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Management strategies in hypertrophic cardiomyopathy: influence of age and morphology

W.J. McKenna*, S. Sharma and P.M. Elliott

Department of Cardiological Sciences, St George's Hospital Medical School, London, U.K.

* Correspondence: William J. McKenna, MD, Department of Cardiological Sciences, St George's Hospital Medical School, Cranmer Terrace, London SW17 ORE, U.K.

Abstract

Management in hypertrophic cardiomyopathy is aimed at improving symptoms and exercise capacity and preventing complications, particularly sudden death. The strategy for management of symptoms is to identify potentially important mechanisms at which treatment can be targeted. Assessment is feasible with non-invasive evaluation. All patients with hypertrophic cardiomyopathy should undergo risk assessment for sudden death, regardless of their symptomatic, morphological and haemodynamic status. Recent prospective studies have examined five risk markers: family history of sudden death (two or more members aged <45 years), unexplained syncope, non-sustained ventricular tachycardia on Holler monitoring, abnormal exercise blood pressure response and severe left ventricular hypertrophy (≥3 cm). The majority of patients had none of these risk factors and did not die suddenly. Annual sudden death rates were at least 2% among patients with two or more of these risk markers, which warrants consideration of prophylactic treatment (amiodarone, implantable cardioverter-defibrillator) in such patients.

Key Words: children • hypertrophic cardiomyopathy • hypertrophy • infants • sudden death

References

  1. Vosberg H-P, McKenna WJ. Cardiomyopathies. Rimoin DL, Connor JM, Pyeritz RE. Emery and Rimoin's Principles and Practice of Medical Genetics. 4th Edition. New York: Churchill Livingstone; 2001. (in press)
  2. Spirito P, Seidman CE, McKenna WJ, Maron BJ. Management of hypertrophic cardiomyopathy. [review]N Engl J Med. 1997;336:775–785[Free Full Text]
  3. Schowengerdt KO, Towbin JA. Genetic basis of inherited cardiomyopathies. Curr Opin Cardiol. 1995;10:312–321[CrossRef][Web of Science][Medline]
  4. Maron BJ, Spirito P, Wesley Y, Arce J. Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. N Engl J Med. 1986;315:610–614[Abstract]
  5. Watkins H, Conner D, Thierfelder L, et al. Mutations in the cardiac myosin binding protein-C gene on chromosome 11 cause familial hypertrophic cardiomyopathy. Nat Genet. 1995;11:434–437[CrossRef][Web of Science][Medline]
  6. Bonne G, Carrier L, Bercovici J, et al. A splice acceptor site mutation in the cardiac myosin binding protein C gene is associated with familial hypertrophic cardiomyopathy. Nat Genet. 1995;11:438–440[CrossRef][Web of Science][Medline]
  7. Maron BJ, Gardin JM, Flack JM, et al. Prevalence of hypertrophic cardiomyopathy in a population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA study. Coronary Artery Risk Development in (Young) Adults. Circulation. 1995;92:785–789[Abstract/Free Full Text]
  8. Sharma S, Elliott P, Whyte G, et al. Utility of cardiopulmonary exercise in the assessment of clinical determinants of functional capacity in hypertrophic cardiomyopathy. Am J Cardiol. 2000;86:162–168[CrossRef][Web of Science][Medline]
  9. Pollick C. Muscular subaortic stenosis: hemodynamic and clinical improvement after disopyramide. N Engl J Med. 1982;307:997–999[Web of Science][Medline]
  10. McCully RB, Nishimura RA, Tajik AJ, et al. Extent of clinical improvement after surgical treatment of hypertrophic obstructive cardiomyopathy. Circulation. 1996;94:467–471[Abstract/Free Full Text]
  11. Montijano Cabrera A, Bouzas Zoubeldia B, Penas Lado M, McKenna WJ. Therapeutic approaches to the management of obstruction in hypertrophic cardiomyopathy. Revista Cardiologica. 2001;: (in press)
  12. Faber L, Meissner A, Ziemssen P, Seggewiss H. Percutaneous transluminal septal myocardial ablation for hypertrophic obstructive cardiomyopathy: long term follow up of the first series of 25 patients. Heart. 2000;83:326–331[Abstract/Free Full Text]
  13. Brugada P, Bar FW, de Zwaan C, et al. ‘Sawfish’ systolic narrowing of the left anterior descending coronary artery: an angiographic sign of hypertrophic cardiomyopathy. Curculation. 1982;66:800–803
  14. Alfonso F, Frenneaux M, McKenna WJ. Clinical sustained uniform ventricular tachycardia in hypertrophic cardiomyopathy: association with left ventricular apical aneurysm. Br Heart J. 1989;61:178–181[Abstract/Free Full Text]
  15. Cecchi F, Montereggi A, Olivotto I, et al. Risk for atrial fibrillation in patients with hypertrophic cardiomyopathy assessed by signal averaged P wave duration. Heart. 1997;78:44–49[Abstract/Free Full Text]
  16. MacRae CA, Ghaisas N, Kass S, et al. Familial hypertrophic cardiomyopathy with Wolff-Parkinson-White syndrome maps to a locus on chromosome 7q3. J Clin Invest. 1995;96:1216–1220
  17. McKenna WJ, Stewart JT, Nihoyannopoulos P, et al. Hypertrophic cardiomyopathy without hypertrophy: two families with myocardial disarray in the absence of increased myocardial mass. Br Heart J. 1990;63:287–290[Abstract/Free Full Text]
  18. Elliott PM, Poloniecki J, Dickie S, et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol. 2000;36:2212–2218[Abstract/Free Full Text]
  19. Elliott PM, Gimeno Blanes JR, Mahon NG, et al. Relation between the severity of left ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet. 2001;357:420–424[CrossRef][Web of Science][Medline]
  20. McKenna WJ, Sadoul N, Slade AK, Saumarez RC. The prognostic significance of nonsustained ventricular tachycardia in hypertrophic cardiomyopathy. [editorial]Circulation. 1994;90:3115–3117[Free Full Text]
  21. Spirito P, Rapezzi C, Autore C, et al. Prognosis of asymptomatic patients with hypertrophic cardiomyopathy and nonsustained ventricular tachycardia. Circulation. 1994;90:2743–2747[Abstract/Free Full Text]
  22. Monserrat L, Elliott P, Prasad K, et al. Circadian variations in the incidence and characteristics of non-sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy. [abstract]Eur Heart J. 1998;19:646
  23. McKenna WJ, Deanfreld JE. Hypertrophic cardiomyopathy: an important cause of sudden death. Arch Dis Child. 1984;59:971–975[Abstract/Free Full Text]
  24. Spirito P, Bellone P, Harris KM, et al. Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med. 2000;342:1778–1785[Abstract/Free Full Text]
  25. Sadoul N, Prasad K, Elliott PM, et al. Prospective assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation. 1997;96:2987–2991[Abstract/Free Full Text]
  26. McKenna WJ, Oakley CM, Krikler DM, Goodwin JF. Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia. Br Heart J. 1985;53:412–416[Abstract/Free Full Text]
  27. Cecchi F, Olivotto I, Montereggi A, et al. Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population. J Am Coll Cardiol. 1995;26:1529–1536[Abstract]
  28. Maron BJ, Shen W-K, Link MS, et al. Efficacy of the implantable cardioverter-defibrillator for the prevention of sudden death in hypertrophic cardiomyopathy. N Engl J Med. 2000;342:365–373[Abstract/Free Full Text]

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