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Double Outlet Right Ventricle

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Abstract

Double outlet right ventricle represents a heterogeneous group of cardiac malformations sharing in common an abnormal ventriculoarterial connection usually associated with a VSD. The location of the VSD and the position of the great arteries may vary considerably. 2D echocardiography in the precordial and apical views displays the location and size of the VSD and its relation to the great arteries. The subaortic and subpulmonary outflow tracts can be well displayed from the subcostal window. Colour Doppler echocardiography allows definition of shunting across the VSD, facilitates detection of possible obstructions of the subpulmonary and subaortic outflow and improves examination of the aortic arch for possible obstruction. PW and CW Doppler are required for quantification of possible obstruction of the subpulmonary outflow tract. Evaluation of the subaortic outflow tract in the neonate has to include careful assessment by 2D echo, since severe obstruction may be present despite the absence of a significant Doppler gradient.

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14.1 Electronic Supplementary Material

Video 14.1

Parasternal long-axis sweep in a patient with DORV and subaortic VSD. The parasternal long-axis view of the left ventricle shows the large VSD in close proximity to the aorta. There is absence of fibrous continuity between the anterior leaflet of the mitral valve and the aorta. Lateral tilt of the transducer towards the parasternal long-axis view of the right ventricular outflow tract reveals a large main pulmonary artery (AVI 57614 kb)

Video 14.2

Parasternal short-axis sweep in a patient with DORV and subaortic VSD (same patient as in Video 14.1) starting at the level of the mitral valve. The sweep shows the large VSD which is in close proximity to the tricuspid valve. The subaortic and subpulmonary outflow are separated by the infundibular (conal) septum (AVI 53060 kb)

Video 14.3

The parasternal short-axis view at the level of the outflow tracts shows separation of the subaortic and subpulmonary outflow by the infundibular (conal) septum (AVI 14835 kb)

Video 14.4

The parasternal short-axis view in this infant with DORV and subaortic VSD shows a large right coronary artery giving rise to a large branch oriented anteriorly towards the ventricular septum representing a left anterior descending coronary branch (AVI 8569 kb)

Video 14.5

Colour Doppler in the parasternal short-axis view (same patient as in Video 14.4) confirms flow in the large right coronary artery, which gives rise to a branch that crosses the right ventricular outflow tract (AVI 3811 kb)

Video 14.6

The subcostal coronal view in a newborn with DORV and subaortic VSD shows marked dextroposition of the aorta, which originates exclusively from the right ventricle (RV) (same patient as in Videos 14.7 and 14.8) (AVI 9005 kb)

Video 14.7

The subcostal coronal view in a more ventral plane (same patient as in Videos 14.6 and 14.8) reveals severe subpulmonary obstruction due to deviation of the infundibular septum. Note the large VSD is not displayed in this plane (AVI 8304 kb)

Video 14.8

Colour Doppler in the subcostal coronal view reveals acceleration of flow and turbulence in the subpulmonary outflow confirming severe subvalvular pulmonary obstruction (same patient as in Videos 14.6 and 14.7) (AVI 3493 kb)

Video 14.9

The subcostal RAO view in an infant with DORV and subaortic VSD shows subpulmonary obstruction due to anterior deviation of the infundibular septum (same patient as in Video 14.10) (AVI 12755 kb)

Video 14.10

Subpulmonary acceleration of flow is apparent on colour Doppler examination in the RAO view (same patient as in Video 14.10) (AVI 3969 kb)

Video 14.11

In an infant with DORV, the subcostal coronal view shows a restrictive subaortic VSD (AVI 23112 kb)

Video 14.12

Restriction of the VSD is confirmed by colour Doppler (same patient as in Video 14.11) in the subcostal coronal view (AVI 4748 kb)318084_1_En_14_MOESM13_ESM.mp4 (AVI 4748 kb)

Video 14.13

The parasternal long-axis view in a patient with DORV and subpulmonary VSD shows the VSD in close proximity to the overriding of the pulmonary artery. The anterior aorta takes a parallel course to the pulmonary artery (AVI 23661 kb)

Video 14.14

This sweep in the parasternal short-axis view of a patient with DORV and subpulmonary VSD starts at the level of the ventricles. Cranial tilt of the transducer reveals a large subpulmonary VSD. The anterior aorta originates exclusively and the posterior pulmonary artery originates predominantly from the right ventricle (AVI 25762 kb)

Video 14.15

The subcostal short-axis view in this newborn with DORV and subpulmonary VSD shows anterior origin of the aorta from the right ventricle. The posterior pulmonary artery overrides the ventricular septum (AVI 9476 kb)

Video 14.16

Colour Doppler in the subcostal short-axis view (same patient as in Video 14.15) confirms the parallel course of both great arteries (AVI 5015 kb)

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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Double Outlet Right Ventricle. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_14

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  • DOI: https://doi.org/10.1007/978-3-319-42919-9_14

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-42917-5

  • Online ISBN: 978-3-319-42919-9

  • eBook Packages: MedicineMedicine (R0)

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