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The recognized markers of risk in HCM and their sensitivity, specificity, and positive and negative predictive accuracy (PPA and NPA)

The recognized markers of risk in HCM and their sensitivity, specificity, and positive and negative predictive accuracy (PPA and NPA)
Risk factor Sensitivity, percent Specificity, percent PPA, percent NPA, percent
Abnormal blood pressure response: <40 years old[1] 75 66 15 97
NSVT: adult <45 years old[2] 69 80 22 97
NSVT: ≤21 years old[3] <10 89 <10 85
Inducible VT/VF: high risk population[4] 82 68 17 98
Syncope: <45 years old*[5] 35 82 25 86
Family history: at least one unexplained sudden death ± HCM*[5] 42 79 28 88
LVH ≥3 cm¶[6] 26 88 13 95
Two or more risk factors¶Δ[7] 45 90 23 96
HCM: hypertrophic cardiomyopathy; LVH: left ventricular hypertrophy; ICD: implantable cardioverter-defibrillator; NPA: negative predictive accuracy; NSVT: nonsustained ventricular tachycardia; PPA: positive predictive accuracy; VF: ventricular fibrillation; VT: ventricular tachycardia.
* Figures provided are for the risk of death from all causes rather than sudden death only.
¶ Figures provided are for risk of sudden death and/or appropriate ICD discharge.
Δ In this data set from Elliott and colleagues, family history and syncope were combined in order to achieve statistical significance of relative risk.
1. McKenna WJ, Franklin RC, Nihoyannopoulos P, et al. Arrhythmia and prognosis in infants, children and adolescents with hypertrophic cardiomyopathy. J Am Coll Cardiol 1988; 11:147.
2. Maron BJ, Savage DD, Wolfson JK, et al. Prognostic significance of 24 hour ambulatory electrocardiographic monitoring in patients with hypertrophic cardiomyopathy: a prospective study. Am J Cardiol 1981; 48:252.
3. McKenna WJ, Oakley CM, Krikler DM, et al. Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia. Br Heart J 1985; 53:412.
4. Fananapazir L, Chang AC, Epstein SE, McAreavey D. Prognostic determinants in hypertrophic cardiomyopathy. Prospective evaluation of a therapeutic strategy based on clinical, Holter, hemodynamic, and electrophysiological findings. Circulation 1992; 86:730.
5. McKenna W, Deanfield J, Faruqui A, et al. Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am J Cardiol 1981; 47:532.
6. Elliott PM, Gimeno BJ, Mahon NG, et al. Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet 2001; 357:420.
7. 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.
Reproduced with permission from: McKenna, WJ, Behr, ER. Hypertrophic cardiomyopathy: management, risk stratification, and prevention of sudden death. Heart 2002; 87:169. Copyright © 2002 BMJ Publishing Group, Ltd.
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