Abstract
BackgroundDouble chambered right ventricle (DCRV) is a relatively rare congenital heart disease, characterized by the abnormal division of the right ventricle into a high-pressure inlet and low-pressure outlet by anomalous muscle bundles. Extra-cardiac right-to-left shunts may present with clinical symptoms in adulthood and should be sought in patients with previous cavo-pulmonary shunt procedures.Case presentationWe report a case of DCRV in a 29 year old Caucasian male presenting in adulthood with a right-to-left shunt secondary to venous collaterals, following cavopulmonary anastomosis for congenital pulmonary atresia and hypoplastic right ventricle.ConclusionMultimodality cardiac imaging using echocardiography, cardiac CT, cardiac MRI and cardiac catheterization is often required for complete characterization of complex congenital heart anomalies in adulthood.
Highlights
Double chambered right ventricle (DCRV) is a relatively rare congenital heart disease, characterized by the abnormal division of the right ventricle into a high-pressure inlet and low-pressure outlet by anomalous muscle bundles
Double chambered right ventricle (DCRV) is a congenital cardiac malformation in which the right ventricle is divided into a proximal high-pressure and distal lowpressure chamber by anomalous muscle bundles [1]
We report a case of DCRV presenting in adulthood with a right to left shunt resulting from systemic venous to pulmonary venous collaterals that developed following superior vena cava to pulmonary artery shunt surgery for pulmonary atresia and hypoplastic right ventricle (RV)
Summary
Multimodality cardiac imaging using echocardiography, cardiac CT, cardiac MRI and cardiac catheterization is often required for complete characterization of complex congenital heart anomalies in adulthood. Consent A written informed consent was obtained from the patient for publication of this case report and all accompanying images. Competing interests No competing issues to report. Authors’ contributions LK, AC, and MS contributed to the acquisition of data and interpretation of the case report. All authors read and approved the final manuscript. Author details 1Department of Radiology, University of Manitoba, Winnipeg, Manitoba, Canada.
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