Advertisement

Longitudinal Case-Based Presentations in HCM

  • Abdul Moiz Hafiz
  • Jonathan Elias
  • Kanika Mody
  • Jenna Kahn
  • Sahil Khera
  • Ankur Kalra
  • Srihari S. Naidu
Chapter

Abstract

A significant volume of data has been published over the past few decades regarding HCM. In 2011 the ACCF/AHA issued guidelines for management and diagnosis, and in 2014 the ESC guidelines were formalized. Nonetheless, the acute and chronic management of HCM is highly nuanced, based on collective experience of many patients. To elucidate the subtleties of management, eight carefully selected cases attempt to depict the medical decision-making process at various stages of illness. Case 1 is a middle-aged male with refractory NYHA Class III symptoms who eventually improved after surgical septal reduction. Case 2 is a young female with history of sudden death and refractory symptoms who required invasive therapy, and a successful alcohol septal ablation was performed due to strong patient preference. Case 3 is a young female without significant outflow tract obstruction but with advanced diastolic heart failure who eventually required a heart transplant. Case 4 depicts severe obstructive HCM in a female with advanced age who after an extended course of medical therapy eventually improved post-alcohol septal ablation. Case 5 is a relatively young female post ICD for SCD with ICD lead complications and atrial fibrillation but well managed medically. Case 6 is a middle-aged male with severe obstructive HCM who required invasive therapy eventually and preferred alcohol septal ablation to surgery. Case 7 is a patient with obstructive HCM referred for alcohol septal ablation who was subsequently determined to have a subaortic membrane and ultimately required surgical treatment. Case 8 is a 40-year-old patient who underwent surgical myectomy but became symptomatic again 3 years after her surgery and eventually underwent alcohol septal ablation. As will become clear, appropriate care of HCM patients requires an individualized and comprehensive approach keeping in view their specific and oftentimes changing presentation, currently available data, and guidelines, all within the confines of a dedicated HCM center.

