Due to the increased levels of humoral substances in the lung tissue, chronic pulmonary vasoconstriction can occur in patients with CPSS. Our patient presented with difficulty breathing due to severe pulmonary hypertension, in addition to hyperammonemia due to concomitant liver dysfunction [4].

Kuo and colleagues performed the first endovascular closure of a portosystemic shunt in 2010. They used a three-stage approach due to associated portal vein hypoplasia. Subsequently, several studies have been published [7].

It is noticeable from the cases summarized in our table that interventional radiologists rely on vascular plugs in the closure of this malformation, whereas pediatric cardiologists perform most of their procedures using duct, atrial septal, and ventricular septal occluders. Vascular plugs are primarily used to treat extracardiac defects. Perhaps this is the reason why they are rarely used by interventional cardiologists and thus rarely available in pediatric cardiac catheter laboratories. This probably explains the bias in the devices used by different teams to close the same defect.

Table 1 shows reports where the endovascular approach was used for CPSS closure [7,8,9,10,11,12,13].

A very important consideration to be taken into consideration, during and after closure, is the possible hypoplasia of intrahepatic pulmonary veins. This hypoplasia is either managed with staged repair or with close follow-up every 3–6 months for any signs of portal hypertension and serial ammonia measurements after shunt closure. Nevertheless, prolonged intrahepatic hypoxia can stimulate neoplastic activity and nodule formation; therefore, experts recommend annual abdominal ultrasound to detect any liver neoplasm [6].