Abstract
Functionally univentricular hearts represent a heterogeneous group of complex congenital cardiac malformations, which are not amenable to biventricular surgical repair. Echocardiographic evaluation in these patients follows the segmental approach with determination of the visceral and atrial situs and position of the heart followed by clarification of the atrioventricular connection. Further steps are description of the ventricular morphology, the atrioventricular connection and the great arteries. In the neonate these details can be clarified by 2D echocardiography from the precordial and subcostal windows. Colour Doppler is extremely valuable in the clarification of the venous connections, especially in the presence of total anomalous pulmonary venous connection. Also it plays an important role in the evaluation of stenoses and regurgitation of the AV valves, the outflow tracts, the aortic arch and the pulmonary arteries. PW and CW Doppler are required for quantification of gradients across the AV valves and the outflow tracts.
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16.1 Electronic Supplementary Material
Video 16.1
The cross section of the upper abdomen in an adolescent with situs solitus shows the aorta to the left and the inferior vena cava to the right of the spine. The position of the inferior vena cava is slightly anterior as compared to the aorta (AVI 9677 kb)
Video 16.2
In a patient with left isomerism and azygos continuation of the inferior vena cava, colour Doppler in the cross section of the upper abdomen displays the azygos vein to the right of the spine but posterior as compared to the aorta (AVI 6900 kb)
Video 16.3
This clip shows a sweep in the longitudinal axis of the upper abdomen (same patient as in Video 16.2). It starts on the right depicting the hepatic veins draining directly into the right-sided atrium. The azygos vein is visualized far posterior to the hepatic veins. Leftward sweep of the transducer displays the abdominal aorta (AVI 13124 kb)
Video 16.4
The cross section of the upper abdomen in a child with right isomerism displays both aorta and inferior vena cava to the right of the spine (AVI 5611 kb)
Video 16.5
Colour Doppler in the subcostal sagittal view of an infant with left isomerism shows azygos continuation of the inferior cava to the superior vena cava. The hepatic veins connect in a more anterior plane to the right-sided atrium (AVI 4869 kb)
Video 16.6
Colour Doppler in the subcostal coronal view of a patient with right isomerism, univentricular heart and dextrocardia shows two superior caval veins draining separately into the right-sided and left-sided atrium (AVI 3996 kb)
Video 16.7
Colour Doppler in a more anterior plane of the subcostal coronal view (same patient as in Video 16.6) shows unobstructed origin of the aorta from the univentricular heart. Severe obstruction of the subpulmonary outflow is characterized by acceleration and turbulence on colour Doppler (AVI 2989 kb)
Video 16.8
The apical four-chamber view in a newborn shows double-inlet left ventricle with both atria draining via two normal-size atrioventricular valves into a morphologic left ventricle. In addition there is some straddling an overriding of the tricuspid valve in the presence of a rudimentary right ventricle (AVI 5640 kb)
Video 16.9
In this newborn with tricuspid atresia, the apical four-chamber view shows absent connection of the right atrium to the right ventricle. The hypoplastic right ventricle is connected to the left ventricle by a ventricular septal defect (AVI 12213 kb)
Video 16.10
Colour Doppler in the apical four-chamber view (same patient as in Video 16.9) shows increased flow across the mitral valve (AVI 2341 kb)
Video 16.11
Severe hypoplasia of the left ventricle is apparent in the apical four-chamber view of this newborn with mitral valve atresia. Note also dysplasia of the thickened tricuspid valve (AVI 14158 kb)
Video 16.12
The apical four-chamber view in patient with right isomerism shows both atria connected to a univentricular heart by a common atrioventricular valve (AVI 8363 kb)
Video 16.13
The apical four-chamber view in this newborn shows mitral stenosis and severe hypoplasia of the left ventricle (AVI 19696 kb)
Video 16.14
Colour Doppler in the four-chamber view (same patient as in Video 16.13) shows LR-shunting across the atrial septum. Acceleration of flow across the atrial septum indicates severe restriction of the foramen ovale (AVI 6936 kb)
Video 16.15
In this newborn with tricuspid atresia, colour Doppler in the subcostal short-axis view reveals RL-shunting across the patent foramen ovale. Note there is also a small amount of intermediate LR-shunting (AVI 2047 kb)
Video 16.16
The parasternal long-axis view in a newborn with double-inlet left ventricle shows anterior origin of the aorta from a rudimentary right ventricular outflow chamber, while the pulmonary artery originates posterior from the left ventricle. The pulmonary valve is thickened; in addition there is subvalvular pulmonary stenosis (AVI 49529 kb)
Video 16.17
Colour Doppler in the parasternal long-axis view (same patient as in Video 16.16) shows laminar flow across the subaortic outlet foramen, while there are acceleration and turbulent flow across the subpulmonary outflow (AVI 5002 kb)
Video 16.18
The subcostal coronal view (same patient as in Videos 16.16 and 16.17) displays anterior origin of the aorta from the rudimentary right ventricular outflow chamber (AVI 42047 kb)
Video 16.19
Colour Doppler in the subcostal coronal view (same patient as in Videos 16.16, 16.17 and 16.18) confirms nonrestrictive flow from the left ventricle (LV) across the outlet foramen (arrow) to the aorta (AVI 11677 kb)
Video 16.20
Colour Doppler in the parasternal short-axis view in an infant with univentricular heart displays the aorta in cross section. The pulmonary artery can be differentiated based on the pulmonary bifurcation. Acceleration of flow and turbulence reveal significant obstruction of the pulmonary outlet (AVI 5730 kb)
Video 16.21
The subcostal short-axis view in a newborn with univentricular heart shows significant discrepancy in size between the anterior aorta and the posterior pulmonary artery (AVI 6444 kb)
Video 16.22
The discrepancy between the anterior aorta and posterior pulmonary artery (same patient as in Video 16.21) is also evident in the high parasternal long-axis view (AVI 8060 kb)
Video 16.23
In this newborn with double-inlet left ventricle, colour Doppler in the subcostal coronal view shows unobstructed flow to the anterior aorta and severely obstructed flow to the posterior pulmonary artery (AVI 9464 kb)
Video 16.24
Colour Doppler in the suprasternal long-axis view in this newborn with double-inlet left ventricle (same patient as in Videos 16.21 and 16.22) reveals severe hypoplasia of the aortic arch distal to the left common carotid artery (AVI 2737 kb)
Video 16.25
Tilting of the transducer from the suprasternal long-axis view towards the left shoulder (same patient as in Videos 16.21, 16.22 and 16.24) shows the ductus arteriosus between the large pulmonary artery and the descending aorta. The ductal view at the end of the clip confirms severe obstruction of the aortic isthmus (AVI 19806 kb)
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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Functionally Univentricular Heart. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_16
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DOI: https://doi.org/10.1007/978-3-319-42919-9_16
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