Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac condition,
with an associated increased risk of ventricular arrhythmias and sudden cardiac death.
Young and asymptomatic patients, including professional athletes, are not spared this
risk. Implantable cardiac defibrillators (ICDs) are highly effective in terminating
malignant ventricular arrhythmias in this group, but they are associated with significant
morbidity, such as inappropriate shocks and device complications. Accurate prognostication
to guide ICD implantation is therefore essential. The interplay of traditional risk
factors, risk modifiers and predictive models creates a complex decision-making environment
for the HCM clinician. Risk stratifying tools are expanding with improved understanding
of advanced imaging modalities, such as late gadolinium enhancement on cardiac magnetic
resonance imaging (cMRI). Once the decision to implant a defibrillator is reached,
the choice of device and programming in HCM is unique and should take into account
disease substrate and younger age of patients.
Keywords
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References
- Asymmetrical hypertrophy of the heart in young adults.Br Heart J. 1958; 20: 1-8
- Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine.J Am Coll Cardiol. 2014; 64: 83-99
- New perspectives on the prevalence of hypertrophic cardiomyopathy.J Am Coll Cardiol. 2015; 65: 1249-1254
- Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population.Circulation. 2000; 102: 858-864
- Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies.J Am Coll Cardiol. 2015; 65: 1915-1928
- A prospective study of sudden cardiac death among children and young adults.N Engl J Med. 2016; 374: 2441-2452
- Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles.JAMA. 1996; 276: 199-204
- Morphology and significance of the left ventricular collagen network in young patients with hypertrophic cardiomyopathy and sudden cardiac death.J Am Coll Cardiol. 2000; 35: 36-44
- Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophic cardiomyopathy.Circulation. 1979; 59: 689-706
- Electrophysiological characteristics of septal hypertrophy in patients with hypertrophic obstructive cardiomyopathy and moderate to severe symptoms.Circulation. 2005; 112: 2096-2101
- Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy.J Am Coll Cardiol. 2002; 40: 2156-2164
- Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy.J Am Coll Cardiol. 1986; 8: 545-557
- Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.J Am Coll Cardiol. 2000; 35: 1493-1501
- Electrophysiologic manifestations of ventricular tachyarrhythmias provoking appropriate defibrillator interventions in high-risk patients with hypertrophic cardiomyopathy.J Cardiovasc Electrophysiol. 2007; 18: 483-487
- Risk markers and appropriate implantable defibrillator therapy in hypertrophic cardiomyopathy.Pacing Clin Electrophysiol. 2016; 39: 291-301
- Contemporary strategies for risk stratification and prevention of sudden death with the implantable defibrillator in hypertrophic cardiomyopathy.Heart Rhythm. 2016; 13: 1155-1165
- ICD therapy for primary prevention in hypertrophic cardiomyopathy.Arrhythm Electrophysiol Rev. 2016; 5: 188-196
- 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. 2014; 35: 2733-2779
- 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-e260
- Update on the diagnosis and management of hypertrophic cardiomyopathy.Heart Lung Circ. 2018; 27: 276-279
- Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy.J Am Coll Cardiol. 1999; 33: 1596-1601
- Long-term outcome of patients with hypertrophic cardiomyopathy successfully resuscitated after cardiac arrest.J Am Coll Cardiol. 1989; 13: 1283-1288
- Family history of sudden death should be a primary indication for implantable cardioverter defibrillator in hypertrophic cardiomyopathy.Can J Cardiol. 2015; 31: 1402-1406
- Sudden death in hypertrophic cardiomyopathy: identification of high risk patients.J Am Coll Cardiol. 2000; 36: 2212-2218
- Predictors of sudden cardiac death in hypertrophic cardiomyopathy.Am J Cardiol. 1998; 82: 774-778
- “Malignant” hypertrophic cardiomyopathy: identification of a subgroup of families with unusually frequent premature death.Am J Cardiol. 1978; 41: 1133-1140
