanced imaging modalities, asymptomatic HCM patients are fortuitously identified more frequently at relatively advanced ages.In addition, risk for HCM-related complications (including sudden death) is paradoxically low in older patients after 60 years of age.18 However, even now most recently diagnosed HCM patients seem surprised (and reassured) to learn this important principle about aging in HCM, having received a generally unfavorable message about their disease.On the other hand, many patients who present to hospitals and clinics experience (or are at risk for) HCM-related complications1,4–7,19–21 (Figure 3). The clinical course may progress along ≥1 adverse disease pathways, often drawing on treatment options designed for patients with other cardiac diseases (Figure 3). Specific management strategies are dictated by these pathways; in principle, one treatment does not fit all patients with this heterogeneous disease.Prevention of Sudden DeathSudden death is the most visible and devastating consequence of HCM, recognized since the initial pathological description in 1958.3 However, prevention of sudden death is now a realistic aspiration for young patients with HCM.1,21 Although the history of HCM is encumbered by sudden death in young patients in the absence of therapy capable of averting that risk, systematic strategy has evolved in the past decade to protect such patients with ICDs.1,21By virtue of automatically sensing and terminating VT/VF, the ICD is the only available treatment option capable of definitively prolonging life in HCM, thereby altering the natural history of the disease for many patients (Figure 1). The ICD in HCM registry, comprising high-risk patients, reports a primary prevention intervention rate of 4%/year, similar in adults and in children.1 Although the ICD is a proven life-saving measure in HCM, the challenge remains to select more precisely those patients who will benefit from this therapy, including the small but important subset in which conventional risk markers are not predictive, but in whom sudden-death events may nevertheless occur.21 Therefore, identifying more at-risk patients with novel markers continues to be an important goal for which contrast-cardiovascular MRI with extensive late-gadolinium enhancement (presumably myocardial scarring) shows significant promise.22Reversal of Heart FailureIn HCM, severe heart failure and exertional disability due to left ventricular outflow obstruction are potentially reversible20 (Figure 2). Surgical septal myectomy, pioneered by Andrew Morrow at National Institutes of Health23 (and also advanced by others including Bill Williams in Toronto, John Kirklin and now Joseph Dearani, both of Mayo Clinic), has evolved to a safe procedure, effective in restoring full quality of life by normalizing left ventricular pressures.Download figureDownload PowerPointFigure 2. Clinical significance of LV outflow obstruction in HCM. Top, Patients with obstruction at rest (gradient, ≥30 mm Hg) are at greater risk for severe progressive heart failure, or heart failure or stroke death in comparison with patients without obstruction. HCM indicates hypertrophic cardiomyopathy; LV, left ventricular; NYHA, New York Heart Association; RR, relative risk. Reproduced with permission from Maron et al.19Bottom, Surgical septal myectomy with abolition of LV outflow gradient is associated with long-term survival similar to that expected in the general US population, and exceeding that in symptomatic but nonoperated patients with obstructive HCM. Reproduced from Ommen et al,20with permission from the publisher. Copyright © 2005, New England Journal of Medicine.Download figureDownload PowerPointFigure 3. Adverse clinical pathways in HCM. Each of these disease complications is associated with ≥1 potentially effective treatment options. AF indicates atrial fibrillation; ICD, implantable cardioverter defibrillator; HCM, hypertrophic cardiomyopathy; and RFA, radiofrequency ablation.Initially, myectomy, even in the most experienced hands, was