Introduction
The surgical outcomes are unsatisfactory for patients having
single-ventricle with total anomalous pulmonary venous connection
(TAPVC) and heterotaxy syndrome. A major concern is that the anatomical
feature of extracardiac TAPVC in right atrial isomerism (RAI) frequently
causes pulmonary venous obstruction (PVO) after surgery (1-3). The
reported rates of hospital mortality and postoperative PVO are 43%-53%
(1,2) and 42% (1), respectively. Thus, both mixed-type TAPVC and
preoperative PVO have been reported as risk factors for PVO after TAPVC
repair (1-3). Alterations in anatomical factors resulting from surgery
may also affect the incidence of PVO. Therefore, the identification of
such factors may help modify the surgical strategy and methods.
The purpose of this study was to evaluate the surgical outcomes of TAPVC
repair and determine anatomical risk factors for postoperative PVO in
neonates and infants with RAI, single ventricle, and extracardiac TAPVC.
We hypothesized that 1) the anatomical relationship between the arch and
apex and 2) the apex rotation into the thoracic cavity after surgery are
both related to postoperative PVO, as they both indicate a narrowing of
the posterior space from the caudal side of the pulmonary artery (PA) to
the lower end of the lower pulmonary vein (PV) and the space between the
left and right phrenic nerves.