PMC9584733_01
Plain radiograph of the chest showing mild cardiomegaly with left atrialization and slight increase in pulmonary blood flow.
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CXR taken at our hospital showed mild cardiomegaly (Figure 1).
Echocardiography showing a four-chamber view with partial anomalous right pulmonary venous drainage with blood flow from the right lower pulmonary vein to inferior vena cava. LA: left atrium; RA: right atrium; RPV: right pulmonary vein.
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Two-dimensional transthoracic echocardiography revealed marked right atrial and right ventricular enlargement with left to right shunt via an inferior SVD with a suspected PAPVD (Figure 2).
Cardiac magnetic resonance imaging shows a residual sinus venosus defect (black arrow) between the right lower pulmonary vein (RLPV) and inferior vena cava. (a) Coronal view and (b) sagittal view from the left side. Anomalous right lower pulmonary vein drainage can also be observed.
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Cardiac magnetic resonance (CMR) imaging confirmed the diagnosis of an inferior SVD that measured 17.2 mm in length with partial anomalous pulmonary venous drainage (PAPVD) of the RLPV into the IVC (Figures 3(a) and 3(b)).
Anterior aspect of a 3-dimensional model from cardiac magnetic resonance imaging. (a) The superior vena cava- (SVC-) right atrium (RA) junction, right pulmonary artery (RPA), right lower pulmonary vein (RLPV), right hepatic vein (RHV), left hepatic vein (LHV), and inferior vena cava (IVC) can be observed. An opening was artificially created (white arrow) to facilitate a simulated view into the lateral aspect of the RA. (b) A sinus venosus defect was identified at the IVC-RA junction (black arrow).
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Based on three-dimensional (3D) reconstruction of the respiratory-navigated and electrocardiography- (ECG-) gated Dixon CMR sequence, a 3D-printed model (Figure 4(a)) was used to simulate the landing zone for each bare and covered stent deployment, including the preferable angle of angiography. The model was used to evaluate an anterior view of the RA (Figure 4(a)) and by looking into an opening that was artificially cut into the lateral wall of the RA to directly visualize the SVD (Figure 4(b)).
A 3-dimensional model was created to simulate balloon sizing in the inferior vena cava (IVC) (a). The critical point at the IVC-right atrium junction was 20.3 mm. A decision was made to position a 24 mm anchored bare stent (black outlined stent) at this location (b). A long wire was placed in the superior vena cava (SVC), and a short wire was placed in both the left hepatic vein (LHV) and the IVC. The aim of the placement of the bare stent (black outlined stent) and the subsequently positioned covered stent (white outlined stent) was to cover the sinus venosus defect and to ensure no obstruction of blood flow in the LHV and the right hepatic vein (RHV).
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The 3D model was also used for simulation of balloon sizing to measure the anchoring area diameter of 20.27 mm (Figure 5(a)). The aim of the covered stent was to cover the superior border of the ASD (Figure 5(b)).
Angiography was performed in the inferior vena cava (IVC). The (a) anterior and (b) lateral views show the connection between the right lower pulmonary vein (RLPV) and the right atrium. The decision was made to place the lower end of the covered stent above the entrance of the left and right hepatic veins (RHV).
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Angiography was performed in the IVC to visualize hepatic vein entrance into the IVC (Figures 6(a) and 6(b)).
The (a) arterial phase and (b) levo phase of right pulmonary artery (RPA) angiography shows drainage of the right lower pulmonary vein (RLPV) into the right atrium.
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The site of the entrance of the RLPV into the IVC and the lower end of the SVD were also profiled in this angiogram (Figures 7 and 8).
Right lower pulmonary vein (RLPV) angiography was performed simultaneous both (a) anterior view and (b) lateral view of the right atrium. The drainage of the RLPV into the right atrium (white arrow) was also shown in both views. (a) The decision was made to place the upper part of the covered stent and anterior and above the entrance of this RLPV (white arrow) to direct the pulmonary venous blood return to the left atrium.
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RLPV drainage. RLPV venography was performed in the anteroposterior (AP) (Figure 8(a)) and lateral (Figure 8(b)) views to determine the location of the RLPV relative to the posterior wall of the RA. The site of the entrance of the RLPV into the IVC and the lower end of the SVD were also profiled in this angiogram (Figures 7 and 8).
