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.