Review on sudden death risk reduction after septal reduction therapies in hypertrophic obstructive cardiomyopathy

  • Angelos G. RigopoulosEmail author
  • Muhammad Ali
  • Elena Abate
  • Marios Matiakis
  • Hannes Melnyk
  • Sophie Mavrogeni
  • Dionyssios Leftheriotis
  • Boris Bigalke
  • Michel Noutsias


Treatment of left ventricular outflow tract (LVOT) obstruction in hypertrophic obstructive cardiomyopathy (HOCM) with septal reduction, either with myectomy or alcohol septal ablation, is aiming to reduce the LVOT gradient and improve symptoms in patients who are refractory to or do not tolerate medical treatment. Apart from contributing to the evolution to heart failure, LVOT obstruction is considered a risk factor for sudden cardiac death (SCD). Both septal reduction treatments have been proven effective in reducing symptoms and seem to improve survival, which has been shown equal to the expected in the normal population. SCD is probably reduced after septal reduction, implying that LVOT obstruction is a major factor predisposing to ventricular tachyarrhythmias. Although available algorithms for SCD stratification have not been tested in patients after septal reduction treatments, effective treatment improves SCD risk profile substantially. Furthermore, high-risk patients with already implanted implantable cardioverter defibrillators (ICDs) before septal reduction show very low appropriate ICD shock rate after effective treatment. It should be noted, however, that the best outcomes for septal myectomy or ablation have been reported in HOCM patients treated in high-volume centres, which substantiates the need to refer patients to centres with high procedural expertise.


Hypertrophic obstructive cardiomyopathy Outcome Surgical myectomy Treatment Alcohol septal ablation Sudden cardiac death Risk stratification Prognosis 



American College of Cardiology


American Heart Association


Alcohol septal ablation


Cardiac magnetic resonance


European Society of Cardiology


Hypertrophic cardiomyopathy


Hypertrophic obstructive cardiomyopathy


Implantable cardioverter defibrillator


Late gadolinium enhancement


Left ventricular


Left ventricular outflow tract


Systolic anterior motion


Sudden cardiac death


Compliance with ethical standards

Conflict of interest

AR and MA have received honoraria for presentations from AstraZeneca. MN has received grants by the Deutsche Forschungsgemeinschaft (DFG) through the Sonderforschungsbereich Transregio 19 “Inflammatory Cardiomyopathy” (SFB TR19) (TP B2), and by the University Hospital Giessen and Marburg Foundation Grant “T cell functionality” (UKGM 10/2009). MP has received a grant by the DFG (SFB TR19 TP A2). MN has been consultant to the IKDT (Institute for Cardiac Diagnosis and Therapy GmbH, Berlin) 2004–2008 and has received honoraria for presentations and/or participated in advisory boards from AstraZeneca, Bayer, Boehringer Ingelheim, Fresenius, Miltenyi Biotech, Novartis, Pfizer and Zoll.

Supplementary material

10741_2018_9767_MOESM1_ESM.docx (16 kb)
ESM 1 (DOCX 15.9 kb)


