Myocardial fibrosis in patients with hypertrophic cardiomyopathy and high risk for sudden death

• Published in: Arquivos brasileiros de cardiologia 2010-04, Vol.94 (4), p.535-540
• Main Authors: Shiozaki, Afonso Akio, Senra, Tiago, Arteaga, Edmundo, Pita, Cristiane Guedes, Martinelli Filho, Martino, Avila, Luis Francisco R, Parga Filho, José Rodrigues, Mady, Charles, Rochitte, Carlos Eduardo
• Format: Article
• Language: Portuguese
• Subjects: Adult; Cardiomyopathy, Hypertrophic - complications; Cardiomyopathy, Hypertrophic - pathology; Cardiomyopathy, Hypertrophic - therapy; Chi-Square Distribution; Death, Sudden, Cardiac - etiology; Defibrillators, Implantable; Electrodes, Implanted; Female; Fibrosis; Humans; Male; Myocardium - pathology; Risk Factors; Tomography, X-Ray Computed - methods
• ISSN: 1678-4170
• DOI: 10.1590/S0066-782X2010005000017

The stratification of risk for sudden death in hypertrophic cardiomyopathy (HCM) continues to be a true challenge due to the great heterogeneity of this disease's presentation, as most individuals remain asymptomatic during their entire lives and others present sudden death as first symptom. Recent studies have suggested that myocardial fibrosis may represent an important substrate for the malignant ventricular arrhythmias, that are responsible for the cases of sudden death related to this disease. To assess the prevalence and quantification of myocardial fibrosis (MF) in hypertrophic cardiomyopathy (HCM) patients with implantablecardioverter - defibrillator (ICD) indicated due to their high risk or recovered from cardiac sudden death. Twenty-eight HCM patients with ICD were submitted to multidetector computed tomography to assess myocardial fibrosis by delayed enhancement technique. Myocardial fibrosis was present in 96% of these HCM patients with (20.38 +/- 15.55 g) comprising 15.96 +/- 10.20% of the total myocardial mass. MF was observed in a significantly higher prevalence as compared to other classical risk factors for sudden death. It is possible to conclude that there is a high prevalence of myocardial fibrosis in hypertrophic cardiomyopathy patients with high-risk or recovered from cardiac sudden death, like those with clinical indication to implantable cardioverter -defibrillator. The higher prevalence of myocardial fibrosis in comparison to classical risk factors of worse prognosis raise the hypothesis that the myocardial fibrosis may be an important substrate in the genesis of lifethreatening arrhythmias in these high risk HCM population.