Among the right ventricular conditions, Uhl's 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's 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.