- Syncope and risk of sudden death in hypertrophic cardiomyopathy.Circulation. 2009; 119: 1703-1710
- Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy.JAMA. 2007; 298: 405-412
- Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy.N Engl J Med. 2000; 342: 1778-1785
- Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy.Lancet. 2001; 357: 420-424
- Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients.J Am Coll Cardiol. 2003; 42: 873-879
- Prognostic implications of nonsustained ventricular tachycardia in high-risk patients with hypertrophic cardiomyopathy.Circ Arrhythm Electrophysiol. 2017; 10
- Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy.J Am Coll Cardiol. 2005; 45: 697-704
- Prognostic determinants in hypertrophic cardiomyopathy. Prospective evaluation of a therapeutic strategy based on clinical, Holter, hemodynamic, and electrophysiological findings.Circulation. 1992; 86: 730-740
- Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy.Circulation. 1997; 96: 2987-2991
- Prognostic value of LGE-CMR in HCM: a meta-analysis.JACC Cardiovasc Imaging. 2016; 9: 1392-1402
- Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy.Circulation. 2014; 130: 484-495
- Significance of late gadolinium enhancement at right ventricular attachment to ventricular septum in patients with hypertrophic cardiomyopathy.Am J Cardiol. 2015; 116: 436-441
- Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance.J Am Coll Cardiol. 2008; 51: 1369-1374
- Evaluation of techniques for the quantification of myocardial scar of differing etiology using cardiac magnetic resonance.JACC Cardiovasc Imaging. 2011; 4: 150-156
- Hypertrophic cardiomyopathy with left ventricular apical aneurysm: implications for risk stratification and management.J Am Coll Cardiol. 2017; 69: 761-773
- Significance of sarcomere gene mutations analysis in the end-stage phase of hypertrophic cardiomyopathy.Am J Cardiol. 2014; 114: 769-776
- Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy.Eur Heart J. 2006; 27: 1933-1941
- Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy.N Engl J Med. 2003; 348: 295-303
- Sudden death in hypertrophic cardiomyopathy. Assessment of patients at high risk.Circulation. 1989; 80: 1489-1492
- A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD).Eur Heart J. 2014; 35: 2010-2020
- QRS fragmentation and QTc duration relate to malignant ventricular tachyarrhythmias and sudden cardiac death in patients with hypertrophic cardiomyopathy.J Cardiovasc Electrophysiol. 2015; 26: 547-555
- Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives.J Am Coll Cardiol. 2012; 60: 705-715
- Compound and double mutations in patients with hypertrophic cardiomyopathy: implications for genetic testing and counselling.J Med Genet. 2005; 42: e59
- Multiple gene variants in hypertrophic cardiomyopathy in the era of next-generation sequencing.Circ Cardiovasc Genet. 2017; 10
- Sudden cardiac arrest in hypertrophic cardiomyopathy in the absence of conventional criteria for high risk status.Am J Cardiol. 2008; 101: 544-547
- Risk stratification and outcome of patients with hypertrophic cardiomyopathy >=60 years of age.Circulation. 2013; 127: 585-593
- Long-Term outcomes of hypertrophic cardiomyopathy diagnosed during childhood: results from a national population-based study.Circulation. 2018; 138: 29-36
- 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. 2015; 8: 829-835
- 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. 2016; 118: 121-126
- Independent assessment of the European Society of Cardiology sudden death risk model for hypertrophic cardiomyopathy.Am J Cardiol. 2015; 116: 757-764
- Implantable cardioverter-defibrillator in patients with hypertrophic cardiomyopathy: efficacy and complications of the therapy in long-term follow-up.J Cardiovasc Electrophysiol. 2010; 21: 883-889
- Long-term follow-up of implantable cardioverter defibrillator therapy for hypertrophic cardiomyopathy.Am J Cardiol. 2004; 93: 1192-1194
- Prevention of sudden cardiac death with implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy.J Am Coll Cardiol. 2013; 61: 1527-1535
- Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.N Engl J Med. 2000; 342: 365-373
