Case report
Sudden Cardiac Arrest in Hypertrophic Cardiomyopathy in the Absence of Conventional Criteria for High Risk Status

https://doi.org/10.1016/j.amjcard.2007.09.101Get rights and content

Two patients with hypertrophic cardiomyopathy are reported from the recent experience of the Hypertrophic Cardiomyopathy Center of the Minneapolis Heart Institute Foundation, demonstrating limitations in the risk stratification algorithm currently used for this disease. One patient, an asymptomatic 21-year-old male college student, was prophylactically implanted with a cardioverter-defibrillator. This decision was based largely on the presence of apparent extensive myocardial fibrosis identified by contrast-enhanced cardiovascular magnetic resonance imaging, currently not considered a risk factor in this disease. Fifteen months later, ventricular fibrillation was interrupted by an appropriate defibrillator shock. The other patient, an asymptomatic 15-year-old male subject without any apparent high-risk markers, died suddenly at home. Necropsy examination of the heart identified scarring confined to portions of both left ventricular papillary muscles, possibly representing a substrate for ventricular tachyarrhythmias. In conclusion, these 2 cases demonstrate that present strategies for assessing high-risk status in hypertrophic cardiomyopathy are inadequate to identify all such patients. However, while the anecdotal nature of these observations cannot yet justify altering the general guidelines for implantation of defibrillators for the primary prevention of sudden death related to hypertrophic cardiomyopathy, 1 of our 2 cases suggests a future role for contrast-enhanced cardiovascular magnetic resonance in the risk stratification of this complex disease.

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)

There are more references available in the full text version of this article.

Cited by (69)

  • Abnormalities of the Mitral Apparatus in Hypertrophic Cardiomyopathy: Echocardiographic, Pathophysiologic, and Surgical Insights

    2016, Journal of the American Society of Echocardiography
    Citation 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 Cardiology
    Citation 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.

View all citing articles on Scopus
View full text