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
Case Reports
. 2024 May 30;24(1):284.
doi: 10.1186/s12872-024-03959-z.

Arrhythmogenic right ventricular cardiomyopathy with sustained ventricular tachycardia: a case report

Ying Ban  1 , Feng-Juan Yao  1 , Wei Li  2
Affiliations
Case Reports

Arrhythmogenic right ventricular cardiomyopathy with sustained ventricular tachycardia: a case report

Ying Ban et al. BMC Cardiovasc Disord. .

Abstract

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an infrequent hereditary disorder distinguished by fibrofatty replacement of the myocardium in the right ventricular, which predisposes individuals to life-threatening arrhythmias. This case delineates an ARVC patient who suffered recurrent bouts of sustained ventricular tachycardia (VT). In this case, we mainly discuss the application of myocardial contrast echocardiography (MCE) in displaying myocardial fibrosis in patients with ARVC.

Case presentation: A 43-year-old male experienced three episodes of unexplained VT over an eight-year period, accompanied by symptoms of chest discomfort, palpitations and dizziness. Coronary angiography revealed no significant coronary stenosis. The electrocardiogram (ECG) results indicated characteristic epsilon waves in right precordial leads, and subsequent echocardiography identified right ventricular enlargement and right ventricular systolic dysfunction. MCE further disclosed regional myocardial ischemia at the epicardium of the left ventricular apex. Ultimately, cardiovascular magnetic resonance imaging (CMR) corroborated the ARVC diagnosis, highlighting linear intensification in the right ventricle during the delayed enhancement.

Conclusion: Prompt identification of ARVC is crucial for timely intervention and management. MCE may offer an effective and valuable technique for the detection of myocardial involvement in ARVC patient.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Myocardial contrast echocardiography; Myocardial fibrosis.

PubMed Disclaimer

Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
The ECG demonstrated epsilon waves in V1-V3 leads
Fig. 2
Fig. 2
A, Echocardiography showed right ventricular enlargement with thinning of the ventricular walls and reduced motion of the right ventricular wall; B, Echocardiography showed a flake isoechoic lesion in the apex of the LV; C, LVO reveals no apparent filling defect at the apex and reduced motion in the anterior, lateral, inferior segments of the left ventricular apex; D, MCE patten shows markedly lower perfusion levels of the subepicardial myocardium at the left ventricular apex compared to the endocardial enhancement at the same level
Fig. 3
Fig. 3
Delayed enhancement in CMR demonstrates subendocardial diffuse patchy linear enhanced lesions in the left ventricular apex-middle free wall
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart. 2000;83(5):588–95. doi: 10.1136/heart.83.5.588. - DOI - PMC - PubMed
    1. Corrado D, Thiene G. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: clinical impact of molecular genetic studies. Circulation. 2006;113(13):1634–7. doi: 10.1161/CIRCULATIONAHA.105.616490. - DOI - PubMed
    1. Mestroni L, Brun F, Spezzacatene A, Sinagra G, Taylor MRG. Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). In: The Right Ventricle in Health and Disease edn.; 2015: 337–360.
    1. McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, Camerini F. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Br Heart J. 1994;71(3):215–8. doi: 10.1136/hrt.71.3.215. - DOI - PMC - PubMed
    1. Corrado D, van Tintelen PJ, McKenna WJ, Hauer RNW, Anastastakis A, Asimaki A, Basso C, Bauce B, Brunckhorst C, Bucciarelli-Ducci C, et al. Arrhythmogenic right ventricular cardiomyopathy: evaluation of the current diagnostic criteria and differential diagnosis. Eur Heart J. 2020;41(14):1414–29. doi: 10.1093/eurheartj/ehz669. - DOI - PMC - PubMed

Publication types

MeSH terms

-