Hypertrophic cardiomyopathy natural history: Difference between revisions
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(/* ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) {{cite journal| author=Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M et al.| title=AC...) |
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' EP testing may be considered for risk assessment for SCD in patients with HCM. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki> | |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' EP testing may be considered for risk assessment for SCD in patients with HCM. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki> | ||
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Amiodarone may be considered for primary prophylaxis against SCD in patients with HCM who | |||
have 1 or more major risk factor for SCD (see Table 7) if ICD implantation is not feasible. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>|} | |||
==Prognosis in Survivors of Sudden Cardiac Death== | ==Prognosis in Survivors of Sudden Cardiac Death== | ||
Revision as of 20:10, 4 October 2012
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Hypertrophic Cardiomyopathy Microchapters |
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Differentiating Hypertrophic Cardiomyopathy from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Hypertrophic cardiomyopathy natural history On the Web |
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Directions to Hospitals Treating Hypertrophic cardiomyopathy |
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Risk calculators and risk factors for Hypertrophic cardiomyopathy natural history |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-In-Chief: Lakshmi Gopalakrishnan, M.B.B.S. [2]
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]. The myosin binding proteins C genetic variant carries a good prognosis. The presence of VT / VF carries the poorest prognosis. The severity of the outflow gradient is also related to prognosis. Athletes should be screened for HOCM based upon a family history of sudden cardiac death and a murmur on physical examination. Electrocardiograms and echocardiograms are not cost effective screening tools in this population with a low pre-test probability of disease.
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.
ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) [17]
Recommendations for Hypertrophic Cardiomyopathy
| Class I |
| "1. ICD therapy should be used for treatment in patients with HCM who have sustained VT and/or VF and who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: B)" |
| Class IIa |
| "1. ICD implantation can be effective for primary prophylaxis against SCD in patients with HCM who have 1 or more major risk factor (see Table 7) for SCD and who are receiving chronic optimal medical therapy and in patients who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)" |
| "2. Amiodarone therapy can be effective for treatment in patients with HCM with a history of sustained VT and/or VF when an ICD is not feasible. (Level of Evidence: C)" |
| Class IIb | |||||||||||||||||
| "1. EP testing may be considered for risk assessment for SCD in patients with HCM. (Level of Evidence: C)" | |||||||||||||||||
| "2. Amiodarone may be considered for primary prophylaxis against SCD in patients with HCM who
have 1 or more major risk factor for SCD (see Table 7) if ICD implantation is not feasible. (Level of Evidence: C)"|} Prognosis in Survivors of Sudden Cardiac DeathSurvivors of SCD have a poor prognosis. Event free survival at 1,5 and 10 years was 83, 65 and 53 percent respectively.
2011 ACCF/AHA Guideline Recommendations: SCD Risk Stratification
Guideline Resources2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy [18][19] References
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