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Published in: Journal of Nuclear Cardiology 4/2019

01-08-2019 | Editorial

Can positron emission tomography help stratify the risk of sudden cardiac death in patients with hypertrophic cardiomyopathy?

Author: G. Neal Kay, MD

Published in: Journal of Nuclear Cardiology | Issue 4/2019

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Excerpt

Hypertrophic cardiomyopathy (HCM) is a heterogeneous cardiac disease based on autosomal genetic mutations of genes coding for proteins involving the sarcomeres and myofilament elements.14 The clinical consequences of these mutations are widely variable, ranging from patients remaining totally asymptomatic to the most dreaded outcome, sudden cardiac death (SCD) which accounts for over 50% of deaths in this population.4 Despite considerable advances in our understanding of the genetic basis for this disease and its relation to the clinical course of patients with these mutations,5 there remains considerable heterogeneity in the risk of adverse outcomes. This is especially relevant as a clinician tries to prevent sudden cardiac death for an individual patient while minimizing the burden of therapy.6
Literature
1.
go back to reference Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy. J Thorac Cardiovasc Surg 2011;142:153-203.CrossRef Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy. J Thorac Cardiovasc Surg 2011;142:153-203.CrossRef
2.
go back to reference Maron BJ, Maron MS. Contemporary strategies for risk stratification and prevention of sudden death with the implantable defibrillator in hypertrophic cardiomyopathy. Heart Rhythm 2016;13:1155-65.CrossRefPubMed Maron BJ, Maron MS. Contemporary strategies for risk stratification and prevention of sudden death with the implantable defibrillator in hypertrophic cardiomyopathy. Heart Rhythm 2016;13:1155-65.CrossRefPubMed
3.
go back to reference Maron MS, Olivotto I, Maron BJ, Prasad SK, Cecchi F, Udelson JE, Camici PG. The case for myocardial ischemia in hypertrophic cardiomyopathy. J Am Coll Cardiol 2009;54:866-75.CrossRefPubMed Maron MS, Olivotto I, Maron BJ, Prasad SK, Cecchi F, Udelson JE, Camici PG. The case for myocardial ischemia in hypertrophic cardiomyopathy. J Am Coll Cardiol 2009;54:866-75.CrossRefPubMed
4.
go back to reference Maron BJ, Olivotto I, Spirito P, et al. Epidemiology of hypertrophic cardiomyopathy-related death: Revisited in a large non-referral-based patient population. Circulation 2000;102:858-64.CrossRefPubMed Maron BJ, Olivotto I, Spirito P, et al. Epidemiology of hypertrophic cardiomyopathy-related death: Revisited in a large non-referral-based patient population. Circulation 2000;102:858-64.CrossRefPubMed
5.
go back to reference Maurizi N, Passantino S, Spaziani G, Girolami F, Arretini A, Targetti M, Pollini I, Tomberli A, Pradella S, Calabri GB, Vinattieri V, Bertaccini B, Leone O, De Simone L, Rapezzi C, Marchionni N, Cecchi F, Favilli S, Olivotto I. Long-term outcomes of pediatric-onset hypertrophic cardiomyopathy and age-specific risk factors for lethal arrhythmic events. JAMA Cardiol 2018. https://doi.org/10.1001/jamacardio.2018.0789.CrossRefPubMedPubMedCentral Maurizi N, Passantino S, Spaziani G, Girolami F, Arretini A, Targetti M, Pollini I, Tomberli A, Pradella S, Calabri GB, Vinattieri V, Bertaccini B, Leone O, De Simone L, Rapezzi C, Marchionni N, Cecchi F, Favilli S, Olivotto I. Long-term outcomes of pediatric-onset hypertrophic cardiomyopathy and age-specific risk factors for lethal arrhythmic events. JAMA Cardiol 2018. https://​doi.​org/​10.​1001/​jamacardio.​2018.​0789.CrossRefPubMedPubMedCentral
7.
