Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Oct 19;10(20):4782.
doi: 10.3390/jcm10204782.

Heart Failure in Patients with Arrhythmogenic Cardiomyopathy

Shi Chen  1 , Liang Chen  1 , Firat Duru  2   3 , Shengshou Hu  1
Affiliations
Review

Heart Failure in Patients with Arrhythmogenic Cardiomyopathy

Shi Chen et al. J Clin Med. .

Abstract

Arrhythmogenic cardiomyopathy (ACM) is a rare inherited cardiomyopathy characterized as fibro-fatty replacement, and a common cause for sudden cardiac death in young athletes. Development of heart failure (HF) has been an under-recognized complication of ACM for a long time. The current clinical management guidelines for HF in ACM progression have nowadays been updated. Thus, a comprehensive review for this great achievement in our understanding of HF in ACM is necessary. In this review, we aim to describe the research progress on epidemiology, clinical characteristics, risk stratification and therapeutics of HF in ACM.

Keywords: arrhythmogenic ventricular cardiomyopathy; heart failure; prognosis; risk stratification.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
The incidence of heart transplantation/death reported from the ACM registry in different countries worldwide.
Figure 2
Figure 2
Heart failure progression in classical ACM.
Figure 3
Figure 3
The CMR and ECG characteristics of ACM HF patients. (A) The CMR image of ACM HF patient; (B) the ECG of ACM HF patient. (Adapted with permission from Liang et al. [42]). CMR, cardiac magnetic resonance; ECG, electrocardiogram; ACM, arrhythmogenic cardiomyopathy; HF, heart failure.
Figure 4
Figure 4
The diagnostic criteria of imaging and ECG in the 2010 Revised Task Force Criteria. 2D, two dimensional; RV, right ventricular; PLAX, parasternal long-axis; RVOT, RV outflow tract; PSAX, parasternal short-axis; BSA, body surface area; CMR, cardiac MRI; EDV, end-diastolic volume; RVEF, right ventricular ejection fraction; RBBB, right bundle branch block; SAECG, signal-averaged ECG; VT, ventricular tachycardia; LBBB, left bundle branch block; PVC, premature ventricular complex.
Figure 5
Figure 5
Plasma biomarker application in ACM.
Figure 6
Figure 6
The gross morphology and histopathological characteristics of ARVC HF patients. (A) The explanted heart from ARVC patient; (B) the diagram of sampling position; (C) Masson staining of six representative sections. (Adapted with permission from Liang et al. [42]). LVAW, anterior wall of left ventricular (LV); LVLW, lateral wall of LV; LVIW, inferior wall of LV; IVS, interventricular septum; RVAW, anterior wall of right ventricle (RV); RVIW, inferior wall of RV.
Figure 7
Figure 7
Risk factors of heart failure in ACM.
Figure 8
Figure 8
Prevention and management of heart failure in ACM. ICD, implantable cardioverter-defibrillators; ACEI, angiotensin converting enzyme inhibitors; ARB, angiotensin II receptor blockers; RV, right ventricular; CRT, cardiac resynchronization therapy.
See this image and copyright information in PMC

References

    1. Basso C., Corrado D., Marcus F.I., Nava A., Thiene G. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009;373:1289–1300. doi: 10.1016/S0140-6736(09)60256-7. - DOI - PubMed
    1. Corrado D., Link M.S., Calkins H. Arrhythmogenic Right Ventricular Cardiomyopathy. N. Engl. J. Med. 2017;376:61–72. doi: 10.1056/NEJMra1509267. - DOI - PubMed
    1. Hoorntje E.T., Te Rijdt W.P.T., James C.A., Pilichou K., Basso C., Judge D.P., Bezzina C.R., Van Tintelen J.P. Arrhythmogenic cardiomyopathy: Pathology, genetics, and concepts in pathogenesis. Cardiovasc. Res. 2017;113:1521–1531. doi: 10.1093/cvr/cvx150. - DOI - PubMed
    1. Bosman L.P., Sammani A., James C.A., Cadrin-Tourigny J., Calkins H., van Tintelen J.P., Hauer R.N.W., Asselbergs F.W., Te Riele A. Predicting arrhythmic risk in arrhythmogenic right ventricular cardiomyopathy: A systematic review and meta-analysis. Heart Rhythm. 2018;15:1097–1107. doi: 10.1016/j.hrthm.2018.01.031. - DOI - PubMed
    1. Cadrin-Tourigny J., Bosman L.P., Nozza A., Wang W., Tadros R., Bhonsale A., Bourfiss M., Fortier A., Lie O.H., Saguner A.M., et al. A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. Eur. Heart J. 2019;40:1850–1858. doi: 10.1093/eurheartj/ehz103. - DOI - PMC - PubMed