Best management in isolated right ventricular hypoplasia with septal defects in adults

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 sh...

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Bibliographic Details
Main Authors: Zahra Khajali, Maedeh Arabian, Maryam Aliramezany
Format: Article
Language:English
Published: Tabriz University of Medical Sciences 2020-09-01
Series:Journal of Cardiovascular and Thoracic Research
Subjects:
Online Access:https://jcvtr.tbzmed.ac.ir/PDF/jcvtr-12-237.pdf
Description
Summary: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.
ISSN:2008-5117
2008-6830