Operative mortality rate of the palliative repair of Asplenia and single ventricle with total anomalous pulmonary venous connection (TAPVC) during neonate is extremity high. Although the surgical results is improving in the modern surgical era, It will be still extremely challenging in these patients. Therefore, alternative method to treat TAPVC in the neonate period has been expected.
Catheter intervention is one of the most important treatments in complex heart disease. Stent implantation for pulmonary venous drainage route early after birth could be effective and less invasive than surgery to prevent pulmonary venous occlusion maintaining stable hemodynamics in early infantile and neonatal period.
In addition, this procedure may delay invasive open cardiac surgery. We succeeded in three neonatal cases for stent implantation to vertical vein (VV) or ductus venosus (DV) for Asplenia syndrome with supracardiac or infracardiac type of TAPVC. One of them was diagnosed during pregnancy by the fetal echocardiogram. We scheduled her delivery and performed stent implantation. For better result, it is expected to make an intrauterine diagnosis by the fetal echocardiogram, premediated delivery and subsequent stent implantation, especially with infracardiac type TAPVC.
Although it is considered that the appropriate size of the stent was 8 mm for these neonates in our study, an appropriate size of the stent for the longer patency should be analyzed in the future.
In conclusion, stent implantation for pulmonary venous drainage route early after birth is effective and less invasive therapy in neonates with complex congenital heart disease.