Case reportSudden Cardiac Arrest in Hypertrophic Cardiomyopathy in the Absence of Conventional Criteria for High Risk Status
Section snippets
Case Descriptions
Patient 1, a 21-year-old asymptomatic college student, had been followed for 10 years after the fortuitous identification of HC at 11 years of age (Table 1). At 21 years of age, cardiovascular magnetic resonance (CMR) was performed as part of a routine annual evaluation. Left ventricular (LV) hypertrophy was confined to the anterior portion of ventricular septum (thickness 21 mm). Mitral valve systolic anterior motion and LV outflow obstruction were absent at rest (Figure 1). Of particular
Comments
The 2 patients with HC reported here demonstrate that the current strategy for assessing high-risk status in HC, although useful as a guide to selecting patients for prophylactic ICD therapy,1, 2, 3, 4 nevertheless is not precise and harbors certain limitations.1, 4 Such experiences underscore the importance of defining additional criteria for the selection of young high-risk patients who would benefit from ICD implantation. This is a crucial consideration, given that ICDs have proved effective
References (19)
- et al.
American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathyA report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines Committee to Develop an Expert Consensus Document on Hypertrophic Cardiomyopathy
J Am Coll Cardiol
(2003) - et al.
Sudden death in hypertrophic cardiomyopathy: identification of high risk patients
J Am Coll Cardiol
(2000) - et al.
Long-term follow-up of implantable cardioverter-defibrillator therapy for hypertrophic cardiomyopathy
Am J Cardiol
(2004) - et al.
Gadolinium-enhanced magnetic resonance imaging in hypertrophic cardiomyopathy: in vivo imaging of the pathologic substrate for premature cardiac death?
J Am Coll Cardiol
(2003) - et al.
The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy
J Am Coll Cardiol
(2004) - et al.
Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance
J Am Coll Cardiol
(2003) - et al.
Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy
J Am Coll Cardiol
(1986) - et al.
Hypertrophic cardiomyopathy and sudden death in the young: pathologic evidence of myocardial ischemia
Hum Pathol
(2000) - et al.
Papillary muscle fibrosis in primary myocardial disease
Am Heart J
(1969)
Cited by (69)
Relation of Late Gadolinium Enhancement and Extracellular Volume Fraction to Ventricular Arrhythmias in Hypertrophic Cardiomyopathy
2020, American Journal of CardiologySudden Cardiac Death and Ventricular Arrhythmias in Hypertrophic Cardiomyopathy
2019, Heart Lung and CirculationAbnormalities of the Mitral Apparatus in Hypertrophic Cardiomyopathy: Echocardiographic, Pathophysiologic, and Surgical Insights
2016, Journal of the American Society of EchocardiographyCitation Excerpt :Fibrosis confined solely to the PMs is rare, and its clinical significance is not known. An intriguing report93 of sudden death in an asymptomatic 15-year-old patient with HCM and isolated PM fibrosis, but no conventional sudden death risk factors, raises the possibility that this lesion has the potential to trigger lethal arrhythmias. A number of clinical entities that cause increased LV wall thickness must be differentiated from HCM.
Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies
2015, Journal of the American College of CardiologyCitation Excerpt :Six of these deaths occurred 5 ± 2 years after they had declined a formal recommendation for prophylactic ICD therapy, at 47 ± 8 years of age (1–7,12). Eleven additional patients did not receive ICDs, either because they were judged to be at low risk without markers (7,28,29) or died in the 1990s, prior to systematic use of ICDs for HCM (15). None of the 40 HCM patients who died had obstructive coronary artery disease on the basis of angiography, angina, myocardial infarction, or acute coronary event.
Hypertrophic cardiomyopathy and sports
2015, Cor et Vasa