Case 2
A 2 year-old infant, weighing 9 kg, with a diagnosis of supracardiac TAPVC underwent surgical correction at 1 month. Preoperative echocardiography and contrast CT showed that the pulmonary veins formed a confluence and drained into the innominate vein through the vertical vein. We diagnosed supracardiac TAPVC without another cardiac anomaly.
During surgery, the vertical vein was ligated on the innominate vein side. Postoperatively, recovery was good and pulmonary venous stenosis (PVS) did not arise. Furthermore, using contrast CT, we confirmed that the connection of the common chamber and left atrium was not restrictive before the patient was discharged. A year after the surgical repair, we performed a follow up catheter examination to check the patients’ cardiac condition. It showed that central venous pressure was 5 mmHg, right ventricular pressure was 23 mmHg, mean pulmonary capillary wedge pressure 9 mmHg, left ventricular pressure was 63 mmHg, left ventricular end-diastolic volume was 104% of normal, and right ventricular end-diastolic volume 103 % of normal.
Although the patient was hemodynamically stable with no pulmonary vein stenosis (PVS) , a second vertical vein was incidentally noticed that originated from the proximal ligated vertical vein through the accessary hemiazygos vein, which drained into the superior vena cava (Figure 3).
On initial assessment, it appeared as though the second vertical vein originated from the same location as the first vein. However, the first vertical vein was obviously dominant; therefore, the second vessel would have had much less perfusion and would have been difficult to be identified before surgery. After surgery, it became visible as it had not spontaneously atrophied but rather had increased perfusion. At the time of discovery, it appeared that the RV volume load was tolerable and would be managed by careful follow-up. However, at the next check-up, tricuspid regurgitation had gradually increased. The RV volume load appeared to be worsening, and we decided to perform occlusion of the second vertical vein.
Next year of first catheter, we performed the treatment. The right femoral vein was accessed with a 5Fr venous sheath, and the accessary hemiazygos veins were accessed from the SVC using a 4 Fr/100 cm GlidecathⅡ catheter with a Berenstein tip (Terumo). A diagnostic contrast angiography using a 2.2 Fr Progreatβ3microcatheter (Terumo) was also performed in the SVC and left pulmonary with occlusion 4Fr wedge in the azygos vein. The left pulmonary artery (LPA) mean pressure measured 22 mmHg, showing an increase from the previous year. We carefully performed coil embolization to avoid occlusion of the pulmonary vein. The first coil (8mm x 250mm MICRUSFRAME®C coil; Johnson & Johnson) was introduced in close proximity to the origin of the VV shunt to avoid occlusion of the pulmonary vein, and angiography showed it was well positioned. Additional coils (6mm x 250mm,5mm x 200mm, 4 x 150mm DELTAFILL® ; Johnson & Johnson) and a final one (3mm x 100mm AZUR®️ ; Terumo) were placed to ensure complete occlusion (Figure 4). Repeat angiography showed disappearance of the residual flow from the VV shunt. Mean left pulmonary artery pressure measurements revealed the same result before and after the procedure, and the patient was discharged from the hospital 2 days later.