Dataset Information

Echocardiographic Hypertrabeculated/Non-compacted Right Ventricle Accompanied by Atrial Septal Defect and Anomalous Pulmonary Vein Connection.

ABSTRACT: Myocardial noncompaction (NC) is a disorder of the embryonic endomyocardial morphogenesis frequently associated with congenital cardiac abnormalities. NC predominantly affects the left ventricle (LV). Right ventricle (RV) NC may occur in association with LV involvement or in isolation. A 47-year-old woman was admitted for atrial septal defect closure. Transthoracic echocardiography revealed hypertrabeculation of the RV apex, consisting of multiple deep recesses with the entrance of blood flow in color Doppler imaging, suggestive of isolated RV hypertrabeculation/NC. The RV and right atrium (RA) were enlarged, and systolic pulmonary arterial pressure was slightly increased. Our patient's associated abnormalities were atrial septal defect (superior sinus venosus type), anomalous connection of the right upper pulmonary vein to the junction of the superior vena cava and the RA, and large patent foramen ovale. Association between atrial septal defect and partial anomalous pulmonary vein connection and isolated hypertrabeculated/noncompacted RV should be considered by cardiologists.

SUBMITTER: Hosseinsabet A

PROVIDER: S-EPMC5353564 | biostudies-literature | 2014 Oct-Dec

REPOSITORIES: biostudies-literature

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Echocardiographic Hypertrabeculated/Non-compacted Right Ventricle Accompanied by Atrial Septal Defect and Anomalous Pulmonary Vein Connection.

Hosseinsabet Ali A

Journal of cardiovascular echography 20141001 4

Myocardial noncompaction (NC) is a disorder of the embryonic endomyocardial morphogenesis frequently associated with congenital cardiac abnormalities. NC predominantly affects the left ventricle (LV). Right ventricle (RV) NC may occur in association with LV involvement or in isolation. A 47-year-old woman was admitted for atrial septal defect closure. Transthoracic echocardiography revealed hypertrabeculation of the RV apex, consisting of multiple deep recesses with the entrance of blood flow in ...[more]

PMID: 28465919

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Project description:BackgroundUniatrial biventricular connection (UBC) is a rare cardiovascular anomaly characterized by absence of one atrioventricular connection and drainage of the other atrium via a solitary atrioventricular valve into both ventricles. The absent atrioventricular connection may affect either the left or right atrium. Because of the absence of one atrioventricular connection hearts with UBC have been classified among functionally univentricular hearts requiring palliative treatment according to the Fontan principle.Aims/objectiveWe report two further patients with UBC. In one of these patients careful echocardiographic examination of the atrioventricular junction in early infancy revealed the possibility of biventricular repair based on the favorable anatomy of the atrioventricular valve and balanced ventricles in the presence of an inlet ventricular septal defect (VSD).Case presentationsBoth patients presented in the neonatal period for evaluation of complex congenital heart disease. The anatomy of the atrioventricular valves in our patients was indistinguishable from atrioventricular septal defects exhibiting the morphology of a common valve with superior and inferior bridging leaflets. The common atrioventricular valve was connected exclusively to the right atrium draining into both ventricles while the left atrium drained into the right atrium via a secundum atrial septal defect. In one of our patients biventricular repair with good longterm-result was performed by reseptation of the atria, patch repair of the VSD and septation of the atrioventricular valve. The second patient underwent univentricular palliation according to the Fontan principle.Discussion/conclusionThe echocardiographic findings in our patients suggest that at least some patients with UBC represent a variant of atrioventricular septal defects associated with extreme ventriculoatrial malalignment resulting in fusion of the deviated primary atrial septum with the lateral aspect of the atrioventricular junction. This offers the option of septation of the common atrioventricular valve and biventricular repair in patients with adequate size of both ventricles. Exact echocardiographic analysis of the morphology of the atrioventricular valve is essential to distinguish these patients with a morphologically common atrioventricular valve in early infancy from other variants of absent atrioventricular connection and to select those who are suitable for biventricular repair.

Dataset Information

Echocardiographic Hypertrabeculated/Non-compacted Right Ventricle Accompanied by Atrial Septal Defect and Anomalous Pulmonary Vein Connection.

Publications

Echocardiographic Hypertrabeculated/Non-compacted Right Ventricle Accompanied by Atrial Septal Defect and Anomalous Pulmonary Vein Connection.

Similar Datasets

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