go back to reference Maron BJ, Spirito P, Shen WK, Haas TS, Formisano F, Link MS, Epstein AE, Almquist AK, Daubert JP, Lawrenz T, Boriani G, Estes NA 3rd, 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;298:405-12.CrossRefPubMed Maron BJ, Spirito P, Shen WK, Haas TS, Formisano F, Link MS, Epstein AE, Almquist AK, Daubert JP, Lawrenz T, Boriani G, Estes NA 3rd, 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;298:405-12.CrossRefPubMed
8.
go back to reference Maron 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;11:e005820. https://doi.org/10.1161/CIRCEP.117.005820.CrossRefPubMed Maron 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;11:e005820. https://​doi.​org/​10.​1161/​CIRCEP.​117.​005820.CrossRefPubMed
9.
10.
go back to reference Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and occurrence of sudden cardiac death in hypertrophic cardiomyopathy. J Am Coll Cardiol 1990;15:1521-6.CrossRefPubMed Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and occurrence of sudden cardiac death in hypertrophic cardiomyopathy. J Am Coll Cardiol 1990;15:1521-6.CrossRefPubMed
11.
go back to reference Christiaans I, van Engelen K, van Langen IM, et al. Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: Systematic review of clinical risk markers. Europace 2010;12:313-21.CrossRefPubMed Christiaans I, van Engelen K, van Langen IM, et al. Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: Systematic review of clinical risk markers. Europace 2010;12:313-21.CrossRefPubMed
12.
go back to reference Spirito P, Autore C, Rapezzi C, et al. Syncope and risk of sudden death in hypertrophic cardiomyopathy. Circulation 2009;119:1703-10.CrossRefPubMed Spirito P, Autore C, Rapezzi C, et al. Syncope and risk of sudden death in hypertrophic cardiomyopathy. Circulation 2009;119:1703-10.CrossRefPubMed
13.
go back to reference Viswanathan K, Suszko AM, Das M, et al. Rapid device-detected nonsustained ventricular tachycardia in the risk stratification of hypertrophic cardiomyopathy. Pacing Clin Electrophysiol 2016;39:642-51.CrossRefPubMed Viswanathan K, Suszko AM, Das M, et al. Rapid device-detected nonsustained ventricular tachycardia in the risk stratification of hypertrophic cardiomyopathy. Pacing Clin Electrophysiol 2016;39:642-51.CrossRefPubMed
14.
go back to reference Olivotto I, Maron BJ, Montereggi A, et al. Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol 1999;33:2044-51.CrossRefPubMed Olivotto I, Maron BJ, Montereggi A, et al. Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol 1999;33:2044-51.CrossRefPubMed
16.
go back to reference Sorajja P, Nishimura RA, Ommen SR, et al. Use of echocardiography in patients with hypertrophic cardiomyopathy: Clinical implications of massive hypertrophy. J Am Soc Echocardiogr 2006;19:788-95.CrossRefPubMed Sorajja P, Nishimura RA, Ommen SR, et al. Use of echocardiography in patients with hypertrophic cardiomyopathy: Clinical implications of massive hypertrophy. J Am Soc Echocardiogr 2006;19:788-95.CrossRefPubMed
17.
go back to reference Maron MS, Olivotto I, Betocchi S, et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 2003;348:295-303.CrossRefPubMed Maron MS, Olivotto I, Betocchi S, et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 2003;348:295-303.CrossRefPubMed
18.
go back to reference Maron BJ, Rowin EJ, Casey SA, et al. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. J Am Coll Cardiol 2015;65:1915-28.CrossRefPubMed Maron BJ, Rowin EJ, Casey SA, et al. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. J Am Coll Cardiol 2015;65:1915-28.CrossRefPubMed
19.
go back to reference Choudhury L, Mahrholdt H, Wagner A, Choi KM, Elliott MD, Klocke FJ, et al. Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2002;40:2156-64.CrossRefPubMed Choudhury L, Mahrholdt H, Wagner A, Choi KM, Elliott MD, Klocke FJ, et al. Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2002;40:2156-64.CrossRefPubMed
20.
