Tabriz University of Medical Sciences Journal of Cardiovascular and Thoracic Research 2008-5117 12 3 2020 09 05 Best management in isolated right ventricular hypoplasia with septal defects in adults 237 243 10.34172/jcvtr.2020.36 EN Zahra Khajali Maedeh Arabian Maryam Aliramezany https://orcid.org/0000-0002-3004-2071 Journal Article 10.34172/jcvtr.2020.36 2020 03 03 2020 08 03 Hypoplastic right ventricle is a rare congenital disease usually associated with pulmonary atresia or tricuspid atresia. Isolated right ventricular hypoplasia is a rare anomaly without important valvular abnormalities. It is associated with inter atrial septal defects leading to the right-to-left shunting of blood. Patients with isolated right ventricular hypoplasia usually have different and variable courses. In some patients, it is recognized in the perinatal period and necessitates prompt intervention; nonetheless, there are some reports of this anomaly in old age with no significant symptoms. In this report, we describe the clinical data and management of 6 adult cases with isolated right ventricular hypoplasia treated medically or surgically based on the severity of the disease and symptoms and then offer an in-depth discussion regarding this rare anomaly.