*Corresponding author email: rwgoodga@utmb.edu Symbiosis Group Symbiosis www.symbiosisonline.org www.symbiosisonlinepublishing.com The Frequency and Significance of Portopulmonary Venous Anastomosis (PPVA): A Case Report and Literature Review Muhannad Al Hanayneh, Deborah Majchel-Koss, Eric Walser, and Richard W Goodgame* Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Texas Medical Branch, USA Journal of Gastroenterology, Pancreatology & Liver Disorders Open Access Case Report Received: October 15, 2014; Accepted: December 04, 2014; Published: December 15, 2014 *Corresponding author: Richard W. Goodgame, Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Texas Medical Branch, USA, Tel: 409-772-2653; Fax: 409-772-3394; E-mail: rwgoodga@utmb.edu Introduction Portal hypertension is a common complication of cirrhosis. The increased pressure in the portal system leads to the development of portosystemic collateral vessels. Many pathways of collateral communication between the portal and systemic circulation have been described [1,2]. Some cirrhotic patients with portal hypertension develop collateral communication between the portal system and the pulmonary veins [3,4]. These collaterals have been called Portopulmonary Venous Anastomosis (PPVA). The term PPVA differentiates direct portal to pulmonary vein anastomosis from the intra-pulmonary shunts associated with the Hepatopulmonary Syndrome (HPS). This report describes a cirrhotic patient diagnosed with HPS. He was not a candidate for liver transplantation. In the setting of progressive symptoms of defective oxygenation, further studies were done to find an additional treatable cause of hypoxemia. Computed tomography revealed a PPVA connecting a large paraesophageal varices to the right inferior pulmonary vein. We hypothesized that the shunt may be contributing to the hypoxemia. We also hypothesized that the hypoxia might be improved if the portal pressure was reduced or if the PPVA was obliterated. There is limited data in the literature about the relation between PPVA and clinically significant hypoxia. Case Report The patient was a 52 year old male incarcerated in the state prison system. In August, 2012, he was admitted to a large referral prison hospital for minor trauma. Close questioning revealed episodes of dizziness and near syncope while standing. He had a past medical history of HCV associated cirrhosis (MELD score 13), systemic arterial hypertension, and hyperlipidemia. He also had a history of mild Portosystemic Encephalopathy (PSE) and non- bleeding gastric and esophageal varices. Physical examination showed cutaneous spider angiomata, central cyanosis, and bilateral symmetric upper extremity digital clubbing. Laboratory values showed hemoglobin of 14.1 g/dL, platelets of 38,000/mm 3 , and leukocyte count 3,800/mm 3 . During the hospitalization the patient was noted to have abnormal peripheral pulse oximetry on room air: supine 83%; standing 75%. The oximetry corrected to 95% when he breathed 40% oxygen by face mask. Arterial Abstract A Portopulmonary Venous Anastomosis (PPVA) is a direct vascular connection between the portal venous system and the pulmonary veins, close to the left atrium. We describe a patient with advanced cirrhosis and moderately severe Hepatopulmonary Syndrome (HPS) in whom (PPVA) was demonstrated by a high resolution computed tomographic scan. In order to reduce the symptoms of hypoxia in our patient, we sequentially performed Transjugular Intrahepatic Portosystemic Shunt (TIPS) followed by a catheter based obliteration of the PPVA. The case raised three questions which were addressed through an extensive literature review. Among patients with advanced cirrhosis, what is the frequency of PPVA? Our literature review suggests that the frequency of finding a PPVA in patients with portal hypertension depends on the imaging technique used. Transhepatic or transvenous portal venography may demonstrate PPVA in about 20% of patients with cirrhosis and varices. Limited published experience with contrast enhanced (bubble) echocardiography suggests that PPVA may be present in about 30% of such patients. An increasing number of case reports of PPVA have been published in recent years. This observation indicates that routine use of High Resolution Computed Tomography (HRCT) and magnetic resonance imaging is able to identify PPVA in cirrhotic patients. The visualization of PPVA with HRCT depends on the timing of the contrast injection and the expertise of the viewer. Among cirrhotic patients with PPVA, what is the clinical significance of the PPVA? There are multiple causes of clinically significant hypoxia in patients with cirrhosis. A PPVA is a right to left shunt that, theoretically, could be associated with clinically significant systemic arterial hypoxemia or emboli. There is not definitive evidence in the published literature that PPVA alone causes clinically significant hypoxia in cirrhotic patients. PPVA is, however, a documented important risk factor for systemic emboli when needle or catheter techniques are used to treat or prevent bleeding from gastroesophageal varices. What is the evidence that the obliteration of a documented PPVA will improve oxygenation in a cirrhotic patient with hypoxemia? Modern imaging methods may demonstrate a PPVA when patients with cirrhosis are investigated because of hypoxia. Previous authors have argued that the right to left shunting of blood in a PPVA does not have enough flow to significantly lower arterial oxygen concentration. Our report documents that the obliteration of a PPVA can be accomplished. However, our patient’s oxygenation did not improve with obliteration of the PPVA. We do not believe that PPVA causes hypoxia. But knowledge of the frequency and clinical significance of PPVA is important for the care of patients with cirrhosis. Keywords: Portal hypertension, TIPS, Hypoxia, Cirrhosis, Balloon-occluded Retrograde Transvenous Obliteration (BRTO)