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Patent ductus venosus in an adult presenting as pulmonary hypertension, right-sided heart failure, and portosystemic encephalopathy

      Ductus venosus is a normal fetal structure connecting the portal and umbilical veins with the inferior vena cava, allowing a fraction of the umbilical vein blood to bypass the liver and reach the central circulation. Patent ductus venosus is the persistence of this embryonic portosystemic shunt. It is rarely seen in adults. Fewer than 20 cases of patent ductus venosus have been reported in the literature (

      LaBerge JM, Bass NM, Messina LM. SCVIR annual meeting film panel session: diagnosis and discussion of case 5. J Vasc Intervent Radiol. 2000;655:60.

      ,
      • Ohnishi K
      • Hatano H
      • Nakayama T
      • et al.
      An unusual portal-systemic shunt, most likely through a patent ductus venosus a case report.
      ,
      • Piccone V.A
      • Lentino W
      • LeVeen H.H
      Radiological demonstration of a patent ductus venosus in an adult.
      ,
      • Barjon P
      • Lamarque J.L
      • Michel H
      • et al.
      Persistent ductus venosus without portal hypertension in a young alcoholic man.
      ,
      • Ikeda S
      • Jamaguchi Y
      • Sera Y
      • et al.
      Surgical correction of patent ductus venosus in three brothers.
      ,
      • Farrant P
      • Meire H.B
      • Karani J
      Ultrasound diagnosis of portocaval anastomosis in infants a report of eight cases.
      ,
      • Barsky M.F
      • Rankin R.N
      • Wall W.J
      • et al.
      Patent ductus venosus problems in assessment and management.
      ,
      • Orii T
      • Ohkohchi N
      • Kato H
      • et al.
      Liver transplantation for severe hypoxemia caused by patent ductus venosus.
      ,
      • Matsubara T
      • Sumazaki R
      • Saitoh H
      • et al.
      Patent ductus venosus associated with tumor-like lesions of the liver in a young girl.
      ,
      • Uchino T
      • Endo F
      • Ikeda S
      • et al.
      Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus.
      ,
      • Jacob S
      • Farr G
      • De Vun D
      • et al.
      Hepatic manifestations of familial patent ductus venosus in adults.
      ,
      • Schwartz Y
      • Berkowitz D
      • Lorber A
      Transvenous coil embolization of a patent ductus venosus in a 2-month-old child.
      ,
      • Mitchell I.M
      • Pollack J.C.S
      • Bibsion A.A.M
      Patent ductus venosus.
      ), presenting as portosystemic hepatic encephalopathy and hyperammonemia (

      LaBerge JM, Bass NM, Messina LM. SCVIR annual meeting film panel session: diagnosis and discussion of case 5. J Vasc Intervent Radiol. 2000;655:60.

      ,
      • Ohnishi K
      • Hatano H
      • Nakayama T
      • et al.
      An unusual portal-systemic shunt, most likely through a patent ductus venosus a case report.
      ,
      • Barjon P
      • Lamarque J.L
      • Michel H
      • et al.
      Persistent ductus venosus without portal hypertension in a young alcoholic man.
      ). We describe adult patent ductus venosus presenting not only with portosystemic encephalopathy, but also with pulmonary hypertension and right-sided heart failure.
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      References

      1. LaBerge JM, Bass NM, Messina LM. SCVIR annual meeting film panel session: diagnosis and discussion of case 5. J Vasc Intervent Radiol. 2000;655:60.