  1. 1.
    Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P et al (2014) 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 35:2733–2779CrossRefGoogle Scholar
  2. 2.
    Maron MS, Olivotto I, Zenovich AG, Link MS, Pandian NG, Kuvin JT, Nistri S, Cecchi F, Udelson JE, Maron BJ (2006) Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation 114:2232–2239CrossRefGoogle Scholar
  3. 3.
    Maron MS, Olivotto I, Betocchi S, Casey SA, Lesser JR, Losi MA, Cecchi F, Maron BJ (2003) Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 348:295–303CrossRefGoogle Scholar
  4. 4.
    Wigle ED (1987) Hypertrophic cardiomyopathy: a 1987 viewpoint. Circulation 75:311–322CrossRefGoogle Scholar
  5. 5.
    Sherrid MV (2006) Pathophysiology and treatment of hypertrophic cardiomyopathy. Prog Cardiovasc Dis 49:123–151CrossRefGoogle Scholar
  6. 6.
    Elliott PM, Poloniecki J, Dickie S, Sharma S, Monserrat L, Varnava A, Mahon NG, McKenna WJ (2000) Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 36(7):2212–2218Google Scholar
  7. 7.
    Ommen SR, Maron BJ, Olivotto I, Maron MS, Cecchi F, Betocchi S, Gersh BJ, Ackerman MJ, McCully RB, Dearani JA, Schaff HV, Danielson GK, Tajik AJ, Nishimura RA (2005) Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol 46:470–476CrossRefGoogle Scholar
  8. 8.
    Woo A, Williams WG, Choi R, Wigle ED, Rozenblyum E, Fedwick K, Siu S, Ralph-Edwards A, Rakowski H (2005) Clinical and echocardiographic determinants of long-term survival after surgical myectomy in obstructive hypertrophic cardiomyopathy. Circulation 111:2033–2041CrossRefGoogle Scholar
  9. 9.
    Sorajja P, Ommen SR, Holmes DR Jr, Dearani JA, Rihal CS, Gersh BJ, Lennon RJ, Nishimura RA (2012) Survival after alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Circulation 126:2374–2380CrossRefGoogle Scholar
  10. 10.
    Veselka J, Jensen MK, Liebregts M, Januska J, Krejci J, Bartel T, Dabrowski M, Hansen PR, Almaas VM, Seggewiss H, Horstkotte D, Tomasov P, Adlova R, Bundgaard H, Steggerda R, ten Berg J, Faber L (2016) Long-term clinical outcome after alcohol septal ablation for obstructive hypertrophic cardiomyopathy: results from the Euro-ASA registry. Eur Heart J 37:1517–1523CrossRefGoogle Scholar
  11. 11.
    Westphal JG, Rigopoulos AG, Bakogiannis C, Ludwig SE, Mavrogeni S, Bigalke B, Doenst T, Pauschinger M, Tschöpe C, Schulze PC, Noutsias M (2017) The MOGE(S) classification for cardiomyopathies: current status and future outlook. Heart Fail Rev 22:743–752CrossRefGoogle Scholar
  12. 12.
    Ball W, Ivanov J, Rakowski H, Wigle ED, Linghorne M, Ralph-Edwards A, Williams WG, Schwartz L, Guttman A, Woo A (2011) Long-term survival in patients with resting obstructive hypertrophic cardiomyopathy comparison of conservative versus invasive treatment. J Am Coll Cardiol 58:2313–2321CrossRefGoogle Scholar
  13. 13.
    Elliott PM, Gimeno JR, Tome MT, Shah J, Ward D, Thaman R et al (2006) Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy. Eur Heart J 27:1933–1941CrossRefGoogle Scholar
  14. 14.
    Maron BJ (2018) Clinical course and management of hypertrophic cardiomyopathy. N Engl J Med 379:655–668CrossRefGoogle Scholar
  15. 15.
    Wang N, Xie A, Tjahjono R, Tian DH, Phan S, Yan TD, Bajona P, Phan K (2017) Implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: an updated systematic review and meta-analysis of outcomes and complications. Ann Cardiothorac Surg 6:298–306CrossRefGoogle Scholar
  16. 16.
    Schinkel AF, Vriesendorp PA, Sijbrands EJ, Jordaens LJ, ten Cate FJ, Michels M (2012) Outcome and complications after implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: systematic review and meta-analysis. Circ Heart Fail 5:552–559CrossRefGoogle Scholar
  17. 17.
    Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE et al (2003) 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. Eur Heart J 24:1965–1991CrossRefGoogle Scholar
  18. 18.
    Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, 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 on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2011;58:e212–e260Google Scholar
  19. 19.
    O’Mahony C, Jichi F, Pavlou M, Monserrat L, Anastasakis A, Rapezzi C, Biagini E, Gimeno JR, Limongelli G, McKenna WJ, Omar RZ, Elliott PM, for the Hypertrophic Cardiomyopathy Outcomes Investigators (2014) A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J 35:2010–2020CrossRefGoogle Scholar
  20. 20.