encumbered by a 5% to 8% operative mortality, which undoubtedly tempered enthusiasm for this surgery. Consequently, the number of myectomy operations remained relatively small.Only during the past 10 to 15 years has the frequency and importance of obstruction within the clinical spectrum of HCM been fully recognized19,24 (Figure 2). Surgical septal myectomy has become an attractive and accessible option, evident by the dramatic increase in the number of operations performed annually at major centers.25,26 This renewed interest in myectomy is attributable to a strikingly low operative mortality of ≤1% due to contemporary myocardial preservation techniques, and increased surgical experience and innovation.25,26 For example, development of the extended myectomy approach recognizes the important contribution of submitral structures to left ventricular outflow tract obstruction.4–7,27 Notably, in addition to relief of outflow obstruction with enhanced quality of life, the myectomy operation also extends longevity with survival similar to that expected in the general population, and exceeding that of nonoperated symptomatic patients with obstructive HCM20 (Figure 2).A percutaneous interventional strategy, ie, alcohol septal ablation, provides an important alternative to myectomy,5–7,28 selectively for those patients who are older and/or judged suboptimal surgical candidates because of comorbidity, or a strong personal aversion to surgery.4–7Although alcohol ablation has proved effective in reducing outflow gradient and symptoms, it produces a potentially arrhythmogenic alcohol-induced transmural myocardial infarction, and in this respect differs significantly from myectomy. Nevertheless, the exuberance with which alcohol ablation has been embraced by the interventional cardiology community has stimulated visibility for HCM in general, and paradoxically has increased the focus on relieving outflow obstruction with myectomy.25,26,28,29Rare patients who experience particularly advanced drug refractory and irreversible heart failure usually associated with systolic dysfunction and adverse left ventricular remodeling, due to microvascular ischemia and diffuse scarring (the end-stage phase), are candidates for heart transplantation as the only definitive treatment option.30 Posttransplant survival in HCM patients is equivalent to that of other heart diseases, 85% at 1 year, 75% at 5 years, and 61% at 10 years, in comparison with 82%, 70%, and 49%, respectively, in non-HCM transplant recipients.31Atrial Fibrillation and StrokeAtrial fibrillation (AF) is the most common sustained arrhythmia in HCM, occurring in ≈20% of patients (4-fold that of the general population).32 In some patients, AF can be linked to progressive heart failure, and embolic stroke (although not to risk for sudden unexpected death). Management strategies for AF in HCM are similar to those for non-HCM patients and have similar efficacy. Standard pharmacological agents are administered aggressively to decrease the frequency of paroxysmal episodes, maintain sinus rhythm, control ventricular rate, and prevent embolic stroke with prophylactic anticoagulation. Selected patients with refractory AF are eligible for radiofrequency catheter ablation to obliterate the AF focus, a procedure that. at this early juncture, appears successful in restoring sinus rhythm and improving symptomatic status in the short term in >50% of patients.33Conclusions and PerspectivesHCM has emerged from an era of substantial misunderstanding, stigma, and pessimism to become a contemporary cardiovascular disease in which each of its adverse disease pathways and complications are associated with widely available and effective management strategies. These major therapeutic interventions available to HCM patients are capable of changing the natural history of the disease, including the prevention of sudden cardiac death (with the ICD) and reversal of heart failure with restored quality of life and extended longevity (with surgical myectomy).Notably, the