Keywords

Case series Hypertrophic cardiomyopathy Long term Follow-up 

Abbreviations

ACCF

American College of Cardiology Foundation

AHA

American Heart Association

CMRI

Cardiac magnetic resonance imaging

CPR

Cardiopulmonary resuscitation

DCCV

Direct current cardioversion

ESC

European Society of Cardiology

ETT

Exercise treadmill test

HCM

Hypertrophic cardiomyopathy

HOCM

Hypertrophic obstructive cardiomyopathy

ICD

Implantable cardioverter defibrillator

INR

International normalized ratio

LBBB

Left bundle branch block

LGE

Late gadolinium enhancement

LVOT

Left ventricular outflow tract

NSVT

Non-sustained ventricular tachycardia

NYHA

New York Heart Association

RBBB

Right bundle branch block

SAM

Systolic anterior motion of mitral valve

SCD

Sudden cardiac death

TEE

Transesophageal echocardiography

TTE

Transthoracic echocardiography

VT

Ventricular tachycardia

References

  1. 1.
    Gersh BJ, Maron BJ, Bonow RO, 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:e212–60.CrossRefGoogle Scholar
  2. 2.
    Authors/Task Force members, Elliott PM, Anastasakis A, et al. 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. 2014;35:2733–79.CrossRefGoogle Scholar
  3. 3.
    Rickers C, Wilke NM, Jerosch-Herold M, et al. Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation. 2005;112:855–61.CrossRefGoogle Scholar
  4. 4.
    Moon JC, Fisher NG, McKenna WJ, Pennell DJ. Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with non-diagnostic echocardiography. Heart. 2004;90:645–9.CrossRefGoogle Scholar
  5. 5.
    Maron MS, Appelbaum E, Harrigan CJ, et al. Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy. Circ Heart Fail. 2008;1:184–91.CrossRefGoogle Scholar
  6. 6.
    Choudhury L, Mahrholdt H, Wagner A, et al. Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2002;40:2156–64.CrossRefGoogle Scholar
  7. 7.
    Talreja DR, Nishimura RA, Edwards WD, et al. Alcohol septal ablation versus surgical septal myectomy: comparison of effects on atrioventricular conduction tissue. J Am Coll Cardiol. 2004;44:2329–32.CrossRefGoogle Scholar
  8. 8.
    Spirito P, Autore C, Rapezzi C, et al. Syncope and risk of sudden death in hypertrophic cardiomyopathy. Circulation. 2009;119:1703–10.CrossRefGoogle Scholar
  9. 9.
    Zipes DP, Wellens HJ. Sudden cardiac death. Circulation. 1998;98:2334–51.CrossRefGoogle Scholar
  10. 10.
    Elliott PM, Poloniecki J, Dickie S, et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol. 2000;36:2212–8.CrossRefGoogle Scholar
  11. 11.
    Ackerman MJ. Genetic testing for risk stratification in hypertrophic cardiomyopathy and long QT syndrome: fact or fiction? Curr Opin Cardiol. 2005;20:175–81.CrossRefGoogle Scholar
  12. 12.
    Noseworthy PA, Newton-Cheh C. Genetic determinants of sudden cardiac death. Circulation. 2008;118:1854–63.CrossRefGoogle Scholar
  13. 13.
    Van Tintelen JP, Pieper PG, Van Spaendonck-Zwarts KY, Van Den Berg MP. Pregnancy, cardiomyopathies, and genetics. Cardiovasc Res. 2014;101:571–8.CrossRefGoogle Scholar
  14. 14.
    Avila WS, Rossi EG, Ramires JA, et al. Pregnancy in patients with heart disease: experience with 1,000 cases. Clin Cardiol. 2003;26:135–42.CrossRefGoogle Scholar
  15. 15.
    Autore C, Conte MR, Piccininno M, et al. Risk associated with pregnancy in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2002;40:1864–9.CrossRefGoogle Scholar
  16. 16.
    Tromp CH, Nanne AC, Pernet PJ, Tukkie R, Bolte AC. Electrical cardioversion during pregnancy: safe or not? Neth Heart J. 2011;19:134–6.CrossRefGoogle Scholar
  17. 17.
    European Society of G, Association for European Paediatric C, German Society for Gender M, et al. ESC guidelines on the management of cardiovascular diseases during pregnancy: the task force on the management of cardiovascular diseases during pregnancy of the European Society of Cardiology (ESC). Eur Heart J. 2011;32:3147–97.CrossRefGoogle Scholar
  18. 18.
    Yu EH, Omran AS, Wigle ED, Williams WG, Siu SC, Rakowski H. Mitral regurgitation in hypertrophic obstructive cardiomyopathy: relationship to obstruction and relief with myectomy. J Am Coll Cardiol. 2000;36:2219–25.CrossRefGoogle Scholar
  19. 19.
    Kofflard MJ, van Herwerden LA, Waldstein DJ, et al. Initial results of combined anterior mitral leaflet extension and myectomy in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 1996;28:197–202.CrossRefGoogle Scholar
