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Late diagnosis of total anomalous pulmonary venous drainage in a 5.5-month-old infant

Wojciech Mądry
Wojciech Mądry
, 
Maciej A. Karolczak
Maciej A. Karolczak
, 
Weronika Rygier
Weronika Rygier
, 
Radosław Kunikowski
Radosław Kunikowski
, 
Michał Buczyński
Michał Buczyński
 and 
Danuta Roik
Danuta Roik
   | Apr 30, 2019
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Article Category: case-report
Published Online: Apr 30, 2019
Page range: 75 - 79
Received: Nov 04, 2018
Accepted: Jan 22, 2019
DOI: https://doi.org/10.15557/jou.2019.0012
Keywords
© 2019 Wojciech Mądry et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Fig. 1.

Echocardiographic view of upper mediastinum in the frontal plane. Presentation of flow with color Doppler. Diastole. The confluence of the pulmonary veins with two right and one (inferior) left pulmonary veins is visible (1, 2, 3). Also visible are: the connection of the confluence (C) with the left-sided vertical vein (VV), the part of the main pulmonary artery (PA), right pulmonary artery, transverse section of the ascending aorta and superior vena cava, dilated out of proportion regarding the flow it carries. The flow in pulmonary veins and in the confluence is directed upward, therefore is coded red. There is no flow in the arteries (diastolic phase). The flow in the superior vena cava would be expected to be directed exclusively downward and therefore shown in blue (F1); instead, the intensive, continuous red (directing upward) stream of flow (F2) is present close to the left wall of the SVC. This picture strongly suggests a connection of an additional vessel – most likely the right upper pulmonary vein – with the SVC
Echocardiographic view of upper mediastinum in the frontal plane. Presentation of flow with color Doppler. Diastole. The confluence of the pulmonary veins with two right and one (inferior) left pulmonary veins is visible (1, 2, 3). Also visible are: the connection of the confluence (C) with the left-sided vertical vein (VV), the part of the main pulmonary artery (PA), right pulmonary artery, transverse section of the ascending aorta and superior vena cava, dilated out of proportion regarding the flow it carries. The flow in pulmonary veins and in the confluence is directed upward, therefore is coded red. There is no flow in the arteries (diastolic phase). The flow in the superior vena cava would be expected to be directed exclusively downward and therefore shown in blue (F1); instead, the intensive, continuous red (directing upward) stream of flow (F2) is present close to the left wall of the SVC. This picture strongly suggests a connection of an additional vessel – most likely the right upper pulmonary vein – with the SVC

Fig. 2.

Slight anterior tilt of the probe revealed a wide, left-sided vertical vein (VV) – a connection of the pulmonary confluence and systemic veins. The SVC seems wider in this view compared to the former figure, also the upwardly directed flow appears clearer (F2)
Slight anterior tilt of the probe revealed a wide, left-sided vertical vein (VV) – a connection of the pulmonary confluence and systemic veins. The SVC seems wider in this view compared to the former figure, also the upwardly directed flow appears clearer (F2)

Fig. 3.

The measurement of the velocity of this flow with spectral Doppler revealed the spectrum typical for the flow in the pulmonary vein in the case of large left to right shunt (high velocity, turbulent), again – upward, directed oppositely than anticipated normal flow in the SVC
The measurement of the velocity of this flow with spectral Doppler revealed the spectrum typical for the flow in the pulmonary vein in the case of large left to right shunt (high velocity, turbulent), again – upward, directed oppositely than anticipated normal flow in the SVC

Fig. 4.

Subcostal sagittal view showing the entrance of the SVC (SVC2) to the right atrium (RA) and the interatrial septum. The diameter of the proximal part of the SVC seems normal; however, its more distal part is markedly dilated (SVC1). The valve of the foramen ovale is shifted toward the left atrium. Also visible are: transverse section of the right pulmonary artery (RPA) and the pulmonary veins’ confluence (C). The layer of the fluid in the pericardial space is suggestive of heart failure
Subcostal sagittal view showing the entrance of the SVC (SVC2) to the right atrium (RA) and the interatrial septum. The diameter of the proximal part of the SVC seems normal; however, its more distal part is markedly dilated (SVC1). The valve of the foramen ovale is shifted toward the left atrium. Also visible are: transverse section of the right pulmonary artery (RPA) and the pulmonary veins’ confluence (C). The layer of the fluid in the pericardial space is suggestive of heart failure

Fig. 5.

The same view as in Fig. 4, flow coded with color. Two streams of flow are visible within the SVC: typical inflow from SVC to the RA, coded in red, and, appearing at the level of SVC/RPA crossing, directed oppositely – coded in blue. Both the direction and possible site of connection suggest an additional pulmonary vein joining the SVC
The same view as in Fig. 4, flow coded with color. Two streams of flow are visible within the SVC: typical inflow from SVC to the RA, coded in red, and, appearing at the level of SVC/RPA crossing, directed oppositely – coded in blue. Both the direction and possible site of connection suggest an additional pulmonary vein joining the SVC

Fig. 6.

Angio-tomography 3-D reconstruction. The heart seen from the posterior perspective after removal of the vertebral column and posterior parts of the ribs. C: pulmonary vein confluence; PVs – pulmonary veins; 1 left lower, 2 – right lower, 3 – right upper. VV – vertical vein, VBC – left brachiocephalic vein, SVC – superior vena cava, * ostium of the azygos vein; RPA – right pulmonary artery. All vascular structures are clearly delineated on this picture. The only vein joining the superior caval vein has typical features of an azygos vein: runs in sagittal plane, does not receive any veins from the lungs
Angio-tomography 3-D reconstruction. The heart seen from the posterior perspective after removal of the vertebral column and posterior parts of the ribs. C: pulmonary vein confluence; PVs – pulmonary veins; 1 left lower, 2 – right lower, 3 – right upper. VV – vertical vein, VBC – left brachiocephalic vein, SVC – superior vena cava, * ostium of the azygos vein; RPA – right pulmonary artery. All vascular structures are clearly delineated on this picture. The only vein joining the superior caval vein has typical features of an azygos vein: runs in sagittal plane, does not receive any veins from the lungs

Fig. 7.

The heart seen from the right side, 3D angio-CT reconstruction. From this perspective, the unusual dilatation of the distal SVC as well as the left brachiocephalic vein (VBC) with relatively narrow proximal part of SVC is more apparent
The heart seen from the right side, 3D angio-CT reconstruction. From this perspective, the unusual dilatation of the distal SVC as well as the left brachiocephalic vein (VBC) with relatively narrow proximal part of SVC is more apparent

Fig. 8.

A corresponding 2D CT-angiographic view. The marked disproportion between the proximal and medial and distal part of the SVC is apparent. The prominent venous channel joining the SVC just above the crossing with RPA has typical features of azygos vein (*); runs in sagittal plane, emerges from the paravertebral space, does not receive any veins from the lungs
A corresponding 2D CT-angiographic view. The marked disproportion between the proximal and medial and distal part of the SVC is apparent. The prominent venous channel joining the SVC just above the crossing with RPA has typical features of azygos vein (*); runs in sagittal plane, emerges from the paravertebral space, does not receive any veins from the lungs
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