Characterization of the electroanatomic substrate in a case of noncompaction left ventricle.
ABSTRACT An apparently healthy 43-year-old man was submitted to cardiological evaluation for sport activity. Two-dimensional echocardiography led to suspicion of noncompaction deformity, later confirmed by magnetic resonance imaging (MRI), left ventricular catheterization and endomyocardial biopsies. To exclude life-threatening arrhythmias, the patient was submitted to an electrophysiological study and to a real-time three-dimensional electroanatomic reconstruction of left ventricle. The electroanatomic map revealed extensive area of electrical abnormalities. Extent and localization of scar areas mostly corresponded to the areas of enhancement observed at MRI. The present report is the first on electroanatomic substrate evaluation in a noncompaction left ventricle. Our findings show that ventricular noncompaction is characterized by electrical abnormalities including low voltage and scar areas, mainly related to the presence and extent of myocardial fibrosis rather than noncompacted myocardium. Electroanatomic mapping may contribute to detect and quantify fibrotic areas in patients presenting this rare cardiomyopathy.
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ABSTRACT: Both supraventricular and ventricular rhythm disorders are frequently observed in patients with isolated left ventricular noncompaction (IVNC). Most importantly, these patients are prone to develop life-threatening ventricular arrhythmias, which are amongst their most frequent causes of death. Data regarding risk stratification of ventricular arrhythmias, however, are scarce due to the rareness of the disease. Indeed, even invasive electrophysiological studies may be of limited value in this regard in the majority of patients. Implantable cardioverter defibrillators (ICDs) have been demonstrated to be highly effective for the prevention of sudden arrhythmic death in IVNC and should be considered in patients who are clinically judged to be at high risk for ventricular tachyarrhythmias. These include patients with a severely reduced ejection fraction as well as those with a prior history of sustained ventricular tachycardia or fibrillation, recurrent syncope of unknown etiology, or a family history of ventricular tachyarrhythmias or sudden cardiac death. This review summarizes the electrocardiographic and electrophysiological findings in patients with IVNC and discusses possibilities for risk stratification as well as the rationale for ICD implantation for the prevention of sudden cardiac death.Annals of Medicine 06/2011; 44(2):101-8. DOI:10.3109/07853890.2011.554427 · 4.73 Impact Factor
Revista Espa de Cardiologia 07/2009; 62(7):822-823. DOI:10.1016/S0300-8932(09)71698-7 · 3.34 Impact Factor
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ABSTRACT: Left ventricular noncompaction (LVNC) is an uncommon cardiomyopathy characterized by the persistence of fetal myocardium with a pattern of prominent trabecular meshwork and deep intertrabecular recesses, systolic dysfunction and left ventricular dilatation. It is thought to be caused by the arrest of normal endomyocardial morphogenesis. There is no consensus on the definition, diagnostic criteria, pathogenesis or treatment of LVNC. We report the case of a 43 year-old patient with LVNC, nonsustained ventricular tachycardia and family history of sudden cardiac death (SCD). An implantable cardioverter-defibrillator (ICD) was prophylactically implanted because of the individual's high SCD risk. Although ICD is an effective option for preventing SCD, data on the long-term follow-up of patients with LVNC is limited.Cardiology journal 11/2011; 18(6):691-4. DOI:10.5603/CJ.2011.0035 · 1.22 Impact Factor