        • Ohnishi K
        • Hatano H
        • Nakayama T
        • et al.
        An unusual portal-systemic shunt, most likely through a patent ductus venosus.
        Gastroenterology. 1983; 85: 962-965
        • Piccone V.A
        • Lentino W
        • LeVeen H.H
        Radiological demonstration of a patent ductus venosus in an adult.
        Gastroenterology. 1967; 52: 263-266
        • Barjon P
        • Lamarque J.L
        • Michel H
        • et al.
        Persistent ductus venosus without portal hypertension in a young alcoholic man.
        Gut. 1972; 13: 982-985
        • Ikeda S
        • Jamaguchi Y
        • Sera Y
        • et al.
        Surgical correction of patent ductus venosus in three brothers.
        Dig Dis Sci. 1999; 44: 582-589
        • Farrant P
        • Meire H.B
        • Karani J
        Ultrasound diagnosis of portocaval anastomosis in infants.
        Br J Radiol. 1996; 69: 389-393
        • Barsky M.F
        • Rankin R.N
        • Wall W.J
        • et al.
        Patent ductus venosus.
        Can J Surg. 1989; 32: 271-275
        • Orii T
        • Ohkohchi N
        • Kato H
        • et al.
        Liver transplantation for severe hypoxemia caused by patent ductus venosus.
        J Pediatric Surg. 1997; 32: 1795-1797
        • Matsubara T
        • Sumazaki R
        • Saitoh H
        • et al.
        Patent ductus venosus associated with tumor-like lesions of the liver in a young girl.
        J Pediatr Gastroenterol Nutr. 1996; 22: 107-111
        • Uchino T
        • Endo F
        • Ikeda S
        • et al.
        Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus.
        Gastroenterology. 1996; 110: 1964-1968
        • Jacob S
        • Farr G
        • De Vun D
        • et al.
        Hepatic manifestations of familial patent ductus venosus in adults.
        Gut. 1999; 45: 442-445
        • Schwartz Y
        • Berkowitz D
        • Lorber A
        Transvenous coil embolization of a patent ductus venosus in a 2-month-old child.
        Pediatrics. 1999; 103: 1045-1047
        • Mitchell I.M
        • Pollack J.C.S
        • Bibsion A.A.M
        Patent ductus venosus.
        Pediatr Cardiol. 1991; 12: 181-183
        • Gitzlmann R
        • Arbenz U.V
        • Willi U.V
        Hypergalactosaemia and portosystemic encephalopathy due to persistence of ductus venosus arantii.
        Eur J Pediatr. 1992; 151: 564-568
        • Wanless I.R
        • Lentz J
        • Roberts E.A
        Partial nodular transformation of liver in an adult with persistent ductus venosus.
        Arch Pathol Lab Med. 1985; 109: 427-432
        • Mori K
        • Dohi T
        • Yamamoto H
        • et al.
        An enormous shunt between the portal and hepatic veins associated with multiple coronary artery fistulas.
        Pediatr Radiol. 1990; 21: 66-68
        • Braverman A.C
        • Steiner M.A
        • Picus D
        • et al.
        High-output congestive heart failure following transjugular intrahepatic portal-systemic shunting.
        Chest. 1995; 107: 1467-1469
        • Senior R.M
        • Britton R.C
        • Turino G.M
        • et al.
        Pulmonary hypertension associated with cirrhosis and with portacaval shunts.
        Circulation. 1968; 37: 88-96
        • Hadengue A
        • Benhayoun M.K
        • Lebrec D
        • et al.
        Pulmonary hypertension complicating portal hypertension. Prevalence and relation to splanchnic hemodynamics.
        Gastroenterology. 1991; 100: 520-528
        • Van der Linden P
        • Le Moine P
        • Ghysels M
        • et al.
        Pulmonary hypertension after transjugular intrahepatic portosystemic shunt.
        Hepatology. 1996; 23: 982-987
        • Bernthal A.C
        • Eybel C.E
        • Payne J.A
        Primary pulmonary hypertension after portocaval shunt.
        J Clin Gastroenterol. 1983; 5: 353-356
        • Shah H.A
        • Piris J
        • Finlayson N.D.C
        Primary pulmonary hypertension developing 11 years after a splenorenal shunt for portal hypertension in hepatic cirrhosis.
        Eur J Gastroenterol Hepatol. 1995; 7: 283-286
        • Gelman S
        • Aldrete J.S
        • Halpern N
        Hemodynamics during portacaval shunt surgery in humans.
        Anesthes Analges. 1982; 61: 185-186
        • Shioyama Y
        • Matsueda K
        • Horihata K
        • et al.
        Post-TIPS hepatic encephalopathy treated by occlusion balloon-assisted retrograde embolization of a coexisting spontaneous splenorenal shunt.
        Cardiovasc Intervent Radiol. 1996; 19: 53-55
        • Sakurabayashi S
        • Sezai S
        • Yamamoto Y
        • et al.
        Embolization of portal-systemic shunts in cirrhotic patients with chronic recurrent hepatic encephalopathy.
        Cardiovasc Intervent Radiol. 1997; 20: 120-124
        • Strouse P.J
        • DiPietro M.A
        Persistent patency of the ductus venosus. Consequence of portal hypertension in an infant?.
        J Ultrasound Med. 1997; 16: 559-561
        • Hanna S.S
        • Smith R.S
        • Henderson J.M
        • et al.
        Reversal of hepatic encephalopathy after occlusion of total portasystemic shunts.
        Am J Surg. 1981; 142: 285-289
        • Nishie A
        • Yoshimitsu K
        • Honda H
        • et al.
        Treatment of hepatic encephalopathy by retrograde transcaval coil embolization of an ileal vein-to-right gonadal vein portosystemic shunt.
        Cardiovasc Intervent Radiol. 1997; 20: 222-224