Abstract
Hypertrophic cardiomyopathy has intrigued clinicians since the condition was first described in eight patients by Donald Teare almost 40 years ago.1 The classic morphology of asymmetrical septal hypertrophy remains the most common form of the condition, but the increased availability of echocardiography, together with family and routine screening has demonstrated a diversity of cardiac phenotype.2–5 Patients may be profoundly disabled or asymptomatic,6 and presentation may occur at any age.7–9 Although the early studies focused on the classic form of the disease, the heterogeneity of the cardiac phenotype precludes a satisfactory definition, and diagnostic difficulty is relatively common. A working definition is to regard hypertrophic cardiomyopathy as an idiopathic condition characterised by a hypertrophied and non-dilated left and/or right ventricle in the absence of an identified cause such as hypertension or valvular heart disease.10,11 A positive family history greatly strengthens the diagnosis in uncertain cases.12,13
Keywords
- Left Ventricular Hypertrophy
- Sudden Cardiac Death
- Hypertrophic Cardiomyopathy
- Myocardial Hypertrophy
- Cardiac Phenotype
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References
Teare RD. Asymmetrical hypertrophy of the heart in young adults. Brit Heart J 1958: 20: 1.
Maron BJ, et al. Patterns and significance of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients. Am J Cardiol 1981: 48: 418.
Shapiro LM, McKenna WJ. Distribution of left ventricular hypertrophy in hypertrophic car-diomuopathy: a two dimensional echocardiographic study. J Am Coll Cardiol 1983: 2: 437.
McKenna W, et al. Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am J Cardiol 1981: 47: 532.
Maron BJ, et al. How common is hypertrophic cardiomyopathy? Echocardiographically identified prevalance in a general population of young adults. (The CARDIA Study). Circulation 1993: 88: 211.
Spirito P, et al. Severe functional limitation in patients with hypertrophic cardiomyopathy and only mild localised left ventricular hypertrophy. J Am Coll Cardiol 1986: 8: 537.
Spirito P, Maron BJ. realtion between of left ventricular hypertrophy and age in hypertrophic cardiomyopathy. J Am Coll Cardiol 1989: 13: 820.
Lewis JF, Maron BJ. clinical and morphologic expression of hypertrophic cardiomyopathy in patients> or = 65 years of age. Am J Cardiol 1994: 73: 1105.
Pomerance A, Davies MJ. Patholical features of hypertrophic obstructive cardiomyopathy (HOCM) in the elderly. Brit Heart J 1975: 37: 305.
Maron BJ, Epstein SE. Hypertrophic cardiomyopathy: a discussion of nomenclature. Am J Cardiol 1979: 43: 1242.
Report of the WHO/IFSC task force on the definition and classification of cardiomyopathies. Brit Heart J 1980: 44: 672.
Greaves SC, et al. Inheritance of hypertrophic cardiomyopathy: a cross-sectional and M-mode study of 50 families. Brit Heart J 1987: 58: 259.
Maron BJ, et al. Patterns of inheritance in hypertrophic cardiomyopathy: assessment by M-mode and two-dimensional echocardiography. Am J Cardiol 1984: 53: 1087.
Lewis JF, et al. Usefulness of Doppler echocardiographic assessment of diastolic filling in distinguishing“athlete’s heart”from hypertrophic cardiomyopathy. Br Heart J 1992: 68: 296.
Cousineau D, et al. Catecholamines in coronary sinus during exrecise in man before and after training. J Appl Physiol 1977: 43: 801.
Coplan NL, et al. Exercise-related changes in serum catecholamines and potassium: effect of sustained exercise above and below lactate threshold. Am Heart J 1989: 117: 1070.
Lehman M, et al. Plasma catecholamines in trained and untrained volunteers during graduated exercise. Int J Sports Med 1981: 2: 143.
McKenna WJ, et al. Hypertrophic cardiomyopathy without hypertrophy: two families with myocardial disarray in the absence of increased myocardial mass. Br Heart J 1990: 63: 287.
Watkins H, et al. Characteristics and prognostic implications of myosin missense mutations in familial hypertrophic cardiomyopathy. N Engl J Med 1992: 326: 1108.