mature HCM management strategies addressed here are directly attributable to purely clinical principles and research, and are independent of the notable advances in molecular biology. The latter have defined the gene mutations encoding sarcomeric proteins that cause HCM, with the clinical impact largely confined to family screening,34 but so far have assumed little role in the treatment and prognostic assessment of clinically expressed HCM.Much work remains for this complex genetic disease, particularly in the area of risk stratification. Nevertheless, the ultimate objective of providing HCM patients with the opportunity to achieve normal life expectancy with good quality of life has become a realistic aspiration through the important contributions of many clinical practitioners and investigators since the infancy of this disease >5 decades ago.DisclosuresDr Maron is a Consultant for GeneDx and research grantee for Medtronic. Dr Braunwald reports no conflicts.FootnotesCorrespondence to Barry J. Maron, MD, Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, 920 East 28th St, Suite 620, Minneapolis, MN 55407. E-mail hcm.[email protected]orgReferences1. Maron BJ, Spirito P, Shen W-K, Haas TS, Formisano F, Link MS, Epstein AE, Almquist AK, Daubert JP, Lawrenz T, Boriani G, Estes NAM, Favale S, Piccininno M, Winters SL, Santini M, Betocchi S, Arribas F, Sherrid MV, Buja G, Semsarian C, Bruzzi P. 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Kubo T, Baba Y, Hirota T, Tanioka K, Yamasaki N, Doi Y and Kitaoka H (2017) Prognostic Significance of Non-Dilated Left Ventricular Size and Mitral Regurgitation in Patients With Dilated Phase of Hypertrophic Cardiomyopathy, International Heart Journal, 10.1536/ihj.16-109, 58:1, (63-68), . Castagnoli H, Ferrantini C, Coppini R, Passeri A, Baldini K, Berti V, Cecchi F, Olivotto I and Sciagrà R (2016) Role of quantitative myocardial positron emission tomography for risk stratification in patients with hypertrophic cardiomyopathy: a 2016 reappraisal, European Journal of Nuclear Medicine and Molecular Imaging, 10.1007/s00259-016-3465-7, 43:13, (2413-2422), Online publication date: 1-Dec-2016. Maron M, Rowin E, Olivotto I, Casey S, Arretini A, Tomberli B, Garberich R, Link M, Chan R, Lesser J and Maron B (2016) Contemporary Natural History and Management of Nonobstructive Hypertrophic Cardiomyopathy, Journal of the American College of Cardiology, 10.1016/j.jacc.2016.01.023, 67:12, (1399-1409), Online publication date: 1-Mar-2016. Maron B and Fox P (2015) Hypertrophic cardiomyopathy in man and cats, Journal of Veterinary Cardiology, 10.1016/j.jvc.2015.03.007, 17, (S6-S9), Online publication date: 1-Dec-2015. Maron M (2015) Family History of Sudden Death Should Be a Primary Indication for Implantable Cardioverter Defibrillator in Hypertrophic Cardiomyopathy, Canadian Journal of Cardiology, 10.1016/j.cjca.2015.05.004, 31:11, (1402-1406), Online publication date: 1-Nov-2015. Pennacchini E, Musumeci M, Conte M, Stöllberger C, Formisano F, Bongioanni S, Francia P, Volpe M and Autore C (2015) Electrocardiographic evolution in patients with hypertrophic cardiomyopathy who develop a left ventricular apical aneurysm, Journal of Electrocardiology, 10.1016/j.jelectrocard.2015.06.004, 48:5, (818-825), Online publication date: 1-Sep-2015. Maron B, Casey S, Chan R, Garberich R, Rowin E and Maron M (2015) Independent Assessment of the European Society of Cardiology Sudden Death Risk Model for Hypertrophic Cardiomyopathy, The American Journal of Cardiology, 10.1016/j.amjcard.2015.05.047, 116:5, (757-764), Online publication date: 1-Sep-2015. Maron B (2015) Importance and Feasibility of Creating Hypertrophic Cardiomyopathy Centers in Developing Countries: The Experience in India, The American Journal of Cardiology, 10.1016/j.amjcard.2015.04.027, 116:2, (332-334), Online publication date: 1-Jul-2015. Maron B (2015) Historical Perspectives on the Implantable Cardioverter–Defibrillator and Prevention of Sudden Death in Hypertrophic Cardiomyopathy, Cardiac