Literature DB >> 8774331

Familial right ventricular dysplasia with biventricular involvement and inflammatory infiltration. Heart Muscle Disease Study Group.

B Pinamonti1, D Miani, G Sinagra, R Bussani, F Silvestri, F Camerini.   

Abstract

The aetiology of right ventricular dysplasia/cardiomyopathy is presently unknown. A genetic background has been suggested, but myocarditis may play a part in its pathogenesis. Two familial cases of right ventricular dysplasia, one of whom had also a diagnosis of myocarditis, are reported. Both patients presented with ventricular arrhythmias. The father subsequently had a "flu-like" syndrome, heart failure, and biventricular dysfunction; "active" myocarditis was found at endomyocardial biopsy. Then the patient died suddenly. The daughter developed progressive biventricular dysfunction; then she was resuscitated from a cardiac arrest, and subsequently died suddenly. In both patients necropsy showed severe right ventricular atrophy and fibro-adipose substitution, associated with biventricular fibrosis. Inflammatory infiltration was also present in the first patient. This study shows the association of right ventricular dysplasia and myocarditis in the same family. These cases may represent a link between inherited and acquired ("inflammatory") forms of the disease.

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Year:  1996        PMID: 8774331      PMCID: PMC484429          DOI: 10.1136/hrt.76.1.66

Source DB:  PubMed          Journal:  Heart        ISSN: 1355-6037            Impact factor:   5.994


  18 in total

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  3 in total

1.  Sudden death after major head and neck cancer surgery due to undetected arrhythmogenic right ventricle cardiomyopathy (ARVC).

Authors:  Ákos Bicsák; Alice Jellen; Herwig Tuppy; Wolfgang Paul Poeschl
Journal:  Oral Maxillofac Surg       Date:  2016-06-29

2.  No detection of enteroviral genome in the myocardium of patients with arrhythmogenic right ventricular cardiomyopathy.

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Journal:  J Clin Pathol       Date:  2000-05       Impact factor: 3.411

3.  Arrhythmogenic Right Ventricular Cardiomyopathy in Pediatric Patients: An Important but Underrecognized Clinical Entity.

Authors:  Anneline S J M Te Riele; Cynthia A James; Hugh Calkins; Adalena Tsatsopoulou
Journal:  Front Pediatr       Date:  2021-12-02       Impact factor: 3.418

  3 in total

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