Manuscript
Abstract: Giant superior vena cava (SVC) aneurysm in
obstructive supracardiac total anomalous venous connection (TAPVC) is
rare, and there has not been a giant SVC aneurysm reported in
non-obstructive TAPVC. Here we reported a 29‐year‐old female with
non-obstructive TAPVC and a giant SVC aneurysm. Routine TAPVC correction
and partial venoctomy were done, considering that such a giant aneurysm
in a non-obstructive venous arch might have its histological etiology
and higher risk of thrombosis and/or rupture.
Key words: total anomalous pulmonary venous connection,
superior vena cava, aneurysm
Giant superior vena cava (SVC) aneurysm in obstructive supracardiac
total anomalous venous connection (TAPVC) is
rare(1-3), and there has not been a giant SVC aneurysm
reported in non-obstructive TAPVC. Here we reported a 29‐year‐old female
with non-obstructive TAPVC and a giant SVC aneurysm.
The patient was seen in the clinic due to her dyspnea and impaired
exercise tolerance. Preoperative echocardiography and computed
tomography (CT) confirmed the diagnosis of supracardiac TAPVC with a
giant venous arch complicated with a 22 mm atrial septal defect (ASD)
(Figure 1A-1B;supplementary material online, video 1). The maximum
diameter of the SVC was measured 7.68×6.32 cm (Figure 1C). Neither the
echocardiography nor the CT found any obstruction throughout the venous
pathway. Angiography was conducted which showed a mean pulmonary artery
pressure of 22 mmHg and low pulmonary resistance. Intraoperative
findings were consistent with all preoperative diagnosis (Figure 1D).
While establishing the cardiopulmonary bypass, along with the routine
IVC cannulation, two separate venous catheters were placed into the
right brachiocephalic vein and the innominate vein respectively. Routine
TAPVC correction was done by anastomosing the posterior wall of left
atrium with the pulmonary venous confluence, ligating the vertical vein
and repairing the ASD.
Although some publications concluded that, comparing with a saccular
shape, a fusiform SVC aneurysm will usually have a benign
prognosis(4-7). However, considering that such a giant
SVC aneurysm presented in a non-obstructive environment, we thought it
might have its histological etiology and would take a higher risk of
thrombosis and/or rupture. Therefore, a partial venoctomy was
reasonable. By controlling the distal veins, the wall of the aneurysm
was partially resected, and the diameter of SVC was reduced to a normal
size (Figure 1E). Pathology of the SVC revealed desmoplasia with hyaline
degeneration and calcification in the vascular wall and mucoid
degeneration in the focal area. The patient had an uncomplicated
postoperative course and was discharged on the seventh postoperative
day.