  20. 20.
    McIntosh CL, Maron BJ, Cannon RO 3rd, Klues HG. Initial results of combined anterior mitral leaflet plication and ventricular septal myotomy-myectomy for relief of left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy. Circulation. 1992;86:II60–7.PubMedGoogle Scholar
  21. 21.
    Reis RL, Bolton MR, King JF, Pugh DM, Dunn MI, Mason DT. Anterion-superior displacement of papillary muscles producing obstruction and mitral regurgitation in idiopathic hypertrophic subaortic stenosis. Operative relief by posterior-superior realignment of papillary muscles following ventricular septal myectomy. Circulation. 1974;50:II181–8.CrossRefGoogle Scholar
  22. 22.
    Schoendube FA, Klues HG, Reith S, Flachskampf FA, Hanrath P, Messmer BJ. Long-term clinical and echocardiographic follow-up after surgical correction of hypertrophic obstructive cardiomyopathy with extended myectomy and reconstruction of the subvalvular mitral apparatus. Circulation. 1995;92:II122–7.CrossRefGoogle Scholar
  23. 23.
    Desai MY, Bhonsale A, Smedira NG, et al. Predictors of long-term outcomes in symptomatic hypertrophic obstructive cardiomyopathy patients undergoing surgical relief of left ventricular outflow tract obstruction. Circulation. 2013;128:209–16.CrossRefGoogle Scholar
  24. 24.
    Stassano P, Di Tommaso L, Triggiani D, Contaldo A, Gagliardi C, Spampinato N. Mitral valve replacement and limited myectomy for hypertrophic obstructive cardiomyopathy: a 25-year follow-up. Tex Heart Inst J. 2004;31:137–42.PubMedPubMedCentralGoogle Scholar
  25. 25.
    Minakata K, Dearani JA, Nishimura RA, Maron BJ, Danielson GK. Extended septal myectomy for hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles or chordae. J Thorac Cardiovasc Surg. 2004;127:481–9.CrossRefGoogle Scholar
  26. 26.
    O’Mahony C, Jichi F, Pavlou M, et al. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J. 2014;35:2010–20.CrossRefGoogle Scholar
  27. 27.
    Karibe A, Tobacman LS, Strand J, et al. Hypertrophic cardiomyopathy caused by a novel alpha-tropomyosin mutation (V95A) is associated with mild cardiac phenotype, abnormal calcium binding to troponin, abnormal myosin cycling, and poor prognosis. Circulation. 2001;103:65–71.CrossRefGoogle Scholar
  28. 28.
    Geske JB, Sorajja P, Nishimura RA, Ommen SR. Evaluation of left ventricular filling pressures by Doppler echocardiography in patients with hypertrophic cardiomyopathy: correlation with direct left atrial pressure measurement at cardiac catheterization. Circulation. 2007;116:2702–8.CrossRefGoogle Scholar
  29. 29.
    Pasipoularides A. LV twisting and untwisting in HCM: ejection begets filling. Diastolic functional aspects of HCM. Am Heart J. 2011;162:798–810.CrossRefGoogle Scholar
  30. 30.
    Gersh BJ, Maron BJ, Bonow RO, 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. Circulation. 2011;124:e783–831.PubMedGoogle Scholar
  31. 31.
    Mehra MR, Kobashigawa J, Starling R, et al. Listing criteria for heart transplantation: International Society for Heart and Lung Transplantation guidelines for the care of cardiac transplant candidates – 2006. J Heart Lung Transplant. 2006;25:1024–42.CrossRefGoogle Scholar
  32. 32.
    Fifer MA, Vlahakes GJ. Management of symptoms in hypertrophic cardiomyopathy. Circulation. 2008;117:429–39.CrossRefGoogle Scholar
  33. 33.
    Sanderson JE, Gibson DG, Brown DJ, Goodwin JF. Left ventricular filling in hypertrophic cardiomyopathy. An angiographic study. Br Heart J. 1977;39:661–70.CrossRefGoogle Scholar
  34. 34.
    Rosing DR, Kent KM, Borer JS, Seides SF, Maron BJ, Epstein SE. Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy. I Hemodynamic effects. Circulation. 1979;60:1201–7.CrossRefGoogle Scholar
  35. 35.
    Bonow RO, Rosing DR, Epstein SE. The acute and chronic effects of verapamil on left ventricular function in patients with hypertrophic cardiomyopathy. Eur Heart J. 1983;4 Suppl F:57–65.CrossRefGoogle Scholar
  36. 36.
    Spicer RL, Rocchini AP, Crowley DC, Vasiliades J, Rosenthal A. Hemodynamic effects of verapamil in children and adolescents with hypertrophic cardiomyopathy. Circulation. 1983;67:413–20.CrossRefGoogle Scholar
  37. 37.
