scholarly journals Right Ventricular Sarcoidosis: Is It Time for Updated Diagnostic Criteria?

2014 ◽  
Vol 41 (2) ◽  
pp. 203-207 ◽  
Author(s):  
Kairav Vakil ◽  
Elina Minami ◽  
Daniel P. Fishbein
Keyword(s):  
Right Ventricular ◽  
Diagnostic Criteria ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Care Center ◽  
Tertiary Care ◽  
Right Ventricular Failure ◽  
Ventricular Cardiomyopathy ◽  
Ventricular Failure ◽  
Diagnostic Challenge ◽  
End Stage

A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient's clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient's right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. In addition to discussing the patient's case, we review the relevant medical literature and discuss the need for updated differential diagnostic criteria for end-stage right ventricular failure that mimics arrhythmogenic right ventricular cardiomyopathy.

scholarly journals Profile of arrythmogenic right ventricular cardiomyopathy/dysplasia (ARCV/D) patients presenting with sustained ventricular tachycardia in a tertiary care center

2018 ◽  
Vol 6 (4) ◽  
pp. 1211
Author(s):  
Rahul Sudan ◽  
Irfan Yaqoob ◽  
Khursheed Aslam ◽  
Mehroz Ahmad
Keyword(s):  
Ventricular Tachycardia ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Care Center ◽  
Tertiary Care ◽  
Sustained Ventricular Tachycardia ◽  
Clinical Profile ◽  
Haemodynamic Instability ◽  
Ventricular Cardiomyopathy

Background: Arrhythmogenic right ventricular cardiomyopathy /dysplasia (ARVC/D) is a genetic form of cardiomyopathy and is one among the most common causes of sudden cardiac death (SCD). The aim of our study was to analyze the clinical profile of (ARVC/D) patients presenting with sustained Ventricular Tachycardia (VT).Methods: This single center cohort study evaluated 107 patients who presented with sustained ventricular tachycardia (VT) in our hospital. After aetiological evaluation of all these patients, 15 patients were found to have ARVC/D as the cause of sustained ventricular tachycardia (VT) as per the Modified Task Force Criteria. The clinical profile of these patients was observed in detail to enhance our knowledge about this entity in our part of the world.Results: Mean age at presentation was 30 years and 12 patients were males. Nine patients were haemodynamically stable at the time of sustained VT and the rest of patients were haemodynamically unstable. Left Bundle Branch Block (LBBB) was the most common ECG morphology present in 11 patients. Antiarrhythmic drugs terminated VT in 7 patients. All the 6 patients presenting in a state of haemodynamic instability received DC cardioversion. Mortality occurred in 2 patients during the hospital stay.Conclusions: ARVC/D presenting with sustained VT is an important manifestation of the disease. Males are more commonly affected than females. Haemodynamic instability at the time of presentation carries a poor prognosis.

scholarly journals Arrhythmogenic right ventricular cardiomyopathy/dysplasia on the basis of the revised diagnostic criteria in affected families with desmosomal mutations

2011 ◽  
Vol 32 (9) ◽  
pp. 1097-1104 ◽  
Author(s):  
Nikos Protonotarios ◽  
Aris Anastasakis ◽  
Loizos Antoniades ◽  
Gregory Chlouverakis ◽  
Petros Syrris ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Diagnostic Criteria ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy
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