Surgical ductal stent implantation in total anomalous pulmonary venous connection to vena porta with right pulmonary sequestration in a mature newborn.

In many cases of total anomalous pulmonary venous connection (TAPVC), the four pulmonary veins (PV) join together behind the left atrium, where they form a collector. This collector can drain into the right atrium directly through the innominate vein into the superior vena cava (SVC), into the coronary sinus (CS), or through the diaphragm to the venous structures of the abdomen. In our case, a mature newborn had TAPVC draining into the vena porta along with severe pulmonary hypertension. Additionally, there were right pulmonary sequestration, dextrocardia, transposition of the great arteries, severe pulmonary stenosis, and single ventricular pathology in echocardiographic examination. Clinical signs manifested in the first 7 days of life. Diagnostic tools used were echocardiography and angiography. A ductal stent was surgically implanted into the ductus arteriosus by angiography. TAPVC was found to be nonobstructive. Therefore, we would like to emphasize the rareness and hardness to perform the surgical ductal implant technique in our particular case of TAPVC with pulmonary sequestration draining into the vena porta. The prognosis in TAPVC is poor and related mainly to the existence of pulmonary venous obstruction.