    Rosing DR, Idanpaan-Heikkila U, Maron BJ, Bonow RO, Epstein SE. Use of calcium-channel blocking drugs in hypertrophic cardiomyopathy. Am J Cardiol. 1985;55:185B–95B.CrossRefGoogle Scholar
  38. 38.
    Toshima H, Koga Y, Nagata H, Toyomasu K, Itaya K, Matoba T. Comparable effects of oral diltiazem and verapamil in the treatment of hypertrophic cardiomyopathy. Double-blind crossover study. Jpn Heart J. 1986;27:701–15.CrossRefGoogle Scholar
  39. 39.
    Nishimura RA, Trusty JM, Hayes DL, et al. Dual-chamber pacing for hypertrophic cardiomyopathy: a randomized, double-blind, crossover trial. J Am Coll Cardiol. 1997;29:435–41.CrossRefGoogle Scholar
  40. 40.
    Maron BJ, Nishimura RA, McKenna WJ, Rakowski H, Josephson ME, Kieval RS. Assessment of permanent dual-chamber pacing as a treatment for drug-refractory symptomatic patients with obstructive hypertrophic cardiomyopathy. A randomized, double-blind, crossover study (M-PATHY). Circulation. 1999;99:2927–33.CrossRefGoogle Scholar
  41. 41.
    Nagueh SF, Groves BM, Schwartz L, et al. Alcohol septal ablation for the treatment of hypertrophic obstructive cardiomyopathy. A Multicenter North American Registry. J Am Coll Cardiol. 2011;58:2322–8.CrossRefGoogle Scholar
  42. 42.
    Veselka J, Krejčí J, Tomašov P, Zemánek D. Long-term survival after alcohol septal ablation for hypertrophic obstructive cardiomyopathy: a comparison with general population. Eur Heart J. 2014:eht495.Google Scholar
  43. 43.
    Charitakis K, Basson CT. Can genetic testing improve our aim in hypertrophic cardiomyopathy? Circ Res. 2010;106:1446–8.CrossRefGoogle Scholar
  44. 44.
    Inada K, Seiler J, Roberts-Thomson KC, et al. Substrate characterization and catheter ablation for monomorphic ventricular tachycardia in patients with apical hypertrophic cardiomyopathy. J Cardiovasc Electrophysiol. 2011;22:41–8.CrossRefGoogle Scholar
  45. 45.
    Lim KK, Maron BJ, Knight BP. Successful catheter ablation of hemodynamically unstable monomorphic ventricular tachycardia in a patient with hypertrophic cardiomyopathy and apical aneurysm. J Cardiovasc Electrophysiol. 2009;20:445–7.CrossRefGoogle Scholar
  46. 46.
    Kapa S, Curwin JH, Coyne RF, Winters SL. Inappropriate defibrillator shocks from depolarization—repolarization mismatch in a patient with hypertrophy cardiomyopathy. Pacing Clin Electrophysiol. 2007;30:1408–11.CrossRefGoogle Scholar
  47. 47.
    Koneru JN, Swerdlow CD, Wood MA, Ellenbogen KA. Minimizing inappropriate or “unnecessary” implantable cardioverter-defibrillator shocks: appropriate programming. Circ Arrhythm Electrophysiol. 2011;4:778–90.CrossRefGoogle Scholar
  48. 48.
    Maron BJ, Spirito P, Shen WK, et al. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA. 2007;298:405–12.PubMedPubMedCentralGoogle Scholar
  49. 49.
    Holmes DR Jr, Kar S, Price MJ, et al. Prospective randomized evaluation of the Watchman Left Atrial Appendage Closure device in patients with atrial fibrillation versus long-term warfarin therapy: the PREVAIL trial. J Am Coll Cardiol. 2014;64:1–12.CrossRefGoogle Scholar
  50. 50.
    Reddy VY, Sievert H, Halperin J, et al. Percutaneous left atrial appendage closure vs warfarin for atrial fibrillation: a randomized clinical trial. JAMA. 2014;312:1988–98.CrossRefGoogle Scholar
  51. 51.
    Sylvester J, Seidenberg P, Silvis M. The dilemma of genotype positive-phenotype negative hypertrophic cardiomyopathy. Curr Sports Med Rep. 2014;13:94–9.  https://doi.org/10.1249/JSR.0000000000000037.CrossRefPubMedGoogle Scholar
  52. 52.
    Sylvester J, Seidenberg P, Silvis M. The dilemma of genotype positive-phenotype negative hypertrophic cardiomyopathy. Curr Sports Med Rep. 2014;13:94–9.CrossRefGoogle Scholar
  53. 53.
    Maron BJ, Chaitman BR, Ackerman MJ, et al. Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases. Circulation. 2004;109:2807–16.CrossRefGoogle Scholar
  54. 54.
    Sadoul N, Prasad K, Elliott PM, Bannerjee S, Frenneaux MP, McKenna WJ. Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation. 1997;96:2987–91.CrossRefGoogle Scholar
  55. 55.
    Olivotto I, Maron BJ, Montereggi A, Mazzuoli F, Dolara A, Cecchi F. 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.CrossRefGoogle Scholar
  56. 56.
    Alam M, Dokainish H, Lakkis NM. Hypertrophic obstructive cardiomyopathy-alcohol septal ablation vs. myectomy: a meta-analysis. Eur Heart J. 2009;30:1080–7.CrossRefGoogle Scholar
