BACKGROUND: Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated. METHODS: Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005. RESULTS: Patent ductus venosus was detected in 5 patients, including 3 patients with an absence of the portal vein. The presence of either a gastrorenal or splenorenal shunt was evident in another 4 patients. Six patients had a history of hypergalactosemia with normal enzyme activities, as seen during neonatal screening. Six (66.7%) of the 9 patients were identified to have clinically significant pulmonary arterial hypertension (mean pulmonary artery pressure: 34-79 mm Hg; pulmonary vascular resistances: 5.12-38.07 U). The median age at the onset of pulmonary arterial hypertension was 12 years and 3 months. Histologic studies of lung specimens, which were available in 4 of the 9 patients with congenital portosystemic venous shunt, showed small arterial microthrombotic lesions in 3 patients. This characteristic finding was recognized even in the congenital portosystemic venous shunt patients without PAH. CONCLUSIONS: This study demonstrated thromboembolic pulmonary arterial hypertension to be a crucial complication in congenital portosystemic venous shunt, and this pathologic state may be latently present in patients with pulmonary arterial hypertension of unknown etiology.
BACKGROUND:Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated. METHODS: Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005. RESULTS: Patent ductus venosus was detected in 5 patients, including 3 patients with an absence of the portal vein. The presence of either a gastrorenal or splenorenal shunt was evident in another 4 patients. Six patients had a history of hypergalactosemia with normal enzyme activities, as seen during neonatal screening. Six (66.7%) of the 9 patients were identified to have clinically significant pulmonary arterial hypertension (mean pulmonary artery pressure: 34-79 mm Hg; pulmonary vascular resistances: 5.12-38.07 U). The median age at the onset of pulmonary arterial hypertension was 12 years and 3 months. Histologic studies of lung specimens, which were available in 4 of the 9 patients with congenital portosystemic venous shunt, showed small arterial microthrombotic lesions in 3 patients. This characteristic finding was recognized even in the congenital portosystemic venous shunt patients without PAH. CONCLUSIONS: This study demonstrated thromboembolic pulmonary arterial hypertension to be a crucial complication in congenital portosystemic venous shunt, and this pathologic state may be latently present in patients with pulmonary arterial hypertension of unknown etiology.
Authors: William N Evans; Alvaro Galindo; Ruben J Acherman; Abraham Rothman; Dean P Berthoty Journal: Pediatr Cardiol Date: 2009-07-23 Impact factor: 1.655
Authors: Jeong-Eun Yi; Hae-Ok Jung; Ho-Joong Youn; Jong Young Choi; Ho Jong Chun; Jae Young Lee Journal: J Korean Med Sci Date: 2014-04-01 Impact factor: 2.153
Authors: Myung Jin Kim; Jae Sung Ko; Jeong Kee Seo; Hye Ran Yang; Ju Young Chang; Gi Beom Kim; Jung-Eun Cheon; Woo Sun Kim Journal: Eur J Pediatr Date: 2011-09-13 Impact factor: 3.183
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