Benefits of surgery in obstructive hypertrophic cardiomyopathy: bring septal myectomy back for European patients
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TLDR
It is the aspiration that this discussion will generate reconsideration and resurgence of interest in surgical septal myectomy as a treatment option for severely symptomatic obstructive HCM patients within Europe.Abstract:
Hypertrophic cardiomyopathy (HCM), a heterogeneous genetic heart disease with global distribution, is an important cause of heart failure disability at any age. For 50 years, surgical septal myectomy has been the preferred and primary treatment strategy for most HCM patients with progressive, drug refractory functional limitation due to left ventricular (LV) outflow tract obstruction. With very low surgical mortality at experienced centres, septal myectomy reliably abolishes impedance to LV outflow and heart failure-related symptoms, restores quality of life, and importantly is associated with long-term survival similar to that in the general population. Nevertheless, alternatives to surgical management are necessary for selected HCM patients. For example, after a brief flirtation with dual-chamber pacing 20 years ago, percutaneous alcohol septal ablation has garnered a large measure of enthusiasm and a dedicated following in the interventional cardiology community, achieving benefits for patients, paradoxically, by virtue of producing a transmural myocardial infarct. However, an unintended consequence has been the virtual obliteration of the surgical option for HCM patients in Europe, where several robust myectomy programmes once existed. Therefore, clear differences are now evident internationally regarding management strategies for symptomatic obstructive HCM. The surgical option is now unavailable to many patients based solely on geography, including some who would likely benefit more substantially from surgical myectomy than from catheter-based alcohol ablation. It is our aspiration that this discussion will generate reconsideration and resurgence of interest in surgical septal myectomy as a treatment option for severely symptomatic obstructive HCM patients within Europe.read more
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Mechanisms of disease: hypertrophic cardiomyopathy
TL;DR: Several potential pathways are discussed, which include altered calcium cycling and sarcomeric calcium sensitivity, increased fibrosis, disturbed biomechanical stress sensing, and impaired cardiac energy homeostasis, which could result in greater specificity and success of therapies for patients with HCM.
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Contemporary Natural History and Management of Nonobstructive Hypertrophic Cardiomyopathy
Martin S. Maron,Ethan J. Rowin,Iacopo Olivotto,Susan A. Casey,A Arretini,Benedetta Tomberli,Ross Garberich,Mark S. Link,Raymond H. Chan,John R. Lesser,Barry J. Maron +10 more
TL;DR: HCM patients with nonobstructive disease appear to experience a relatively benign clinical course, associated with a low risk for advanced heart failure symptoms, other disease complications, and HCM-related mortality, and largely without the requirement for major treatment interventions.
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Long-term clinical outcome after alcohol septal ablation for obstructive hypertrophic cardiomyopathy: results from the Euro-ASA registry.
Josef Veselka,Morten Kvistholm Jensen,Max Liebregts,Jaroslav Januška,Jan Krejčí,Thomas Bartel,Maciej Dabrowski,Peter Riis Hansen,Vibeke M. Almaas,Hubert Seggewiss,Dieter Horstkotte,Pavol Tomašov,Radka Adlová,Henning Bundgaard,Robbert C. Steggerda,Jurriën M. ten Berg,Lothar Faber +16 more
TL;DR: The Euro-ASA registry demonstrated low peri-procedural and long-term mortality after ASA and provided durable relief of symptoms and a reduction of LV outflow tract obstruction in selected and highly symptomatic patients with obstructive HCM.
Journal ArticleDOI
A contemporary European experience with surgical septal myectomy in hypertrophic cardiomyopathy
Attilio Iacovoni,Paolo Spirito,Caterina Simon,Maria Iascone,Giovanni Di Dedda,Paolo De Filippo,Samuele Pentiricci,Luca Boni,Michele Senni,Antonello Gavazzi,Paolo Ferrazzi +10 more
TL;DR: The results show that the development of a myectomy programme at a centre without previous experience with this procedure is feasible and can lead to highly favourable clinical results.