go back to reference Bruder O, Wagner A, Jensen CJ, Schneider S, Ong P, Kispert EM, et al. Myocardial scar visualized by cardiovascular magnetic resonance imaging predicts major adverse events in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2010;56:875-87.CrossRefPubMed Bruder O, Wagner A, Jensen CJ, Schneider S, Ong P, Kispert EM, et al. Myocardial scar visualized by cardiovascular magnetic resonance imaging predicts major adverse events in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2010;56:875-87.CrossRefPubMed
21.
go back to reference 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. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 2014;130:484-95.CrossRefPubMed 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. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 2014;130:484-95.CrossRefPubMed
22.
go back to reference Green JJ, Berger JS, Kramer CM, Salerno M. Prognostic value of late gadolinium enhancement in clinical outcomes for hypertrophic cardiomyopathy. J Am Coll Cardiol Imaging 2012;5:370-7.CrossRef Green JJ, Berger JS, Kramer CM, Salerno M. Prognostic value of late gadolinium enhancement in clinical outcomes for hypertrophic cardiomyopathy. J Am Coll Cardiol Imaging 2012;5:370-7.CrossRef
23.
go back to reference O’Hanlon R, Grasso A, Roughton M, Moon JC, Clark S, Wage R, et al. Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy. J Am Coll Cardiol 2010;56:867-74.CrossRefPubMed O’Hanlon R, Grasso A, Roughton M, Moon JC, Clark S, Wage R, et al. Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy. J Am Coll Cardiol 2010;56:867-74.CrossRefPubMed
24.
go back to reference Maron MS, Appelbaum E, Harrigan CJ, Buros J, Gibson CM, Hanna C, et al. Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy. Circulation 2008;1:184-91.PubMed Maron MS, Appelbaum E, Harrigan CJ, Buros J, Gibson CM, Hanna C, et al. Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy. Circulation 2008;1:184-91.PubMed
25.
go back to reference Basso C, Thiene G, Corrado D, Buja G, Melacini P, Nava A. Hypertrophic cardiomyopathy and sudden death in the young: Pathologic evidence of myocardial ischemia. Hum Pathol 2000;31:988-98.CrossRefPubMed Basso C, Thiene G, Corrado D, Buja G, Melacini P, Nava A. Hypertrophic cardiomyopathy and sudden death in the young: Pathologic evidence of myocardial ischemia. Hum Pathol 2000;31:988-98.CrossRefPubMed
26.
go back to reference Dilsizian V, Bonow RO, Epstein SE, Fananapazir L. Myocardial ischemia detected by thallium scintigraphy is frequently related to cardiac arrest and syncope in young patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1993;22:796-804.CrossRefPubMed Dilsizian V, Bonow RO, Epstein SE, Fananapazir L. Myocardial ischemia detected by thallium scintigraphy is frequently related to cardiac arrest and syncope in young patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1993;22:796-804.CrossRefPubMed
27.
go back to reference Cecchi F, Olivotto I, Gistri R, Lorenzoni R, Chiriatti G, Camici PG. Coronary microvascular dysfunction and prognosis in hypertrophic cardiomyopathy. N Engl J Med 2003;349:1027-35.CrossRefPubMed Cecchi F, Olivotto I, Gistri R, Lorenzoni R, Chiriatti G, Camici PG. Coronary microvascular dysfunction and prognosis in hypertrophic cardiomyopathy. N Engl J Med 2003;349:1027-35.CrossRefPubMed
28.
go back to reference Lu DY, Yalçin H, Yalçin F, Zhao M, Sivalokanathan S, Valenta I, Tahari A, Pomper MG, Abraham TP, Schindler TH, Abraham MR. Stress myocardial blood flow heterogeneity is a positron emission tomography biomarker of ventricular arrhythmias in patients with hypertrophic cardiomyopathy. Am J Cardiol 2018;121:1081-9.CrossRefPubMedPubMedCentral Lu DY, Yalçin H, Yalçin F, Zhao M, Sivalokanathan S, Valenta I, Tahari A, Pomper MG, Abraham TP, Schindler TH, Abraham MR. Stress myocardial blood flow heterogeneity is a positron emission tomography biomarker of ventricular arrhythmias in patients with hypertrophic cardiomyopathy. Am J Cardiol 2018;121:1081-9.CrossRefPubMedPubMedCentral
