Right ventricular dysplasia in the elderly: a case report from autopsy

Right ventricular dysplasia (RVD) is a rare disease of the heart that primarily affects the right ventricle. It is a clinical and pathological entity that presents classically with palpitations, syncope, or even sudden death. It presents rarely in the elderly. Where sudden death is the first and onl...

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Main Authors: Alban Ikenna Mgbehoma, Oluwaseye Olumide Onayemi, Sunday Sokunle Soyemi, John Oladapo Obafunwa
Format: Article
Language:English
Published: The Pan African Medical Journal 2021-04-01
Series:The Pan African Medical Journal
Subjects:
Online Access: https://www.panafrican-med-journal.com/content/article/38/404/pdf/404.pdf
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author Alban Ikenna Mgbehoma
Oluwaseye Olumide Onayemi
Sunday Sokunle Soyemi
John Oladapo Obafunwa
author_facet Alban Ikenna Mgbehoma
Oluwaseye Olumide Onayemi
Sunday Sokunle Soyemi
John Oladapo Obafunwa
author_sort Alban Ikenna Mgbehoma
collection DOAJ
description Right ventricular dysplasia (RVD) is a rare disease of the heart that primarily affects the right ventricle. It is a clinical and pathological entity that presents classically with palpitations, syncope, or even sudden death. It presents rarely in the elderly. Where sudden death is the first and only presentation, an autopsy is required to make the diagnosis. However, the pathomorphological features of RVD can easily be overlooked or missed at autopsy. We report the case of a 68-year-old male with the past medical history of hypertension, gout and inflammatory bowel syndrome. He was admitted on account of difficulty in breathing, abdominal swelling and reduced urination. Physical examination revealed hypertension with cardiac murmurs, widespread crepitations, distended abdomen and lower limb oedema. Provisional diagnoses of acute-on-chronic kidney disease and congestive cardiac failure secondary to hypertensive heart disease, precipitated by probable gastrointestinal infection were made. While on admission, he had an episode of syncope. Electrocardiogram revealed bigeminy and bradycardic sinus rhythm with unifocal ventricular premature contraction. He died after 10 weeks of admission. Autopsy revealed an enlarged heart weighing 600gm; there was thinning of the apical aspect of the right ventricular wall with subtotal fibrofatty replacement. Microscopic examination revealed a transmural replacement of cardiac myocytes by fibroadipose tissue extending inwards, in the most parts, from the epicardium to the endocardial surface. Our aim is to increase the awareness of these pathomorphological features among anatomic/forensic pathologists.
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spelling doaj.art-d42bbbad1447468ab3c564aca82c3f5d2022-12-21T18:40:37ZengThe Pan African Medical JournalThe Pan African Medical Journal1937-86881937-86882021-04-013840410.11604/pamj.2021.38.404.2925029250Right ventricular dysplasia in the elderly: a case report from autopsyAlban Ikenna Mgbehoma0Oluwaseye Olumide Onayemi1Sunday Sokunle Soyemi2John Oladapo Obafunwa3 Department of Pathology and Forensic Medicine, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria Department of Pathology and Forensic Medicine, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria Department of Pathology and Forensic Medicine, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria Department of Pathology and Forensic Medicine, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria Right ventricular dysplasia (RVD) is a rare disease of the heart that primarily affects the right ventricle. It is a clinical and pathological entity that presents classically with palpitations, syncope, or even sudden death. It presents rarely in the elderly. Where sudden death is the first and only presentation, an autopsy is required to make the diagnosis. However, the pathomorphological features of RVD can easily be overlooked or missed at autopsy. We report the case of a 68-year-old male with the past medical history of hypertension, gout and inflammatory bowel syndrome. He was admitted on account of difficulty in breathing, abdominal swelling and reduced urination. Physical examination revealed hypertension with cardiac murmurs, widespread crepitations, distended abdomen and lower limb oedema. Provisional diagnoses of acute-on-chronic kidney disease and congestive cardiac failure secondary to hypertensive heart disease, precipitated by probable gastrointestinal infection were made. While on admission, he had an episode of syncope. Electrocardiogram revealed bigeminy and bradycardic sinus rhythm with unifocal ventricular premature contraction. He died after 10 weeks of admission. Autopsy revealed an enlarged heart weighing 600gm; there was thinning of the apical aspect of the right ventricular wall with subtotal fibrofatty replacement. Microscopic examination revealed a transmural replacement of cardiac myocytes by fibroadipose tissue extending inwards, in the most parts, from the epicardium to the endocardial surface. Our aim is to increase the awareness of these pathomorphological features among anatomic/forensic pathologists. https://www.panafrican-med-journal.com/content/article/38/404/pdf/404.pdf right ventricular cardiomyopathyright ventricular dysplasiacase report
spellingShingle Alban Ikenna Mgbehoma
Oluwaseye Olumide Onayemi
Sunday Sokunle Soyemi
John Oladapo Obafunwa
Right ventricular dysplasia in the elderly: a case report from autopsy
The Pan African Medical Journal
right ventricular cardiomyopathy
right ventricular dysplasia
case report
title Right ventricular dysplasia in the elderly: a case report from autopsy
title_full Right ventricular dysplasia in the elderly: a case report from autopsy
title_fullStr Right ventricular dysplasia in the elderly: a case report from autopsy
title_full_unstemmed Right ventricular dysplasia in the elderly: a case report from autopsy
title_short Right ventricular dysplasia in the elderly: a case report from autopsy
title_sort right ventricular dysplasia in the elderly a case report from autopsy
topic right ventricular cardiomyopathy
right ventricular dysplasia
case report
url https://www.panafrican-med-journal.com/content/article/38/404/pdf/404.pdf
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