Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management
Our understanding of arrhythmogenic right ventricular cardiomyopathy (ARVC) has advanced considerably over the past 30- 40 years. This is an inherited cardiomyopathy with complicated genetic inheritance and variable penetrance. Desmosomal dysfunction underlies most cases, and appreciating this patho...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2017-12-01
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| Series: | Cardiogenetics |
| Subjects: | |
| Online Access: | http://www.pagepressjournals.org/index.php/cardiogen/article/view/6995 |
| _version_ | 1819074493618847744 |
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| author | Rachel Bastiaenen Marc W. Deyell Andrew D. Krahn |
| author_facet | Rachel Bastiaenen Marc W. Deyell Andrew D. Krahn |
| author_sort | Rachel Bastiaenen |
| collection | DOAJ |
| description | Our understanding of arrhythmogenic right ventricular cardiomyopathy (ARVC) has advanced considerably over the past 30- 40 years. This is an inherited cardiomyopathy with complicated genetic inheritance and variable penetrance. Desmosomal dysfunction underlies most cases, and appreciating this pathophysiology has contributed to patient management, particularly with respect to exercise restriction to reduce disease progression. The diagnosis is made according to a series of Task Force Criteria, and subsequent management is guided by expert consensus in the absence of comparative data. ARVC is associated with sudden cardiac death (SCD), particularly in young athletic individuals who unknowingly harbour the condition. Risk stratification is important to guide implantable cardioverter- defibrillator use and reduce SCD. Residual gaps in our understanding, particularly surrounding incomplete penetrance, the underlying pathophysiology and risk stratification, are being targeted by collaborative efforts, large registries, prospective studies and translational research. |
| first_indexed | 2024-12-21T18:10:24Z |
| format | Article |
| id | doaj.art-34355c3d15c742d8ae22a8dd5b0837bf |
| institution | Directory Open Access Journal |
| issn | 2035-8253 2035-8148 |
| language | English |
| last_indexed | 2024-12-21T18:10:24Z |
| publishDate | 2017-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cardiogenetics |
| spelling | doaj.art-34355c3d15c742d8ae22a8dd5b0837bf2022-12-21T18:54:48ZengMDPI AGCardiogenetics2035-82532035-81482017-12-017110.4081/cardiogenetics.2017.69955509Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in managementRachel Bastiaenen0Marc W. Deyell1Andrew D. Krahn2Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, BCHeart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, BCHeart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, BCOur understanding of arrhythmogenic right ventricular cardiomyopathy (ARVC) has advanced considerably over the past 30- 40 years. This is an inherited cardiomyopathy with complicated genetic inheritance and variable penetrance. Desmosomal dysfunction underlies most cases, and appreciating this pathophysiology has contributed to patient management, particularly with respect to exercise restriction to reduce disease progression. The diagnosis is made according to a series of Task Force Criteria, and subsequent management is guided by expert consensus in the absence of comparative data. ARVC is associated with sudden cardiac death (SCD), particularly in young athletic individuals who unknowingly harbour the condition. Risk stratification is important to guide implantable cardioverter- defibrillator use and reduce SCD. Residual gaps in our understanding, particularly surrounding incomplete penetrance, the underlying pathophysiology and risk stratification, are being targeted by collaborative efforts, large registries, prospective studies and translational research.http://www.pagepressjournals.org/index.php/cardiogen/article/view/6995Arrhythmogenic right ventricular cardiomyopathysudden cardiac deathventricular arrhythmias. |
| spellingShingle | Rachel Bastiaenen Marc W. Deyell Andrew D. Krahn Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management Cardiogenetics Arrhythmogenic right ventricular cardiomyopathy sudden cardiac death ventricular arrhythmias. |
| title | Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management |
| title_full | Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management |
| title_fullStr | Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management |
| title_full_unstemmed | Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management |
| title_short | Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management |
| title_sort | arrhythmogenic right ventricular cardiomyopathy from pathophysiology to diagnosis and advances in management |
| topic | Arrhythmogenic right ventricular cardiomyopathy sudden cardiac death ventricular arrhythmias. |
| url | http://www.pagepressjournals.org/index.php/cardiogen/article/view/6995 |
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