Submitted: 03 Mar 2020
Revision: 12 Jun 2020
Accepted: 03 Aug 2020
ePublished: 27 Aug 2020
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J Cardiovasc Thorac Res. 2020;12(3): 237-243.
doi: 10.34172/jcvtr.2020.36
  Abstract View: 84
  PDF Download: 88

Case Series

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

Zahra Khajali 1, Maedeh Arabian 1, Maryam Aliramezany 2* ORCID logo

1 Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran
2 Cardiovascular Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran
*Corresponding Author: Maryam Aliramezany, Email: maliramezany@yahoo.com


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.
Keywords: Right Ventricular Hypoplasia, Adult Congenital Heart Disease, Cyanosis, Atrial Septal Defect
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