"Staghorn" heart. The pathology of the explanted heart of a patient with clinical diagnosis and genetic variants of non-compacted myocardiopathy
Copyright © 2019 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved..
Non-compacted myocardiopathy is rare, the prevalence ranging between 0.01-0.26%. in adults. We present the macroscopic, microscopic and electron microscopy findings of cardiac transplant samples from a 36-year-old patient diagnosed with non-compacted myocardiopathy. This condition shows a high genetic and phenotypic heterogeneity, with superposition of different phenotypes and variability in the hereditary patterns. Clinical diagnosis is established by coupling imaging results to clinical characteristics. The clinical manifestations of non-compacted myocardiopathy are variable, including arrhythmic events and variable degrees of cardiac failure, although some patients may be asymptomatic. In certain cases a heart transplant may be necessary. The differential diagnosis should be made with hypertrophic and dilated myocardiopathy. However, only a few reports can be found in the literature that discuss the pathology of this condition.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:54 |
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| Enthalten in: |
Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de Citologia - 54(2021), 2 vom: 17. Apr., Seite 127-131 |
| Sprache: |
Spanisch |
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| Weiterer Titel: |
Corazón en «asta de ciervo». Estudio anatomopatológico del corazón explantado de una paciente con diagnóstico clínico y variantes genéticas de miocardiopatía no compactada |
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| Beteiligte Personen: |
García Pérez, Juan Luis [VerfasserIn] |
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| Links: |
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| Themen: |
Case Reports |
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| Anmerkungen: |
Date Completed 20.12.2021 Date Revised 20.12.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.patol.2019.01.005 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM322835267 |
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| 245 | 1 | 0 | |a "Staghorn" heart. The pathology of the explanted heart of a patient with clinical diagnosis and genetic variants of non-compacted myocardiopathy |
| 246 | 3 | 3 | |a Corazón en «asta de ciervo». Estudio anatomopatológico del corazón explantado de una paciente con diagnóstico clínico y variantes genéticas de miocardiopatía no compactada |
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| 520 | |a Copyright © 2019 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved. | ||
| 520 | |a Non-compacted myocardiopathy is rare, the prevalence ranging between 0.01-0.26%. in adults. We present the macroscopic, microscopic and electron microscopy findings of cardiac transplant samples from a 36-year-old patient diagnosed with non-compacted myocardiopathy. This condition shows a high genetic and phenotypic heterogeneity, with superposition of different phenotypes and variability in the hereditary patterns. Clinical diagnosis is established by coupling imaging results to clinical characteristics. The clinical manifestations of non-compacted myocardiopathy are variable, including arrhythmic events and variable degrees of cardiac failure, although some patients may be asymptomatic. In certain cases a heart transplant may be necessary. The differential diagnosis should be made with hypertrophic and dilated myocardiopathy. However, only a few reports can be found in the literature that discuss the pathology of this condition | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Fibrosis miocárdica | |
| 650 | 4 | |a Miocardio espongiforme | |
| 650 | 4 | |a Miocardio no compactado | |
| 650 | 4 | |a Miocardiopatía | |
| 650 | 4 | |a Myocardial fibrosis | |
| 650 | 4 | |a Myocardiopathy | |
| 650 | 4 | |a Non-compacted myocardium | |
| 650 | 4 | |a Spongy myocardium | |
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| 700 | 1 | |a Palomino Doza, Addison Julián |e verfasserin |4 aut | |
| 700 | 1 | |a Delgado Jiménez, Juan Francisco |e verfasserin |4 aut | |
| 700 | 1 | |a Enguita Valls, Ana Belén |e verfasserin |4 aut | |
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