- Are implantable cardioverter defibrillator shocks a surrogate for sudden cardiac death in patients with nonischemic cardiomyopathy?.Circulation. 2006; 113: 776-782
- Device complications and inappropriate implantable cardioverter defibrillator shocks in patients with hypertrophic cardiomyopathy.Heart. 2009; 95: 709-714
- Pacing and hypertrophic obstructive cardiomyopathy.Pacing Clin Electrophysiol. 1996; 19: 1141-1142
- Defibrillation thresholds in hypertrophic cardiomyopathy.J Cardiovasc Electrophysiol. 2011; 22: 569-572
- Cardioverter-defibrillator implantation in high-risk patients with hypertrophic cardiomyopathy.Heart Rhythm. 2005; 2: 814-819
- Defibrillation threshold testing in patients with hypertrophic cardiomyopathy.Pacing Clin Electrophysiol. 2010; 33: 1342-1346
- Single-coil defibrillator leads yield satisfactory defibrillation safety margin in hypertrophic cardiomyopathy.Circ J. 2016; 80: 2199-2203
- Outcome and complications after implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: systematic review and meta-analysis.Circ Heart Fail. 2012; 5: 552-559
- Performance and survival of transvenous defibrillation leads: need for a European data registry.Europace. 2009; 11: 31-34
- Risk stratification and role of implantable defibrillators for prevention of sudden death in patients with hypertrophic cardiomyopathy.Circ J. 2010; 74: 2271-2282
- Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: a systematic review and meta-analysis of inappropriate shocks and complications.Heart Rhythm. 2016; 13: 443-454
- Appropriate implantable defibrillator therapy in adults with hypertrophic cardiomyopathy.J Cardiovasc Electrophysiol. 2016; 27: 953-960
- Reduction in inappropriate therapy and mortality through ICD programming.N Engl J Med. 2012; 367: 2275-2283
- Evaluation of subcutaneous ICD early performance in hypertrophic cardiomyopathy from the pooled EFFORTLESS and IDE cohorts.Heart Rhythm. 2016; 13: 1066-1074
- Subcutaneous Implantable cardioverter defibrillator in patients with hypertrophic cardiomyopathy: an initial experience.J Am Heart Assoc. 2016; 5
- Which patients are not suitable for a subcutaneous ICD: incidence and predictors of failed QRS-T-wave morphology screening.J Cardiovasc Electrophysiol. 2014; 25: 494-499
- Evidence that pharmacological strategies lack efficacy for the prevention of sudden death in hypertrophic cardiomyopathy.Heart. 2007; 93: 708-710
- Epicardial ablation of monomorphic ventricular tachycardia in a case of hypertrophic cardiomyopathy with apical aneurysm.Europace. 2013; 15: 296
- Epicardial ablation of monomorphic ventricular tachycardia storm in hypertrophic cardiomyopathy.Europace. 2013; 15: 346
- Radiofrequency catheter ablation of sustained monomorphic ventricular tachycardia in hypertrophic cardiomyopathy.J Cardiovasc Electrophysiol. 1997; 8: 803-806
- Radiofrequency catheter ablation of ventricular arrhythmias in patients with hypertrophic cardiomyopathy: safety and feasibility.Heart Rhythm. 2010; 7: 1036-1042
- Long-term outcomes of combined epicardial and endocardial ablation of monomorphic ventricular tachycardia related to hypertrophic cardiomyopathy.Circ Arrhythm Electrophysiol. 2011; 4: 185-194
- Cardiac sympathectomy for the management of ventricular arrhythmias refractory to catheter ablation.Heart Rhythm. 2018; 15: 56-62
- Videoscopic left cardiac sympathetic denervation for patients with recurrent ventricular fibrillation/malignant ventricular arrhythmia syndromes besides congenital long-QT syndrome.Circ Arrhythm Electrophysiol. 2012; 5: 782-788
- Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery.Heart Rhythm. 2009; 6: 752-759
- What is the role of cardiac sympathetic denervation for recurrent ventricular tachycardia?.Curr Treat Options Cardiovasc Med. 2017; 19: 11
- Surgical septal myectomy decreases the risk for appropriate implantable cardioverter defibrillator discharge in obstructive hypertrophic cardiomyopathy.Eur Heart J. 2007; 28: 2583-2588
- Meta-analyses of septal reduction therapies for obstructive hypertrophic cardiomyopathy: comparative rates of overall mortality and sudden cardiac death after treatment.Circ Cardiovasc Interv. 2010; 3: 97-104
Article info
Publication history
Published online: September 19, 2018
Identification
Copyright
© 2018 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.
