Oesophageal Variceal-Pulmonary Venous Fistula - A Rare Cause of a Right-to-Left Shunt
  • Neeraja Swaminathan
    Albert Einstein Medical Center, Philadelphia, USA
  • Siddique Chaudhary
    Albert Einstein Medical Center, Philadelphia, USA


Right-to-left shunt, oesophageal varices


Oesophageal varices are a dilated submucosal venous plexus in the lower third of the oesophagus which result from increased pressure in the portal venous system. The portal system is connected to the systemic circulation in specific locations referred to as sites of portosystemic anastomosis. An increase in portal venous pressure is therefore reflected at these anastomotic sites, causing manifestations such as oesophageal varices, rectal varices, caput medusae and splenorenal shunts. Varices do not cause symptoms until they leak or rupture and this is the main complication which requires prompt treatment. Here, we present a post-liver transplant patient with metastatic hepatocellular carcinoma who had oesophageal varices that fistularized with a left pulmonary vein, thus creating a right-to-left shunt. Right-to-left shunts are usually intracardiac or intrapulmonary in location. The complications of a right-to-left shunt include predominantly hypoxia, cyanosis and, sometimes, paradoxical emboli in the case of intracardiac shunts. This patient had a very uncommon cause of such a shunt caused by a direct fistulous connection.



  • Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, et al. International liver transplant society practice guidelines: diagnosis and management of hepatopulmonary syndrome and portopulmonary hypertension. Transplantation 2016;100:1440–1452.

  • Schenk P, Schöniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Müller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 2003;125:1042–1052.

  • Krowka MJ, Tajik AJ, Dickson ER, Wiesner RH, Cortese DA. Intrapulmonary vascular dilatations (IPVD) in liver transplant candidates. Screening by two-dimensional contrast-enhanced echocardiography. Chest 1990;97:1165–1170.

  • Stoller JK, Lange PA, Westveer MK, Carey WD, Vogt D, Henderson JM. Prevalence and reversibility of the hepatopulmonary syndrome after liver transplantation. The Cleveland Clinic experience. West J Med 1995;163:133–138.

  • Hopkins WE, Waggoner AD, Barzilai B. Frequency and significance of intrapulmonary right-to-left shunting in end-stage hepatic disease. Am J Cardiol 1992;70:516–519.

  • O'Driscoll BR, Howard LS, Earis J, Mak V; British Thoracic Society Emergency Oxygen Guideline Group. BTS guideline for oxygen use in adults in healthcare and emergency settings. Thorax 2017;72 Suppl 1:ii1–ii90.

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    Published: 2020-03-12
    Issue: 2020: Vol 7 No 4 (view)

    How to cite:
    Swaminathan N, Chaudhary S. Oesophageal Variceal-Pulmonary Venous Fistula - A Rare Cause of a Right-to-Left Shunt. EJCRIM 2020;7 doi:10.12890/2020_001482.