The Diagnosis and Treatment of Hypertrophic Cardiomyopathy

Abstract

A near-normal life expectancy and a highly satisfactory quality of life are now realistic treatment goals for patients with HCM.

Determining the role of fibrosis in hypertrophic cardiomyopathy

“…On imaging (Figures 3 & 4), wall thickening is the morphological hallmark of the disease [15,16,23]. Pump function, expressed as Review ejection fraction, usually remains normal for years, frequently along the entire life span.…”

“…An exercise test is thus mandatory to differentiate the two subtypes: in addition to those 30% of the HCM patients who have a resting gradient, another 30% only exhibit this obstruction with provocation. Only, a minority (again ~30%) of HCM-cases is 'truly' nonobstructive (HNCM) under all conditions [15,16,27].…”

“…HCM is an autosomal-dominant inherited cardiac condition, defined by wall thickening of the left and occasionally the right ventricle not explained by load conditions (e.g., aortic stenosis or arterial hypertension) [15][16][17][18]. The prevalence of HCM is estimated to be approximately one case per 500-1000 population.…”

“…The phenotype and the clinical expression of the disease are extremely heterogeneous [15][16][17][18]. The amount of wall thickening may range from merely detectable to massive -that is, >50 mm -while the condition may be compatible with athletic performance one one hand or cause disabling heart failure symptoms on the other.…”

“…However, subgroups exist with a more than fourfold risk (4-5%/year) [15][16][17]. The identification of these highrisk patients, with the aim of protecting them against arrhythmogenic death by implantation of an implantable cardioverter-defibrillator (ICD) is a matter of controversial debate.…”