    Vriesendorp PA, Schinkel AF, Liebregts M, Theuns DA, van Cleemput J, Ten Cate FJ et al (2015) Validation of the 2014 European Society of Cardiology Guidelines risk prediction model for the primary prevention of sudden cardiac death in hypertrophic cardiomyopathy. Circ Arrhythm Electrophysiol 8:829–835CrossRefGoogle Scholar
  21. 21.
    Fernandez A, Quiroga A, Ochoa JP, Mysuta M, Casabe JH, Biagetti M et al (2016) Validation of the 2014 European Society of Cardiology sudden cardiac death risk prediction model in hypertrophic cardiomyopathy in a reference center in South America. Am J Cardiol 118:121–126CrossRefGoogle Scholar
  22. 22.
    O’Mahony C, Jichi F, Ommen SR, Christiaans I, Arbustini E, Garcia-Pavia P et al (2018) International external validation study of the 2014 European Society of Cardiology Guidelines on sudden cardiac death prevention in hypertrophic cardiomyopathy (EVIDENCE-HCM). Circulation 137:1015–1023CrossRefGoogle Scholar
  23. 23.
    Weissler-Snir A, Adler A, Williams L, Gruner C, Rakowski H (2017) Prevention of sudden death in hypertrophic cardiomyopathy: bridging the gaps in knowledge. Eur Heart J 38:1728–1737Google Scholar
  24. 24.
    Yacoub MH, Afifi A, Saad H, Aguib H, ElGuindy A (2017) Current state of the art and future of myectomy. Ann Cardiothorac Surg 6:307–317CrossRefGoogle Scholar
  25. 25.
    Rigopoulos AG, Sakellaropoulos S, Ali M, Mavrogeni S, Manginas A, Pauschinger M, Noutsias M (2018) Transcatheter septal ablation in hypertrophic obstructive cardiomyopathy: a technical guide and review of published results. Heart Fail Rev 23:907–917CrossRefGoogle Scholar
  26. 26.
    Desai MY, Smedira NG, Dhillon A, Masri A, Wazni O, Kanj M, Sato K, Thamilarasan M, Popovic ZB, Lever HM (2018) Prediction of sudden death risk in obstructive hypertrophic cardiomyopathy: potential for refinement of current criteria. J Thorac Cardiovasc Surg 156:750–759 e3 CrossRefGoogle Scholar
  27. 27.
    Jensen MK, Prinz C, Horstkotte D, van Buuren F, Bitter T, Faber L, Bundgaard H (2013) Alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy: low incidence of sudden cardiac death and reduced risk profile. Heart 99:1012–1017CrossRefGoogle Scholar
  28. 28.
    Veselka J, Krejci J, Tomasov P, Zemanek D (2014) Long-term survival after alcohol septal ablation for hypertrophic obstructive cardiomyopathy: a comparison with general population. Eur Heart J 35:2040–2045CrossRefGoogle Scholar
  29. 29.
    Singh K, Qutub M, Carson K, Hibbert B (2016) Glover C. A meta analysis of current status of alcohol septal ablation and surgical myectomy for obstructive hypertrophic cardiomyopathy, Catheter Cardiovasc IntervGoogle Scholar
  30. 30.
    Valeti US, Nishimura RA, Holmes DR, Araoz PA, Glockner JF, Breen JF, Ommen SR, Gersh BJ, Tajik AJ, Rihal CS, Schaff HV, Maron BJ (2007) Comparison of surgical septal myectomy and alcohol septal ablation with cardiac magnetic resonance imaging in patients with hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol 49:350–357CrossRefGoogle Scholar
  31. 31.
    Maron BJ (2000) Role of alcohol septal ablation in treatment of obstructive hypertrophic cardiomyopathy. Lancet 355:425–426CrossRefGoogle Scholar
  32. 32.
    Seggewiss H, Rigopoulos A, Welge D, Ziemssen P, Faber L (2007) Long-term follow-up after percutaneous septal ablation in hypertrophic obstructive cardiomyopathy. Clin Res Cardiol 96:856–863CrossRefGoogle Scholar
  33. 33.
    Brugada P, de Swart H, Smeets JL, Wellens HJ (1989) Transcoronary chemical ablation of ventricular tachycardia. Circulation 79:475–482CrossRefGoogle Scholar
  34. 34.
    Frontera A, Johnson TW, Thomas G, Duncan E (2015) Transcoronary ethanol ablation for incessant ventricular tachycardia: a salvage technique when faced with left ventricular thrombus. Neth Heart J 23:555–556CrossRefGoogle Scholar
  35. 35.
    Tokuda M, Sobieszczyk P, Eisenhauer AC, Kojodjojo P, Inada K, Koplan BA, Michaud GF, John RM, Epstein LM, Sacher F, Stevenson WG, Tedrow UB (2011) Transcoronary ethanol ablation for recurrent ventricular tachycardia after failed catheter ablation: an update. Circ Arrhythm Electrophysiol 4:889–896CrossRefGoogle Scholar
  36. 36.
    Raute-Kreinsen U (2003) Morphology of necrosis and repair after transcoronary ethanol ablation of septal hypertrophy. Pathol Res Pract 199:121–127CrossRefGoogle Scholar
  37. 37.
