Hypertrophic cardiomyopathy (HCM) is characterized by extreme clinical heterogeneity, ranging from sudden cardiac death to long-term disease progression and heart failure-related complications. Myocardial ischemia, occurring at the microvascular level, is a major determinant of clinical expression and outcome. Accordingly, the severity of this microvascular dysfunction has been shown to represent an early and powerful predictor of unfavorable outcome in HCM. The assessment of microvascular function in vivo is technically challenging, although critical to a truly comprehensive evaluation and risk stratification of HCM patients. Available technologies include positron emission tomography and cardiac magnetic resonance (CMR). Studies of regional myocardial blood flow using positron emission tomography have demonstrated that the vasodilator response to dipyridamole is impaired in most HCM patients, not only in the hypertrophied ventricular septum but also in the less hypertrophied or non-thickened left ventricular free wall. CMR also allows measurement of myocardial flow, although the technique is currently time-consuming and largely limited to research situations. CMR provides further insight into the effects of ischemia in HCM patients, by visualizing the distribution and extent of fibrosis at the intramyocardial level. Late gadolinium enhancement (LGE) is a potential predictor of risk in HCM patients, and is believed to largely reflect replacement fibrosis resulting from recurrent microvascular ischemia. LGE is associated with increased prevalence of ventricular arrhythmias, and associated with microvascular dysfunction. The present review is to provide a concise overview for the available evidence of microvascular ischemia and its consequences in HCM.
Cardiomyopathy, Hypertrophic ; diagnostic imaging ; pathology ; Humans ; Magnetic Resonance Imaging ; Myocardium ; pathology ; Positron-Emission Tomography

Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden cardiac death in young people. The implantable cardioverter-defibrillator (ICD), has recently proved to be a safe and effective therapeutic intervention in patients with HCM, both for the primary and secondary prevention of sudden death. Based on recent substantial experience, the ICD intervenes appropriately to terminate ventricular tachycardia/fibrillation (VT/VF), at a rate of 5.5%/year. ICD discharge rate is 4%/year in those patients implanted prophylactically due to one or more major risk markers, but often with considerable delays of up to 10 years before the device is required to intervene appropriately to terminate potentially letal ventricular tachyarrhythmias. Primary prevention of VT/VF occurs with similar frequency in high-risk patients having either 1, 2 or > or =3 noninvasive risk markers, and about one-third of patients with appropriate device interventions had been implanted for only one risk factor. The ICD has proved reliable in HCM despite the extreme and complex phenotypes often present with massive degrees of left ventricular hypertrophy, microvascular ischemia, diastolic dysfunction, or dynamic left ventricular outflow tract obstruction. Failure to convert life-threatening ventricular tachyarrhythmias to normal rhythm is extraordinarily rare. In conclusion, in high-risk HCM patients, ICDs perform in a highly effective fashion, frequently preventing sudden death by aborting primary life-threatening ventricular tachyarrhythmias. A single marker of high risk can be sufficient evidence to justify the recommendation for a prophylactic ICD in selected patients with HCM.
Adolescent ; Adult ; Cardiomyopathy, Hypertrophic ; complications ; prevention & control ; therapy ; Death, Sudden, Cardiac ; etiology ; prevention & control ; Defibrillators, Implantable ; Humans ; Middle Aged ; Retrospective Studies

The natural history of hypertrophic cardiomyopathy (HCM) is extremely heterogeneous. Many patients remain asymptomatic throughout life, some develop severe symptoms of heart failure, but others die suddenly, often in the absence of previous symptoms and at a young age. Therefore, identification of those patients at high risk of sudden death represents a major clinical problem and has become an even greater challenge since the implantable cardioverter-defibrillator (ICD) has proved to be highly effective in preventing sudden death in HCM. Patients who have survived a cardiac arrest, or one or more episodes of sustained ventricular tachycardia, are considered to be at high risk and are candidates for an ICD. However, this patient subset represents a small proportion of the HCM population. The greatest difficulty concerns the identification of high risk patients who are candidates for primary prevention of sudden death with a prophylactic ICD. Decisions are based on generally accepted clinical markers which are associated with increased risk, including: family history of sudden death, extreme left ventricular (LV) wall thickness ( > or =30 mm), nonsustained ventricular tachycardia on Holter monitoring, unexplained (non-neurocardiogenic) syncope particularly in young patients, and hypotensive blood pressure response to exercise. Patients with end-stage HCM or a LV apical aneurysm represent important arrhythmogenic subsets also associated with substantially increased risk. Multiple or single strong risk markers are associated with increased sudden death risk and justify consideration for a prophylactic ICD.
Cardiomyopathy, Hypertrophic ; complications ; Death, Sudden ; etiology ; Humans ; Risk Assessment ; methods ; Risk Factors

Atrial fibrillation (AF) is the most common sustained arrhythmia in patients with hypertrophic cardiomyopathy (HCM), and represents an important complication in the clinical course of the disease, with adverse consequences on functional status and outcome. Studies on community-based HCM patient populations have shown that AF is associated with long-term clinical deterioration, cardioembolic stroke and increased cardiovascular mortality due to heart failure and stroke. Moreover, acute onset of AF may cause severe hemodynamic impairment and represent a trigger of potentially lethal ventricular arrhythmias. However, the consequences of AF on the long-term prognosis of HCM patients are not uniformly unfavorable, and may be compatible with an uneventful course, when properly managed. Management of AF in HCM is challenging, particularly when onset occurs at a young age. Both paroxysmal and permanent AF represent clear indications for oral anticoagulation. In most patients, maintenance of sinus rhythm is highly desirable but made difficult by the limited long-term efficacy and potentially hazardous side effects of available pharmacological options. In selected patients with HCM and severely symptomatic AF, radiofrequency catheter ablation may represent an effective therapeutic alternative, improving functional status, and reducing or postponing the need for antiarrhythmic drugs. In patients with persistent AF, in whom maintenance of sinus rhythm is not feasible, adequate ventricular rate control should be pursued aggressively by atrio-ventricular node blocking agents.
Atrial Fibrillation ; epidemiology ; therapy ; Cardiomyopathy, Hypertrophic ; epidemiology ; therapy ; Humans ; Incidence ; Prognosis ; Risk Assessment