RATIONALE Apoptosis signal-regulating kinase (ASK)1 is a central upstream kinase in the greater mitogen-activated protein kinase cascade that mediates growth and death decisions in cardiac myocytes in response to diverse pathological stimuli. OBJECTIVE However, the role that ASK1 plays in regulating the cardiac hypertrophic response in vivo remains controversial. METHODS AND RESULTS Here, w...

The clinical diagnostic hallmark of hypertrophic cardiomyopathy (HCM) is unexplained cardiac hypertrophy, commonly found on an echocardiogram and in unfortunate occasions, in an autopsy. The latter is most tragic as HCM, a relatively common disease (1) often presenting with sudden cardiac death (SCD) in apparently healthy young individuals (2,3). Indeed, HCM is considered the most common cause ...

This year marks the fiftieth anniversary of the first contemporary account of hypertrophic cardiomyopathy by Dr Robert Donald Teare, a pathologist working at St George’s hospital in London. In a now seminal case series in the British Heart Journal, Teare reported asymmetric hypertrophy of the interventricular septum in 8 patients between the ages of 14 and 44.1 Although widely recognised as a c...

BACKGROUND Autonomic dysfunction has been found to be a powerful predictor of arrhythmic events and sudden death after myocardial infarction. Hypertrophic cardiomyopathy carries a risk of sudden death and this risk is increased by the occurrence of syncope. OBJECTIVES To determine if autonomic dysfunction occurs in patients with hypertrophic cardiomyopathy and if it is associated with the occ...

BACKGROUND In the early 1980s, studies performed in highly selected referral patients with hypertrophic cardiomyopathy reported a strong association between the presence of brief episodes of ventricular tachycardia (VT) on ambulatory ECG monitoring and sudden death. These observations led to antiarrhythmic treatment in many patients with hypertrophic cardiomyopathy and brief episodes of VT. In ...

OBJECTIVES We sought to identify patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden death (SD). BACKGROUND Relatively low mortality rates in HCM make conventional analysis of multiple clinical risk markers for SD problematic. This study used a referral center registry to investigate a smaller number of generally accepted noninvasive risk markers. METHODS We studied 368 p...

Although idiopathic cardiomyopathies are prognostically important and are a common indication for cardiac transplantation in all age groups, the incidence and age distribution of idiopathic cardiomyopathies in a well-defined pediatric population have been poorly characterized. A retrospective study was carried out in Finland in 1980-1991 to obtain information on the epidemiology of childhood ca...

Aims To perform a systematic literature review and meta-analysis of clinical risk factors for sudden cardiac death (SCD) in childhood hypertrophic cardiomyopathy. Methods Medline and PubMed databases were searched for original articles published in English from 1963 through to December 2015 that included patients under 18 years of age with a primary or secondary end-point of either SCD or SCD-e...

INTRODUCTION We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. METHODS A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. RESULTS A total of 29 centers participated and 1042 patients were rec...

a hypertrophic scar is defined as an excess healing response that is a dilemma for physicians. several therapies are available: intralesional corticosteroids, topical treatments, cryotherapy, surgery, radiation, silicone gel dressing and laser therapy.pulsed-dye, nd-yag and co2 lasers have been used for treatment of keloids and hypertrophic scars but recurrence is common. recently low-level las...