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The Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

NCT03900208 · Status: COMPLETED · Type: OBSERVATIONAL · Enrollment: 10

Last updated 2019-04-04

No results posted yet for this study

Summary

The arrhythmogenic right ventricular cardiomyopathy, also known as arrhythmogenic right ventricular dysplasia (ARVC/D) is a rare myocardial disease with a prevalence estimated to range from 1 case on 5000 persons in the general population. It have a dominant genetic transmission characterized by alterations of desmosomial proteins and predominantly affects the right ventricle. The morphological alteration of the myocardium characterized by fibro-fatty substitution predisposes to arrhythmic events that can be fatal and cause death especially in young people and athletes. International guidelines provide a classification that includes clinical and histological criteria for diagnosis based on fibrous tissue substitution, percentage and right ventricle localization. Indeed, it is mainly affected the right ventricle but in some cases also the left ventricle result involved and the fatty tissue or fibro-fatty tissue with particular arrangement may affect the full-thickness wall are pathognomonic. Authors identified 10 cases of arrhythmogenic cardiomyopathy in forensic autopsy collocated in the 2003 to 2017, included 8 males and 2 females. Their age ranged from 16 to 45 years with an average age of 28.8±8.1 years. However, authors would like to demonstrate that with ARVC/D cannot be include only cases with increased fibrosis and exclusive localization to the right ventricle nor even cases with electrocardiographic alterations and other evident clinical criteria. Indeed, in the analyzed cases authors observed an amount of fibrosis often less than the percentage reported in the literature and an involvement not only of the right ventricle but also of the septum and associated sinister ventricular modifications. To this must be added that the cases of death did not have a sudden death of relatives in family history as described in guidelines and therefore this criterion is not reliable for the purpose of a classification.

Conditions

  • Right Ventricle Abnormality

Interventions

DIAGNOSTIC_TEST

Hystological analysis of ventricular tissue

Sections (4μm thick) will be stained with hematoxylin and eosin stain (H\&E) for diagnosis. After diagnosis, sections of the representative heart tissue will be stained with a PicroSirius Red/Fast Green for fibrosis evaluation. All stained samples will be examined under digital and light microscope. Beside the location of adipose/fibrous tissue in the right or left ventricles and in the septum, seven high magnification fields (40x) will be evaluated in five regions of the myocardium.

Sponsors & Collaborators

  • University of Campania Luigi Vanvitelli

    lead OTHER

Eligibility

Min Age
18 Years
Max Age
75 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2017-01-01
Primary Completion
2018-01-01
Completion
2019-01-01

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Read the full study record

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View NCT03900208 on ClinicalTrials.gov