Indian Journal of Medical Research

REVIEW ARTICLE
Year
: 2010  |  Volume : 131  |  Issue : 1  |  Page : 35--45

Comparison of Uhl俟Q製 anomaly, right ventricular outflow tract ventricular tachycardia (RVOT VT) & arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with an insight into genetics of ARVD/C


Pranathi R Pamuru, Maithili V Dokuparthi, S Remersu, N Calambur, P Nallari 
 Department of Genetics, Osmania University, and Cardiology Care Hospital, The Institute of Medical Sciences, Hyderabad, India

Correspondence Address:
Pranathi R Pamuru
Department of Genetics, Osmania University, and Cardiology Care Hospital, The Institute of Medical Sciences, Hyderabad, India

Among the right ventricular conditions, Uhl俟Q製 anomaly, arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD/C) and right ventricular outflow tract ventricular tachycardia (RVOT VT) are disorders that exhibit pathogenic changes involving the right ventricular (RV) myocardium, and are expected to be severe or milder forms of the same condition. The review focuses on the aspect whether the three RV disorders are a spectrum of the same disease. ARVD/C is the only condition among these to be genetically well characterized. Also, variations in the clinical expression of ARVD/C due to the genetic heterogeneity are examined. Based on clinical manifestations, age at onset, gender ratio and the possible molecular mechanisms implicated, Uhl俟Q製 anomaly, ARVD/C and RVOT VT may be considered as separate entities. Further, to differentiate between the three RV disorders, the molecular studies on ARVD/C might be helpful. An attempt was made to differentiate between the eleven different types of ARVD/Cs based on clinical symptoms presented including the progression of the disease to the left ventricle, ventricular arrhythmias and clinical characteristics like ECG, SAECG, ECHO and histopathological studies.


How to cite this article:
Pamuru PR, Dokuparthi MV, Remersu S, Calambur N, Nallari P. Comparison of Uhl's anomaly, right ventricular outflow tract ventricular tachycardia (RVOT VT) & arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with an insight into genetics of ARVD/C.Indian J Med Res 2010;131:35-45


How to cite this URL:
Pamuru PR, Dokuparthi MV, Remersu S, Calambur N, Nallari P. Comparison of Uhl's anomaly, right ventricular outflow tract ventricular tachycardia (RVOT VT) & arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with an insight into genetics of ARVD/C. Indian J Med Res [serial online] 2010 [cited 2020 Nov 24 ];131:35-45
Available from: https://www.ijmr.org.in/article.asp?issn=0971-5916;year=2010;volume=131;issue=1;spage=35;epage=45;aulast=Pamuru;type=0