Hypertrophic cardiomyopathy natural history
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Editors-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The natural history of hypertrophic cardiomyopathy is quite variable. Signs and symptoms range from none, to atrial fibrillation, to heart failure, to embolic stroke, to sudden cardiac death[1][2][3][4][5]. Signs and symptoms are quite variable from individual to individual but are also quite variable within a given family (all of whom carry the same mutation). The disease is quite variable in the timing of its appearance and may appear anywhere from infancy to late in adult life. About 25% of HCM patients achieve normal longevity[6][7][8][9].
Time and Age Dependent Appearance of Left Ventricular Hypertrophy
Left ventricular hypertrophy may be absent in childhood. It may then appear following the rapid growth of adolescence and may first appear at age 17 to 18[10][11][12].
Sudden Cardiac Death
The incidence of sudden cardiac death (SCD) in patients with HCM is 2 to 4 percent per year in adults, and a 4 to 6 percent per year in children and adolescents[13].
Among end stage patients with a left ventricular ejection fraction < 50%, the risk of SCD over 5 years may be as high as 47%. In this population, syncope has been identified as an independent correlate of sudden cardiac death (hazard ratio = 6.15; 95% confidence interval, 2.40-15.75; P < .001)[14].
A review of 78 patients with HCM who died suddenly or survived a cardiac arrest episode showed that 71 percent were younger than 30 years of age, 54 percent were without functional limitation, and 61 percent were performing sedentary or minimal physical activity at the time of cardiac arrest.
Predictors of Sudden Cardiac Death
There are few predictors of SCD in patients with HCM.
- Onset of symptoms in childhood [15][16].
- A clinical history of spontaneous, sustained monomorphic ventricular tachycardia (VT) or sudden death in family members.
- History of impaired consciousness
- Syncope
- Atrial arrhythmias
- Development of systolic dysfunction
- Non-sustained ventricular tachycardia (NSVT) in patients with symptoms
- Left ventricular wall thickness >30 mm. A recent report of 480 patients showed that left ventricular wall thickness was useful in identifying patients at high risk for sudden cardiac death. However, sudden cardiac death can occur in children and adolescents in the absence of left ventricular hypertrophy as well.
Prognosis in Survivors of Sudden Cardiac Death
Survivors of SCD have a poor prognosis. Event free survival at 1,5 and 10 years was 83, 65 and 53 percent respectively.
References
- ↑ Maron BJ. Hypertrophic cardiomyopathy. Lancet 1997;350:127–33.
- ↑ Maron BJ. Hypertrophic cardiomyopathy. A systematic review. JAMA 2002;287:1308–20.
- ↑ Maki S, Ikeda H, Muro A et al. Predictors of sudden cardiac death in hypertrophic cardiomyopathy. Am J Cardiol 1998;82:774–8.
- ↑ Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 1999;281:650–5.
- ↑ Maron BJ, Olivotto I, Bellone P et al. Clinical profile of stroke in 900 patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2002;39:301–7.
- ↑ Maron BJ. Hypertrophic cardiomyopathy. A systematic review. JAMA 2002;287:1308–20.
- ↑ Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 1999;281:650–5.
- ↑ Fay WP, Taliercio CP, Ilstrup DM, Tajik AJ, Gersh BJ. Natural history of hypertrophic cardiomyopathy in the elderly. J Am Coll Cardiol 1990;16:821–6.
- ↑ Takagi E, Yamakado T, Nakano T. Prognosis of completely asymptomatic adult patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1999;33:206–11.
- ↑ Hagege AA, Dubourg O, Desnos M et al. Familial hypertrophic cardiomyopathy. Cardiac ultrasonic abnormalities in genetically affected subjects without echocardiographic evidence of left ventricular hypertrophy. Eur Heart J 1998;19:490–9.
- ↑ 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;315:610–4.
- ↑ Spirito P, Maron BJ. Absence of progression of left ventricular hypertrophy in adult patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1987;9:1013–7.
- ↑ Elliott PM, Poloniecki J, Dickie S, Sharma S, Monserrat L, Varnava A; et al. (2000). "Sudden death in hypertrophic cardiomyopathy: identification of high risk patients". J Am Coll Cardiol. 36 (7): 2212–8. PMID 11127463.
- ↑ Kawarai H, Kajimoto K, Minami Y, Hagiwara N, Kasanuki H (2011). "Risk of sudden death in end-stage hypertrophic cardiomyopathy". J Card Fail. 17 (6): 459–64. doi:10.1016/j.cardfail.2011.01.015. PMID 21624733.
- ↑ Maron BJ, Tajik AJ, Ruttenberg HD et al. Hypertrophic cardiomyopathy in infants. Clinical features and natural history. Circulation 1982; 65:7–17
- ↑ Skinner JR, Manzoor A, Hayes AM, Joffe HS, Martin RP. A regional study of presentation and outcome of hypertrophic cardiomyopathy in infants. Heart 1997;77:229–33.