Cardiology

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




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

J Cardiovasc Thorac Res. 2020;12(3):237-243

Authors: Khajali Z, Arabian M, Aliramezany M

Abstract
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.

PMID: 33123333 [PubMed]

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