Pathology of pulmonary venous obstruction after total
anomalous pulmonary venous connection repair
Some authors indicate the following pathologies as the causes of PVO
after conventional TAPVC repair: 1) thickened pulmonary vessel walls, 2)
pulmonary lymphangiectasia, and 3) surgical anastomotic stricture
(14,15). Although PVO is usually a serious condition that directly leads
to death (1,2), our five patients of seven patients with postoperative
PVO survived. All PVOs were local branch PVOs and did not show severe
stenosis. The overall PV function was therefore maintained sufficiently
for Fontan candidates except one patient who died after Fontan. The
sutureless technique, using a large circular anastomosis, may have
prevented the three abovementioned pathologies after conventional TAPVC
repair. Although a single PVO is usually well tolerated, any minor PVOs
may affect the long-term results of congenital heart disease.
In the present study, we hypothesized that the following two anatomical
factors that narrow the space behind the heart were associated with
postoperative PVO: 1) the anatomical relationship between the arch and
apex, i.e., whether they are present on the same side or not, and 2)
apex rotation into the thoracic cavity. Both factors may exaggerate the
compression of PVs. Although our hypothesis 1) i.e., the anatomical
relationship between the arch and apex, was denied,
the relationship between heart
apex and PVO was very likely. Furthermore, a change in the V-A angle,
indicating apex rotation, was evident in the PVO group. We speculate the
mechanism of apex rotation into the ipsilateral thorax and the
relationship between apex rotation and postoperative PVO as follows: the
apex rotation would be attributed to the fact that the pericardium was
left open after surgery. Furthermore, the amount of the tissue
surrounding the suture line may have affected PVO: especially, since
sutureless technique needs a large anastomotic site, the area in contact
with the surrounding tissue is also large. Thus, the suture line would
be easily affected by surrounding tissue. In all six PVO cases, the
lower PV branch in the apex side was diffusely stenotic as indicated in
Fig. 1B.
Any modifications of the currently available techniques, e.g., more
reduction of the surrounding tissue by resecting posterior wall of the
atrium might decrease the incidence of postoperative PVO. Although we
attempted to prevent the rotation of the apex into the thoracic cavity
by fixing or closing the pericardium, we had to reopen the pericardium
in all patients due to low cardiac function during or early after the
surgery. We, therefore, could not prove the effectiveness of this
strategy.