Desmoplakin Cardiomyopathy: Comprehensive Review of an Increasingly Recognized Entity
Abstract
:1. Background
2. Clinical Features
2.1. Performance of ACM Diagnostic Criteria in DSP Cardiomyopathy
2.2. Myocarditis-like Episodes
3. Electrocardiographic Features
Ventricular Arrhythmias
4. Cardiovascular Imaging Features
5. Therapy, Risk Stratification, and Prognosis
6. Carrier Status and the Role of Exercise in DSP Cardiomyopathy
7. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Author/Year | Number of Patients | Age at Diagnosis (Years) | M:F (%) | Index Case | Cutaneous Phenotype • | LV+ Involvement | RV+ Involvement | Normal Ventricular Function | Myocardial Injury * | Frequent PVCs |
---|---|---|---|---|---|---|---|---|---|---|
Smith et al., 2020 [17] | 107 | 36 ± 16 † | 31:69 | 41% | 55% | 51% | 14% | 36% | 14% | 56% |
Wang et al., 2022 [19] | 91 | 28 (20–44) ‡ | 33:66 | 49% | - | 28% | 6% | 51% | 22% | 48% |
Bariani et al., 2022 [18] | 73 | 33 (19–50) ‡ | 47:53 | 36% | 44% | 36% | 22% | 15% | 15% | 43% |
Reza et al., 2022 [22] | 19 | - | 37:63 | 58% | - | 37% | 11% | - | - | - |
Di Lorenzo et al., 2023 [23] | 18 | 41 (31–47) ‡ | 50:50 | 100% | - | 55% | 22% | 56% | 39% | 72% |
Instrumental Features | Smith et al., 2020 [17] n = 107 | Wang et al., 2022 [19] n = 91 | Bariani et al., 2022 [18] n = 73 | Reza et al., 2022 [22] n = 19 | Di Lorenzo et al., 2023 [23] n = 18 |
---|---|---|---|---|---|
Frequent PVCs | 56% | 48% | 43% | - | 72% |
TWI V1-V3 | 8% | 20% | 21% | 16% | - |
TWI V4-V6 | 21% | 25% | 22% | 37% | 11% |
Low QRS voltage | - | 15% | 62% | - | 33% |
LVEF, % | 46 ± 14 † | 49 ± 13 † | 52 (44–63) ‡ | - | 49 (42–56) ‡ |
LV LGE | 40% | 49% | 72% | 92% | 100% |
RVEF, % | - | 49 ± 12 † | 55 (47–63) ‡ | - | 53 (44–57) ‡ |
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Brandão, M.; Bariani, R.; Rigato, I.; Bauce, B. Desmoplakin Cardiomyopathy: Comprehensive Review of an Increasingly Recognized Entity. J. Clin. Med. 2023, 12, 2660. https://doi.org/10.3390/jcm12072660
Brandão M, Bariani R, Rigato I, Bauce B. Desmoplakin Cardiomyopathy: Comprehensive Review of an Increasingly Recognized Entity. Journal of Clinical Medicine. 2023; 12(7):2660. https://doi.org/10.3390/jcm12072660
Chicago/Turabian StyleBrandão, Mariana, Riccardo Bariani, Ilaria Rigato, and Barbara Bauce. 2023. "Desmoplakin Cardiomyopathy: Comprehensive Review of an Increasingly Recognized Entity" Journal of Clinical Medicine 12, no. 7: 2660. https://doi.org/10.3390/jcm12072660