Electrophysiology Clinics, 10.1016/j.ccep.2015.03.001, 7:2, (165-171), Online publication date: 1-Jun-2015. Terauchi Y, Kubo T, Baba Y, Hirota T, Tanioka K, Yamasaki N, Furuno T and Kitaoka H (2015) Gender differences in the clinical features of hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations, Journal of Cardiology, 10.1016/j.jjcc.2014.07.010, 65:5, (423-428), Online publication date: 1-May-2015. Spirito P (2015) The Dawn of a Better Day for Patients With Hypertrophic Cardiomyopathy, Journal of the American College of Cardiology, 10.1016/j.jacc.2015.03.516, 65:18, (1929-1930), Online publication date: 1-May-2015. Maron B, Rowin E, Casey S, Link M, Lesser J, Chan R, Garberich R, Udelson J and Maron M (2015) Hypertrophic Cardiomyopathy in Adulthood Associated With Low Cardiovascular Mortality With Contemporary Management Strategies, Journal of the American College of Cardiology, 10.1016/j.jacc.2015.02.061, 65:18, (1915-1928), Online publication date: 1-May-2015. Maron B, Weiner H, Maron M and Roberts W (2015) Surviving Malignant Hypertrophic Cardiomyopathy With all Major Complications in a Single Patient, The American Journal of Cardiology, 10.1016/j.amjcard.2014.11.004, 115:3, (402-404), Online publication date: 1-Feb-2015. Andrews M, Giger M and Roman B (2014) Manganese-enhanced MRI detection of impaired calcium regulation in a mouse model of cardiac hypertrophy, NMR in Biomedicine, 10.1002/nbm.3249, 28:2, (255-263), Online publication date: 1-Feb-2015. Maron B and Maron M (2015) The 20 advances that have defined contemporary hypertrophic cardiomyopathy, Trends in Cardiovascular Medicine, 10.1016/j.tcm.2014.09.004, 25:1, (54-64), Online publication date: 1-Jan-2015. Codreanu A, Delagardelle C, Groben L and Urhausen A (2015) Sport bei Myokarderkrankungen Sportkardiologie, 10.1007/978-3-662-43711-7_22, (291-306), . Maron B and Nishimura R (2014) Revisiting Arrhythmic Risk After Alcohol Septal Ablation, JACC: Heart Failure, 10.1016/j.jchf.2014.07.008, 2:6, (637-640), Online publication date: 1-Dec-2014. Maron B and Kalra A (2014) Hypertrophic cardiomyopathy in the developing world: focus on India, European Heart Journal, 10.1093/eurheartj/ehu280, 35:36, (2492-2495), Online publication date: 2-Sep-2014. Maron B, Ommen S, Semsarian C, Spirito P, Olivotto I and Maron M (2014) Hypertrophic Cardiomyopathy, Journal of the American College of Cardiology, 10.1016/j.jacc.2014.05.003, 64:1, (83-99), Online publication date: 1-Jul-2014. (2014) Further reading A Guide to Hypertrophic Cardiomyopathy, 10.1002/9781118725498.furread, (130-134) Song J (2014) Role of Noninvasive Imaging Modalities to Better Understand the Mechanism of Left Ventricular Outflow Tract Obstruction and Tailored Lesion-Specific Treatment Options, Circulation Journal, 10.1253/circj.CJ-14-0524, 78:8, (1808-1815), . Maron B (2014) Ventricular Arrhythmias in Hypertrophic Cardiomyopathy Cardiac Electrophysiology: From Cell to Bedside, 10.1016/B978-1-4557-2856-5.00086-8, (873-883), . Olivotto I, Tomberli B, Spoladore R, Mugelli A, Cecchi F and Camici P (2013) Hypertrophic cardiomyopathy: The need for randomized trials, Global Cardiology Science and Practice, 10.5339/gcsp.2013.31, 2013:3, (31), Online publication date: 1-Sep-2013. Dixon S and Safian R (2013) The Year in Interventional Cardiology, Journal of the American College of Cardiology, 10.1016/j.jacc.2013.01.040, 61:15, (1637-1652), Online publication date: 1-Apr-2013. Maron B and Maron M (2013) Hypertrophic cardiomyopathy, The Lancet, 10.1016/S0140-6736(12)60397-3, 381:9862, (242-255), Online publication date: 1-Jan-2013. September 25, 2012Vol 126, Issue 13 Advertisement Article InformationMetrics © 2012 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.112.123174PMID: 23008470 Originally publishedSeptember 25, 2012 Keywordsimplanted cardioverter defibrillatorsgeneticssurgerysudden deathhypertrophic cardiomyopathyatrial fibrillationPDF download Advertisement SubjectsComputerized Tomography (CT)Genetics
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