The concept of double inlet-double outlet right ventricle: a distinct congenital heart disease
File(s)Final version Manuscript .pdf (184.88 KB)
Accepted version
Author(s)
Spadotto, V
Frescura, C
Ho, SY
Thiene, G
Type
Journal Article
Abstract
The aim of this study was to estimate the incidence and to analyze the anatomy of double inlet-double outlet right ventricle complex and its associated cardiac anomalies in our autopsy series. Among the 1640 hearts with congenital heart disease of our Anatomical Collection, we reviewed the specimens with double inlet-double outlet right ventricle, according to the sequential-segmental analysis, identifying associated cardiac anomalies and examining lung histology to assess the presence of pulmonary vascular disease. We identified 14 hearts with double inlet-double outlet right ventricle (0.85%). Right atrial isomerism was observed in 10 hearts, situs solitus in 3 and left atrial isomerism in one. Regarding the mode of atrioventricular connection, all hearts but one had a common atrioventricular valve. Systemic or pulmonary venous abnormalities were noted in all patients with atrial isomerism. In nine patients a valvular or subvalvular pulmonary stenosis was present. Among the functionally “univentricular hearts”, double inlet- double outlet right ventricle represents a peculiar entity, mostly in association with right atrial isomerism. Multiple cardiac anomalies are associated and may complicate surgical repair.
Date Issued
2016-09-25
Online Publication Date
2017-09-25T06:00:18Z
Date Acceptance
2016-09-20
ISSN
1879-1336
Publisher
Elsevier
Start Page
39
End Page
44
Journal / Book Title
Cardiovascular Pathology
Volume
26
Copyright Statement
© 2016, Elsevier Ltd. All rights reserved. This manuscript is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/
Source Database
pubmed
Identifier
http://www.ncbi.nlm.nih.gov/pubmed/27866077
S1054-8807(16)30132-6
Subjects
Congenital heart disease
Double inlet right ventricle
Double outlet right ventricle
Univentricular heart
Cardiovascular System & Hematology
1102 Cardiovascular Medicine And Haematology
Publication Status
Published
Country
United States