Elchanan Bruckheimer1, Tamir Dagan2, Eli Atar3, Michael Schwartz3, Ludmila Kachko4, Riccardo Superina2, Gabriel Amir2, Rivka Shapiro5, Einat Birk2. 1. Section of Pediatric Cardiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel. elchananb@bezeqint.net. 2. Section of Pediatric Cardiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel. 3. Section of Radiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel. 4. Section of Anesthesiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel. 5. Section of Gastroenterology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
Abstract
PURPOSE: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. METHODS: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ~50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. RESULTS: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. CONCLUSION: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.
PURPOSE: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. METHODS: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ~50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. RESULTS: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. CONCLUSION: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.
Authors: Francesca Romana Ponziani; Mariella Faccia; Maria Assunta Zocco; Valerio Giannelli; Adriano Pellicelli; Giuseppe Maria Ettorre; Nicoletta De Matthaeis; Fabrizio Pizzolante; Anna Maria De Gaetano; Laura Riccardi; Maurizio Pompili; Gian Ludovico Rapaccini Journal: J Ultrasound Date: 2018-10-24