29.
go back to reference Maron BJ, Epstein SE, Roberts WC. Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries. Am J Cardiol 1979;43:1086-102.CrossRefPubMed Maron BJ, Epstein SE, Roberts WC. Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries. Am J Cardiol 1979;43:1086-102.CrossRefPubMed
30.
go back to reference Maron BJ, Wolfson JK, Epstein SE, Roberts WC. Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy. J Am Coll Cardiol 1986;8:545-57.CrossRefPubMed Maron BJ, Wolfson JK, Epstein SE, Roberts WC. Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy. J Am Coll Cardiol 1986;8:545-57.CrossRefPubMed
31.
go back to reference O’Gorman DJ, Sheridan DJ. Abnormalities of the coronary circulation associated with left ventricular hypertrophy. Clin Sci (Lond) 1991;81:703-13.CrossRef O’Gorman DJ, Sheridan DJ. Abnormalities of the coronary circulation associated with left ventricular hypertrophy. Clin Sci (Lond) 1991;81:703-13.CrossRef
32.
go back to reference Roberts WC, Ferrans VJ. Pathologic anatomy of the cardiomyopathies. Idiopathic dilated and hypertrophic types, infiltrative types, and endomyocardial disease with and without eosinophilia. Hum Pathol 1975;6:287-342.CrossRefPubMed Roberts WC, Ferrans VJ. Pathologic anatomy of the cardiomyopathies. Idiopathic dilated and hypertrophic types, infiltrative types, and endomyocardial disease with and without eosinophilia. Hum Pathol 1975;6:287-342.CrossRefPubMed
33.
go back to reference Bengel FM, Higuchi T, Javadi MS, Lautamaki R. Cardiac positron emission tomography. J Am Coll Cardiol 2009;54:1-15.CrossRefPubMed Bengel FM, Higuchi T, Javadi MS, Lautamaki R. Cardiac positron emission tomography. J Am Coll Cardiol 2009;54:1-15.CrossRefPubMed
34.
go back to reference Olivotto I, Girolami F, Sciagra R, Ackerman MJ, Sotgia B, Bos JM, et al. Microvascular function is selectively impaired in patients with hypertrophic cardiomyopathy and sarcomere myofilament gene mutations. J Am Coll Cardiol 2011;58:839-48.CrossRefPubMed Olivotto I, Girolami F, Sciagra R, Ackerman MJ, Sotgia B, Bos JM, et al. Microvascular function is selectively impaired in patients with hypertrophic cardiomyopathy and sarcomere myofilament gene mutations. J Am Coll Cardiol 2011;58:839-48.CrossRefPubMed
35.
go back to reference Castagnoli H, Ferrantini C, Coppini R, Passeri A, Baldini K, Berti V, et al. Role of quantitative myocardial positron emission tomography for risk stratification in patients with hypertrophic cardiomyopathy: A 2016 reappraisal. Eur J Nucl Med Mol Imaging 2016;43:2413-22.CrossRefPubMed Castagnoli H, Ferrantini C, Coppini R, Passeri A, Baldini K, Berti V, et al. Role of quantitative myocardial positron emission tomography for risk stratification in patients with hypertrophic cardiomyopathy: A 2016 reappraisal. Eur J Nucl Med Mol Imaging 2016;43:2413-22.CrossRefPubMed
36.
go back to reference Bravo PE. Is there a role for cardiac positron emission tomography in hypertrophic cardiomyopathy? J Nucl Cardiol 2018. Bravo PE. Is there a role for cardiac positron emission tomography in hypertrophic cardiomyopathy? J Nucl Cardiol 2018.
Metadata
Title
Can positron emission tomography help stratify the risk of sudden cardiac death in patients with hypertrophic cardiomyopathy?
Author
G. Neal Kay, MD
Publication date
01-08-2019
Publisher
Springer International Publishing
Published in
Journal of Nuclear Cardiology / Issue 4/2019
Print ISSN: 1071-3581
Electronic ISSN: 1532-6551
DOI
https://doi.org/10.1007/s12350-018-1299-3

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