    Baggish AL, Smith RN, Palacios I, Vlahakes GJ, Yoerger DM, Picard MH, Lowry PA, Jang IK, Fifer MA (2006) Pathological effects of alcohol septal ablation for hypertrophic obstructive cardiomyopathy. Heart 92:1773–1778CrossRefGoogle Scholar
  38. 38.
    van Dockum WG, Knaapen P, Hofman MB, Kuijer JP, ten Cate FJ, ten Berg JM et al (2009) Impact of alcohol septal ablation on left anterior descending coronary artery blood flow in hypertrophic obstructive cardiomyopathy. Int J Cardiovasc Imaging 25:511–518CrossRefGoogle Scholar
  39. 39.
    Chan 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 (2014) Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 130:484–495CrossRefGoogle Scholar
  40. 40.
    Varnava AM, Elliott PM, Mahon N, Davies MJ, McKenna WJ (2001) Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy. Am J Cardiol 88:275–279CrossRefGoogle Scholar
  41. 41.
    Doesch C, Tulumen E, Akin I, Rudic B, Kuschyk J, El-Battrawy I et al (2017) Incremental benefit of late gadolinium cardiac magnetic resonance imaging for risk stratification in patients with hypertrophic cardiomyopathy. Sci Rep 7:6336CrossRefGoogle Scholar
  42. 42.
    Hinojar R, Zamorano JL, Gonzalez Gomez A, Plaza Martin M, Esteban A, Rincon LM et al (2017) ESC sudden-death risk model in hypertrophic cardiomyopathy: incremental value of quantitative contrast-enhanced CMR in intermediate-risk patients. Clin Cardiol 40:853–860CrossRefGoogle Scholar
  43. 43.
    Liebregts M, Faber L, Jensen MK, Vriesendorp PA, Hansen PR, Seggewiss H, Horstkotte D, Adlova R, Michels M, Bundgaard H, ten Berg JM, Veselka J (2018) Validation of the HCM risk-SCD model in patients with hypertrophic cardiomyopathy following alcohol septal ablation. Europace 20:f198–f203CrossRefGoogle Scholar
  44. 44.
    McLeod CJ, Ommen SR, Ackerman MJ, Weivoda PL, Shen WK, Dearani JA et al (2007) Surgical septal myectomy decreases the risk for appropriate implantable cardioverter defibrillator discharge in obstructive hypertrophic cardiomyopathy. Eur Heart J 28:2583–2588CrossRefGoogle Scholar
  45. 45.
    Rigopoulos AG, Daci S, Pfeiffer B, Papadopoulou K, Neugebauer A, Seggewiss H (2016) Low occurrence of ventricular arrhythmias after alcohol septal ablation in high-risk patients with hypertrophic obstructive cardiomyopathy. Clin Res Cardiol 105:953–961CrossRefGoogle Scholar
  46. 46.
    Veselka J, Tomasov P, Januska J, Krejci J, Adlova R (2016) Obstruction after alcohol septal ablation is associated with cardiovascular mortality events. Heart 102:1793–1796CrossRefGoogle Scholar
  47. 47.
    Noseworthy PA, Rosenberg MA, Fifer MA, Palacios IF, Lowry PA, Ruskin JN, Sanborn DM, Picard MH, Vlahakes GJ, Mela T, Das S (2009) Ventricular arrhythmia following alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Am J Cardiol 104:128–132CrossRefGoogle Scholar
  48. 48.
    Sorajja P, Binder J, Nishimura RA, Holmes DR Jr, Rihal CS, Gersh BJ, Bresnahan JF, Ommen SR (2013) Predictors of an optimal clinical outcome with alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Catheter Cardiovasc Interv 81:E58–E67CrossRefGoogle Scholar
  49. 49.
    Veselka J, Faber L, Jensen MK, Cooper R, Januska J, Krejci J, Bartel T, Dabrowski M, Hansen PR, Almaas VM, Seggewiss H, Horstkotte D, Adlova R, Bundgaard H, ten Berg J, Liebregts M (2018) Effect of institutional experience on outcomes of alcohol septal ablation for hypertrophic obstructive cardiomyopathy. Can J Cardiol 34:16–22CrossRefGoogle Scholar
  50. 50.
    Kim LK, Feldman DN (2017) Procedural volume and outcomes of septal reduction therapies in patients with hypertrophic obstructive cardiomyopathy-reply. JAMA Cardiol 2:111CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Angelos G. Rigopoulos
    • 1
    Email author return OK on get
  • Muhammad Ali
    • 1
  • Elena Abate
    • 1
  • Marios Matiakis
    • 1
  • Hannes Melnyk
    • 1
  • Sophie Mavrogeni
    • 2
  • Dionyssios Leftheriotis
    • 3
  • Boris Bigalke
    • 4
  • Michel Noutsias
    • 1
  1. 1.Mid-German Heart Center, Department of Internal Medicine III, Division of Cardiology, Angiology and Intensive Medical CareUniversity Hospital Halle, Martin-Luther-University Halle-WittenbergHalle (Saale)Germany
  2. 2.Onassis Cardiac Surgery CenterAthensGreece
  3. 3.2nd Department of Cardiology, University of AthensAttikon University HospitalAthensGreece
  4. 4.Department of Cardiology, Charité - Universitätsmedizin BerlinCampus Benjamin FranklinBerlinGermany

Personalised recommendations