Epstein ND, et al. Differences in clinical expression of hypertrophic cardiomyopathy associated with two distinct mutations in the beta-myosin heavy chain gene. A 908Leu—> Val mutation and a 403Arg—> Gln mutation. Circulation 1992: 86: 345.
Hengstenberg C, Schwartz K. Molecular genetics of familial hypertrophic cardiomyopathy. J Mol Cell Cardiol 1994: 26: 3.
McKenna WJ, et al. Sudden death in hypertrophic cardiomyopathy: Identification and management of high risk patients. In: Advances in cardiomyopathies. Baroldi G, Camerini F, Goodwin JF. eds. Springer-Verlag, Berlin, 1990: 72.
Fananapazir L, Epstein ND. Genotype-phenotype correlations in hypertrophic cardiomyopathy. Insights provided by comparisons of kindreds with distinct and identical beta-myosin heavy chain gene mutations. Circulation 1994: 89: 22.
Hengstenberg C, et al. Clinical and genetical heterogeneity of familial hypertrophic cardiomyopathy. Herz 1994: 19: 84.
Solomon SD, et al. Left ventricular hypertrophy and morphology in familial hypertrophic cardiomyopathy associated with mutations of the beta-myosin heavy chain gene. J Am Coll Cardiol 1993: 22: 498.
Anan R, et al. Prognostic implications of novel beta cardiac myosin heavy chain gene mutations that cause familial hypertrophic cardiomyopathy. J Clin Invest 1994: 93: 280.
Davies MJ. the current status of myocardial disarray in hypertrophic cardiomyopathy. Brit Heart J 1984: 51: 361.
Maron BJ, et al. Realtion between extent of cardiac muscle cell disorganization and left ventricular wall thickness in hypertrophic cardiomyopathy. Am J Cardiol 1192: 70: 785.
Kuck KH, et al. Programmed electrical stilmulation in hypertrophic cardiomyopathy. Results in patients with and without cardiac arrest or synopse. Eur Heart J 1988: 9: 177.
Fananapazir L, et al. Prognostic determinants in hypertrophic cardiomyopathy. Prospective evaluation of a therapeutic strategy based on clinical, Holter, hemodynamic and electrophysiological findings. Circulation 1992: 86: 730.
Fananapazir L, Epstein SE. value of electrophysiologic studies in hypertrophic cardiomyopathy. Am J Cardiol 1191: 67: 175.
Saumarez RC, et al. Ventricular fibrillation in hypertrophic cardiomyopathy is associated with increased fractionation of paced right ventricular electrograms. Circulation 1992: 86: 467.
Lefroy DC, et al. Diffuse reduction of myocardial beta-adrenoceptors in hypertrophic cardiomyopathy: a study with positron emission tomography. J Am Coll Cardiol 1993: 22: 1653.
Davies MJ, Krikler DM. Genetic investigation and counselling of families with hypertrophic cardiomyopathy. Br Heart J 1994, 72: 99.
Al Mahdawi S, et al. The electrogram is a more sensitive indicator than echocardiography of hypertrophic cardiomyopathy in families with a mutation in the MYH7 gene. Br Heart J 1994: 72: 105.
Ryan MP, et al. The standard electrocardiogram as a screening test for hypertrophic cardiomyopathy. Am J Cardiol 1995: 17: 452.
Maron BJ, et al. Development or progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy: identification by two-dimensional echocardiography. New Engl J Med 1986: 315: 610.
Panza JA, et al. Development and determinants of dynamic obstructions to left ventricular outflow in young patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1989: 13: 820.
Spirito P, Maron BJ. Absence of progression of left ventricular hypertrophy in adult patients with hypertrophic cardiomyopathy. Am J Cardiol 1987: 9: 1013.
Spirito P, et al. Occurence and significance of progressive left ventricular wall thinning and relative cavity dilatation in patients with hypertrophic cardiomyopathy. Am J Cardiol 1987: 60: 123.
Maron BJ, et al. Prevalence of hypertrophic cardiomyopathy in an outpatient population referred for echocardiographic study. Am J Cardiol 1994: 73: 577.