References
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Journal ArticleDOI
Hypertrophic cardiomyopathy: A Systematic review
TL;DR: An appreciation that HCM, although an important cause of death and disability at all ages, does not invariably convey ominous prognosis and is compatible with normal longevity should dictate a large measure of reassurance for many patients.
Journal ArticleDOI
American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines
Barry J. Maron,William J. McKenna,Gordon K. Danielson,Lukas Kappenberger,Horst J. Kuhn,Christine E. Seidman,Pravin M. Shah,William H. Spencer,Paolo Spirito,Folkert J. Ten Cate,E. Douglas Wigle,Robert A. Vogel,Jonathan Abrams,Eric R. Bates,Bruce R. Brodie,Peter G. Danias,Gabriel Gregoratos,Mark A. Hlatky,Judith S. Hochman,Sanjiv Kaul,Robert C. Lichtenberg,Jonathan R. Lindner,Robert A. O'Rourke,Gerald M. Pohost,Richard S. Schofield,Cynthia M. Tracy,William L. Winters,Werner Klein,Silvia G. Priori,Angeles Alonso-Garcia,Carina Blomström-Lundqvist,Guy De Backer,Jaap W. Deckers,Markus Flather,Jaromír Hradec,Ali Oto,Alexander Parkhomenko,Sigmund Silber,Adam Torbicki +38 more
TL;DR: In this paper, the authors present a general approach for the natural history and clinical course of clinical diagnostics in the context of vascular flow outflow and genetic and molecular diagnostics.
Journal ArticleDOI
Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy
Barry J. Maron,Paolo Spirito,Win Kuang Shen,Tammy S. Haas,Francesco Formisano,Mark S. Link,Andrew E. Epstein,Adrian K. Almquist,James P. Daubert,Thorsten Lawrenz,Giuseppe Boriani,N.A. Mark Estes,Stefano Favale,Marco Piccininno,Stephen L. Winters,Massimo Santini,Sandro Betocchi,Fernando Arribas,Mark V. Sherrid,Gianfranco Buja,Christopher Semsarian,Paolo Bruzzi +21 more
TL;DR: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm.
Journal ArticleDOI
Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy
TL;DR: A novel catheter-based technique that may replace surgical myocardial reduction in some patients with hypertrophic obstructive cardiomyopathy, which aims at selective destruction of the hypertrophied part of the left side of the intraventricular septum.
Journal ArticleDOI
Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.
Steve R. Ommen,Barry J. Maron,Iacopo Olivotto,Martin S. Maron,Franco Cecchi,Sandro Betocchi,Bernard J. Gersh,Michael J. Ackerman,Robert B. McCully,Joseph A. Dearani,Hartzell V. Schaff,Gordon K. Danielson,A. Jamil Tajik,Rick A. Nishimura +13 more
TL;DR: Surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation.
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Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.
American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines
Barry J. Maron,William J. McKenna,Gordon K. Danielson,Lukas Kappenberger,Horst J. Kuhn,Christine E. Seidman,Pravin M. Shah,William H. Spencer,Paolo Spirito,Folkert J. Ten Cate,E. Douglas Wigle,Robert A. Vogel,Jonathan Abrams,Eric R. Bates,Bruce R. Brodie,Peter G. Danias,Gabriel Gregoratos,Mark A. Hlatky,Judith S. Hochman,Sanjiv Kaul,Robert C. Lichtenberg,Jonathan R. Lindner,Robert A. O'Rourke,Gerald M. Pohost,Richard S. Schofield,Cynthia M. Tracy,William L. Winters,Werner Klein,Silvia G. Priori,Angeles Alonso-Garcia,Carina Blomström-Lundqvist,Guy De Backer,Jaap W. Deckers,Markus Flather,Jaromír Hradec,Ali Oto,Alexander Parkhomenko,Sigmund Silber,Adam Torbicki +38 more