Balloon sizing of the sinus venosus defect that was performed at the inferior vena cava (IVC)-right atrium junction ((a) anterior and (b) lateral views on angiography) showed the right hepatic vein (RHV) to be occluded by the balloon. During the same occlusion, we placed a catheter in the left hepatic vein (LHV) and simultaneously injected contrast showing the LHV connection ((c) anterior and (d) lateral views).
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A simultaneous contrast injection was performed at both right (Figures 9(a) and 9(b)) and left (Figures 9(c) and 9(d)) hepatic veins.
The bare stent, which was placed first, was positioned at the inferior vena cava-right atrium junction ((a) anteroposterior and (b) lateral views on angiography). The decision was then made to position the lower end of the planned covered stent, which will be placed later, above the entrances of the right and also left hepatic veins (black arrow), and that the covered stent would overlap with the bare stent approximately 50%. It was also noted that the distal part of this covered stents should position just above the entrance of the right lower pulmonary vein (white arrow).
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After stent placement, right hepatic vein flow could freely drain into the stent (Figures 10(a) and 10(b)). The decision was made to place the lower end of the covered stent above the entrance of the hepatic veins, but the bare stent had been positioned lower down than proper position making the overlapping segment shorter than 50% as shown in Figure 10 as angiogram is more practical and informative during the procedure than CMR, cardiac CT, or 3D printing. We estimated that this positioning would allow enough distance above the openings of both the right and left hepatic veins (Figure 10).
The covered stent (white arrow), which was placed second, is positioned superior to the anchored bare stent (black arrow) in the inferior vena cava ((a) anteroposterior and (b) lateral views on angiography). The placement of covered stent was supposed to be above the entrance of the right lower pulmonary vein (RLPV) but at the expense of overlapping segment because the bare stent was placed lower enough into the IVC. This can be compared with final position of the covered stent after repositioning almost covering the entrance of RLPV but lower than the first position (Figure 15). The stitch can be used as a guide line.
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Nonetheless, the covered stent was finally deployed at the intended position (Figures 11(a) and 11(b)).
The covered stent unexpectedly embolized into the right atrium as visualized on anterior view angiography of the right atrium. The black arrow and white arrow indicate the position of the bare stent and the covered stent, respectively.
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The covered stent then unexpectedly migrated into the RA shortly after it was deployed (Figure 12).
The balloon inside the covered stent was inflated in an attempt to pull down and reposition the embolized covered stent, but that attempt was unsuccessful. We then deployed another 24 mm x 40 mm Z-Med balloon in an attempt to use an external force to squeeze or pushing the embolized covered stent to a smaller diameter to be able to pull back inside first stent, but that was also unsuccessful due to the strong radial force of the covered graft (a, b). The black arrow and white arrow indicate the position of the bare stent and the covered stent, respectively.
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We then deployed a second 24 mm x 40 mm Z-Med II balloon in an attempt to use an external force to squeeze or push the migrated covered stent to a smaller diameter to be able to pull back inside first stent, squeeze; however, this attempt was also unsuccessful due to the strong radial force of the covered stent (Figures 13(a) and 13(b)).
A 35 mm Amplatz Gooseneck snare was deployed from a distal Amplatz Super Stiff wire (a, b). The snare was used to grasp the middle of the embolized covered stent and to pull it back inside the anchored bare stent (c, d); however, we were not able to release the snare once the covered stent was back inside the bare stent (e, f). We then redeployed the snare at a more distal position and then pulled the embolized stent with balloon inflated in the more distal position to coaxial both stents and eventually released snare (g, h).
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We then deployed a 35 mm Amplatz Gooseneck Snare (Medtronic, Minneapolis, MN, USA) via a delivery catheter in an attempt to grasp distal wire position of from the left femoral vein and was used to snuggle the distal part of the migrated covered stent (Figures 14(a) and 14(b)). We then decided to snare the stent with simultaneous inflation of the distal part of the stent to make it coaxial/telescopic to the anchored bare stent, and this strategy successfully released the migrated stent and allowed us to pull it back into position within the bare stent (Figures 14(c)-14(f)). The snare was then repositioned to the distal part of the stent to facilitate successful release of the snare once the covered stent was repositioned within the bare stent (Figures 14(g) and 14(h)).
Angiography showing the previously embolized covered stent successfully pulled back inside the primary anchored bare stent. The black arrow and white arrow indicate the position of the bare stent and the covered stent, respectively.
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This strategy resulted in the successful repositioning of the covered stent and the successful release of the snare (Figures 15(a) and 15(b)).