  57. 57.
    Dewland TA, Pellegrini CN, Wang Y, Marcus GM, Keung E, Varosy PD. Dual-chamber implantable cardioverter-defibrillator selection is associated with increased complication rates and mortality among patients enrolled in the NCDR implantable cardioverter-defibrillator registry. J Am Coll Cardiol. 2011;58:1007–13.CrossRefGoogle Scholar
  58. 58.
    Sorajja P, Ommen SR, Nishimura RA, Gersh BJ, Berger PB, Tajik AJ. Adverse prognosis of patients with hypertrophic cardiomyopathy who have epicardial coronary artery disease. Circulation. 2003;108:2342–8.CrossRefGoogle Scholar
  59. 59.
    Morrow AG, Reitz BA, Epstein SE, et al. Operative treatment in hypertrophic subaortic stenosis. Techniques, and the results of pre and postoperative assessments in 83 patients. Circulation. 1975;52:88–102.CrossRefGoogle Scholar
  60. 60.
    Maron BJ. Controversies in cardiovascular medicine. Surgical myectomy remains the primary treatment option for severely symptomatic patients with obstructive hypertrophic cardiomyopathy. Circulation. 2007;116:196–206; discussion.CrossRefGoogle Scholar
  61. 61.
    Liebregts M, Vriesendorp PA, Ten Berg JM. Alcohol septal ablation for obstructive hypertrophic cardiomyopathy: a word of endorsement. J Am Coll Cardiol. 2017;70:481–8.CrossRefGoogle Scholar
  62. 62.
    Schulte HD, Borisov K, Gams E, Gramsch-Zabel H, Losse B, Schwartzkopff B. Management of symptomatic hypertrophic obstructive cardiomyopathy – long-term results after surgical therapy. Thorac Cardiovasc Surg. 1999;47:213–8.CrossRefGoogle Scholar
  63. 63.
    Cho YH, Quintana E, Schaff HV, et al. Residual and recurrent gradients after septal myectomy for hypertrophic cardiomyopathy-mechanisms of obstruction and outcomes of reoperation. J Thorac Cardiovasc Surg. 2014;148:909–15; discussion 15–6.CrossRefGoogle Scholar
  64. 64.
    Liebregts M, Vriesendorp PA, Mahmoodi BK, Schinkel AF, Michels M, ten Berg JM. A systematic review and meta-analysis of long-term outcomes after septal reduction therapy in patients with hypertrophic cardiomyopathy. JACC Heart Fail. 2015;3:896–905.CrossRefGoogle Scholar
  65. 65.
    Bhargava B, Agarwal R. Can we predict complete heart block after alcohol ablation for hypertrophic cardiomyopathy? Circulation. 1999;100:e144.CrossRefGoogle Scholar
  66. 66.
    Knight C, Kurbaan AS, Seggewiss H, et al. Nonsurgical septal reduction for hypertrophic obstructive cardiomyopathy: outcome in the first series of patients. Circulation. 1997;95:2075–81.CrossRefGoogle Scholar
  67. 67.
    Faber L, Welge D, Fassbender D, Schmidt HK, Horstkotte D, Seggewiss H. Percutaneous septal ablation for symptomatic hypertrophic obstructive cardiomyopathy: managing the risk of procedure-related AV conduction disturbances. Int J Cardiol. 2007;119:163–7.CrossRefGoogle Scholar
  68. 68.
    Agarwal S, Tuzcu EM, Desai MY, et al. Updated meta-analysis of septal alcohol ablation versus myectomy for hypertrophic cardiomyopathy. J Am Coll Cardiol. 2010;55:823–34.CrossRefGoogle Scholar
  69. 69.
    Liebregts M, Faber L, Jensen MK, et al. Outcomes of alcohol septal ablation in younger patients with obstructive hypertrophic cardiomyopathy. JACC Cardiovasc Interv. 2017;10:1134–43.CrossRefGoogle Scholar
  70. 70.
    Chang SM, Nagueh SF, Spencer WH 3rd, Lakkis NM. Complete heart block: determinants and clinical impact in patients with hypertrophic obstructive cardiomyopathy undergoing nonsurgical septal reduction therapy. J Am Coll Cardiol. 2003;42:296–300.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Abdul Moiz Hafiz
    • 1
  • Jonathan Elias
    • 2
  • Kanika Mody
    • 3
  • Jenna Kahn
    • 4
  • Sahil Khera
    • 4
  • Ankur Kalra
    • 5
  • Srihari S. Naidu
    • 6
  1. 1.Division of CardiologySouthern Illinois University School of MedicineSpringfieldUSA
  2. 2.Piedmont Medical Center and Springs Memorial HospitalRock HillUSA
  3. 3.Division of CardiologyRobert Wood Johnson University HospitalNew BrunswickUSA
  4. 4.Massachusetts General Hospital, Harvard Medical SchoolBostonUSA
  5. 5.University Hospitals Cleveland Medical CenterCase Western Reserve University School of MedicineClevelandUSA
  6. 6.Westchester Medical Center-Hypertrophic Cardiomyopathy Center of ExcellenceValhallaUSA

Personalised recommendations