Reversed Intrapulmonary Right-to-Left Shunt After Banding of the Patent Ductus Venosus

Abstract

Diffuse pulmonary microvascular arteriovenous communication developed in an 8-year-old girl with a patent ductus venosus. Tc-99m macroaggregated albumin (MAA) pulmonary perfusion scintigraphy with total-body imaging demonstrated multiple lung perfusion deficits and abnormal tracer uptake in systemic organs with hepatic radioactivity greater than the kidneys, suggesting the presence of right-to-left shunt and abnormal hepatic hemodynamics. I-123 iodoamphetamine transrectal portal scintigraphy revealed a large portosystemic venous shunt. The follow-up Tc-99m MAA perfusion scans after banding of the patent ductus venosus revealed partial improvement of the perfusion deficits and right-to-left shunt, indicating the possible reversibility of this pulmonary shunt complication.

Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management

“…Two studies use the results of neonatal screening for galactosemia after a few days of feeding to estimate the prevalence of congenital portosystemic shunts 144,145 : High concentrations of blood galactose, unexplained by an abnormal activity of the enzymes of galactose metabolism, can be found in neonates 11,16,24,25,27,29,38,41,44,51,53,55,59,64,68,70,76,84,94,96,101,105,106,[118][119][120][121]126 with CPSS because galactose from milk bypasses the liver. The overall prevalence is close to 1:30,000 births and the prevalence of permanent CPSS can be estimated at 1:50,000.…”

“…Chronic hypoxemia with clinical characteristics similar to those of hepatopulmonary syndrome has been reported in 32 children at ages ranging from 3 months to 11 years (mean: 4 years 4 months). 19,29,44,56,64,66,69,72,[77][78][79]82,[88][89][90]100,104,110,112,115,118,123,127,128,133,[135][136][137]139,140 It was observed with all anatomic types of CPSS and led to the diagnosis of CPSS in 29 instances. Hepatopulmonary syndrome was a complication in 10 (none with biliary atresia) of the 23 children with CPSS and polysplenia syndrome and in 22 of the 242 children without polysplenia syndrome (p < 0.0001; Fisher's exact test).…”

“…Other complications have been reported in a few children that might be consequences of the CPSS: They include heart failure, 7,32,48,84,118 membranoproliferative glomerulopathy, 45,118,131,135 hypoglycemia, 53,55,77,134 hyperandrogenism, 43,52 pancreatitis, 101 rectal or vaginal bleeding, 43,67,105,132 autoimmune diseases, 64,77,99,101 protein-losing gastropathy, 35,132 unforeseen bleeding during surgery for scoliosis, 117 and acute lethal liver failure during an episode of gastroenteritis in a 1.7-year-old boy with an extrahepatic shunt. 54 Hypoxemia due to pulmonary arteriovenous shunting, pulmonary artery hypertension, encephalopathy with hyperammonemia, and glomerulopathy are known complications of cirrhosis, and their occurrence in children with congenital portosystemic shunts and minimal liver histologic lesions argues in support of the major role of communications between the portal blood and the systemic circulation in the origin of these conditions.…”

Angiographic diagnosis for accurate assessment of congenital porto‐systemic shunt and extrahepatic portal vein obstruction in children

“…The prevalence of permanent CPSS is approximately 1 in 50 000 2 . Although some patients may remain asymptomatic for decades, 3 the abnormal connection between the portal and systemic veins in CPSS can cause serious complications, such as hepatic encephalopathy, 4–6 liver tumors, 7–9 pulmonary hypertension (PH), 10–12 and pulmonary arteriovenous fistulas (PAVFs) 13–15 . Congenital porto‐systemic shunt has conventionally been classified into extrahepatic shunt and intrahepatic shunt, while extrahepatic shunt is further classified into type 1, “Abernethy malformation,” a complete absence of the portal vein, and type 2, describing a preserved portal venous flow to the liver 16 .…”

“…2 Although some patients may remain asymptomatic for decades, 3 the abnormal connection between the portal and systemic veins in CPSS can cause serious complications, such as hepatic encephalopathy, [4][5][6] liver tumors, [7][8][9] pulmonary hypertension (PH), [10][11][12] and pulmonary arteriovenous fistulas (PAVFs). [13][14][15] Congenital porto-systemic shunt has conventionally been classified into extrahepatic shunt and intrahepatic shunt, while extrahepatic shunt is further classified into type 1, "Abernethy malformation," a complete absence of the portal vein, and type 2, describing a preserved portal venous flow to the liver. 16 Patients with congenital portal vein absence are candidates for liver transplantation, and others can be treated by surgical closure of the shunts or transcatheter intervention to restore the physiologic blood flow of the intrahepatic portal vein (IHPV).…”