title: Nonischemic left ventricular scar and cardiac sudden death in the young.
abstract: Nonischemic left ventricular scar (NLVS) is a pattern of myocardial injury characterized by midventricular and/or subepicardial gadolinium hyperenhancement at cardiac magnetic resonance, in absence of significant coronary artery disease. We aimed to evaluate the prevalence of NLVS in juvenile sudden cardiac death and to ascertain its etiology at autopsy. We examined 281 consecutive cases of sudden death of subjects aged 1 to 35 years. NLVS was defined as a thin, gray rim of subepicardial and/or midmyocardial scar in the left ventricular free wall and/or the septum, in absence of significant stenosis of coronary arteries. NLVS was the most frequent finding (25%) in sudden deaths occurring during sports. Myocardial scar was localized most frequently within the left ventricular posterior wall and affected the subepicardial myocardium, often extending to the midventricular layer. On histology, it consisted of fibrous or fibroadipose tissue. Right ventricular involvement was always present. Patchy lymphocytic infiltrates were frequent. Genetic and molecular analyses clarified the etiology of NLVS in a subset of cases. Electrocardiographic (ECG) recordings were available in more than half of subjects. The most frequent abnormality was the presence of low QRS voltages (<0.5 mV) in limb leads. In serial ECG tracings, the decrease in QRS voltages appeared, in some way, progressive. NLVS is the most frequent morphologic substrate of juvenile cardiac sudden death in sports. It can be suspected based on ECG findings. Autopsy study and clinical screening of family members are required to differentiate between arrhythmogenic right ventricular cardiomyopathy/dysplasia and chronic acquired myocarditis.
authors: Department of Radiological, Oncological and Pathological Sciences, Sapienza, University of Rome, Rome, Italy 00161.
authors: Cira R T
authors: CR
authors: di Gioia
authors: Department of Radiological, Oncological and Pathological Sciences, Sapienza, University of Rome, Rome, Italy 00161.
authors: Carla
authors: C
authors: Giordano
authors: Department of Radiological, Oncological and Pathological Sciences, Sapienza, University of Rome, Rome, Italy 00161.
authors: Bruna
authors: B
authors: Cerbelli
authors: Department of Radiological, Oncological and Pathological Sciences, Sapienza, University of Rome, Rome, Italy 00161.
authors: Annalinda
authors: A
authors: Pisano
authors: Department of Radiological, Oncological and Pathological Sciences, Sapienza, University of Rome, Rome, Italy 00161.
authors: Elena
authors: E
authors: Perli
authors: Department of Biomedicine and Prevention, University of Rome Tor Vergata, Rome, Italy 00173.
authors: Enrico
authors: E
authors: De Dominicis
authors: Sports Medicine Unit, Catholic University of Sacred Heart, Rome, Italy 00168.
authors: Barbara
authors: B
authors: Poscolieri
authors: Sports Medicine Unit, Catholic University of Sacred Heart, Rome, Italy 00168.
authors: Vincenzo
authors: V
authors: Palmieri
authors: Department of Anatomical, Histological, Forensic and Orthopaedic Sciences, Sapienza, University of Rome, Rome, Italy 00161.
authors: Costantino
authors: C
authors: Ciallella
authors: Sports Medicine Unit, Catholic University of Sacred Heart, Rome, Italy 00168.
authors: Paolo
authors: P
authors: Zeppilli
authors: Department of Radiological, Oncological and Pathological Sciences, Sapienza, University of Rome, Rome, Italy 00161. Electronic address: giulia.damati@uniroma1.it.
authors: Giulia
authors: G
authors: d'Amati
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D001344:Autopsy; D001706:Biopsy; D009202:Cardiomyopathies / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D002921:Cicatrix / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology; D004252:DNA Mutational Analysis; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000473:pathology*; D003937:Diagnosis, Differential; D004562:Electrocardiography; D005260:Female; D005819:Genetic Markers; D020022:Genetic Predisposition to Disease; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008297:Male; D025202:Molecular Diagnostic Techniques; D009154:Mutation; D009206:Myocardium / Q000378:metabolism*; D010641:Phenotype; D011237:Predictive Value of Tests; D015995:Prevalence; D012307:Risk Factors; D055815:Young Adult
chemical_list: D005819:Genetic Markers
keywords: ARVC/D; Cardiac sudden death; LDAC; Myocarditis; Nonischemic left ventricular scar