Bohn M, et al. Insertion/deletion (I/D) polymorphism at the locus for angiotensin I-converting enzyme and parental history of myocardial infarction. Clin Genet 44: 298.
Schunkert H, et al. Association between a deletion polymorphism of the angiotensin-converting enzyme gene and left ventricular hypertrophy. N Engl J Med 1994: 330: 1634.
Marian AJ, et al. Angiotensin-converting enzyme polymorphism in hypertrophic cardiomyopathy and sudden cardiac death. Lancet 1993: 342: 1085.
Schunkert H, Paul M. Cardiac angiotensin converting enzyme and diastolic function of the heart. Agents Actions Suppl 38: 119.
Kofflard MJ, et al. Prognosis in hypertrophic cardiomyopathy observed in a large clinic population. Am J Cardiol 1993: 72: 939.
Maron BJ, Fananapazir L. Sudden cardiac death in hypertrophic cardiomyopathy. Circulation 1992: 85: 157.
Maron BJ, et al. Prognostic significance of 24-hour ambulatory monitoring in patients with hypertrophic cardiomyopathy. Am J Cardiol 1981: 48: 252.
Frenneaux P, et al. Abnormal blood pressure response during exercise in hypertrophic cardiomyopathy. Circulation 1991: 82: 1995.
McKenna WJ, et al. Syncope in hypertrophic cardiomyopathy. Brit Heart J 1982: 47: 177.
Counihan PJ, et al. Abnormal vascular responses to supine exercise in hypertrophic cardiomyopathy. Circulation 1991: 84: 686.
Nihoyannopoulos P, et al. Diastolic function in hypertrophic cardiomyopathy: relation to exercise capacity. J Am Coll Cardiol 1992: 19: 536.
Messmer BJ. Extended myectomy for hypertrophic obstructive cardiomyopathy. Am Thorac Surg 1994: 58: 575.
Delahaye F, et al. Postoperative and long-term prognosis of myotomy-myomectomy for obstructive hypertrophic cardiomyopathy: influence of associated mitral valve replacement. Eur Heart J 1993: 14: 1229.
Stone CD, et al. Operative treatment of pediatric obstructive hypertrophic cardiomyopathy: a 26-year experience. Ann thorac Surg 1993: 56: 1308.
Wagner JA, et al. Calcium-antagonist receptors in the atrial tissue of patients with hypertrophic cardiomyopathy. N Engl J Med 1989: 320: 755.
Doiuchi J, et al. Comparative effects of calcium-channel blockers and beta-adrenergic blocker on early diastolic time intervals and A-wave ratio in patients with hypertrophic cardiomyopathy. Clin Cardiol 1987: 10: 26.
Gilligan DM, et al. A double-blind, placebo-controlled crossover trial of nadolol and verapamil in mild and moderately symptomatic hypertrophic cardiomyopathy. J Am Coll Cardiol 1993: 21: 1672.
Fananapazir L, et al. Impact of dual-chamber permanent pacing in patients with obstructive hypertrophic cardiomyopathy with symproms refractory to verapamil and beta-adrenergic blocker therapy. Circulation 1992: 85: 2149.
Jeanrenaud X, et al. Effects of dual-chamber pacing in hypertrophic obstructive cardiomyopathy. Lancet 1992: 339: 1318.
McAreavey D, Fananapazir L. Altered cardiac hemodynamic and electrical state in normal sinus rhythm after chronic dual-chamber pacing for relief of left ventricular outflow obstruction in hypertrophic cardiomyopathy. Am J Cardiol 70: 651.
Cannon RO, et al. Results of permanent dual-chamber pacing in sympromatic nonobstructive hypertrophic cardiomyopathy. Am J Cardiol 1994: 73: 571.
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Dutka, D.P., Oakley, C.M. (1999). Hypertrophic Cardiomyopathy. In: Contemporary Concepts in Cardiology. Developments in Cardiovascular Medicine, vol 217. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-5007-5_15
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DOI: https://doi.org/10.1007/978-1-4615-5007-5_15
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