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Double-Outlet Right Ventricle Without Interventricular Communication: An Unusual and Challenging Problem

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Abstract

A ventriculoseptal defect (VSD) usually is present in patients with a double-outlet right ventricle. This report describes a case of double-outlet right ventricle with a completely shrouded inlet VSD and no interventricular shunting. This was associated with a severely regurgitant dysplastic mitral valve. The anatomy and clinical management of the patient is discussed together with an overview of the existing literature.

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Author information

Correspondence to Luc Mertens.

Electronic supplementary material

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Supplementary movie S1 Color subcostal long-axis sweep demonstrating the origin of the aorta and the pulmonary artery from the right ventricle and the lack of any obvious interventricular communication. Also, the arrangement of the outflow tracts is shown. (AVI 33992 kb)

Supplementary movie S1 Color subcostal long-axis sweep demonstrating the origin of the aorta and the pulmonary artery from the right ventricle and the lack of any obvious interventricular communication. Also, the arrangement of the outflow tracts is shown. (AVI 33992 kb)

Supplementary movie S2 Color apical four-chamber view showing a thickened and dysplastic mitral valve with severe regurgitation and no ventriculoseptal defect (VSD). (AVI 3344 kb)

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Singla, M., Al-Radi, O. & Mertens, L. Double-Outlet Right Ventricle Without Interventricular Communication: An Unusual and Challenging Problem. Pediatr Cardiol 34, 1941–1944 (2013) doi:10.1007/s00246-012-0483-5

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Keywords

  • Congenital heart disease
  • Double-outlet right ventricle
  • Rare defects