Jacqueline Modock , BSN, RN 2.5 ANCC Contact Hours Acute Pulmonary Hypertension After Transjugular Intrahepatic Portosystemic Shunt A Potentially Deadly but Commonly Forgotten Complication ABSTRACT Hepatitis C virus (HCV) is a common cause of chronic liver disease and is the most common indication for liver trans- plantation in the United States. As increasing numbers of the population experience complications from chronic liver disease, management of these complications comes into focus. One such management technique is a transjugular intrahepatic portosystemic shunt (TIPS). As the number of patients with HCV cirrhosis increases, the proportion of TIPS procedures performed will also increase. It is, therefore, paramount to understand the potential adverse effects of this increasingly used procedure. This case report focuses on a 52-year-old man with HCV cirrhosis who developed the complication of acute pulmonary hypertension after receiving a TIPS procedure. In this case report, we discuss this important but commonly missed complication of TIPS, including incidence, diagnosis, and treatment. s a disease, Hepatitis C is the most common Background indication for liver transplantation in the A serious complication associated with chronic United States. It is estimated that 3.2 million Hepatitis C infection is the development of chronic liver Americans are infected with the virus. The disease. The CDC (2013b) reports that the fifth leading ACenters for Disease Control and Prevention (CDC) cause of death in individuals 45–54 years old in 2009 identified these patients at increased risk for hepatitis was liver disease. During 2005–2010, the number of C infection: injection drug users, recipients of clotting annual chronic liver disease deaths attributable to factor concentrates made before 1987, recipients of Hepatitis C infection was 12,000 (CDC, 2013a). The blood transfusions or solid organ transplants before CDC predicted that “the number of deaths attributable July 1992, chronic hemodialysis patients, HIV-infected to HCV-related chronic liver disease could increase sub- patients, and children born to HCV-positive mothers stantially during the next 10–20 years as this group of (2009). While there are recommended treatment infected persons reaches ages at which complications options for Hepatitis C virus (HCV) infection, the from chronic liver disease typically occur” (CDC, 1998). majority of infections remain undiagnosed and untreat- As increasing numbers of the population experience ed leading to approximately 75%–85% of HCV infec- complications from chronic liver disease, the need for tions becoming chronic (CDC, 2009). medical management of these complications will also rise. One such management technique is a transjugular intrahepatic portosystemic shunt (TIPS). Received April 28, 2012; accepted September 15, 2012. Transjugular intrahepatic portosystemic shunt has About the author: Jacqueline Modock, BSN, RN, is Adult Gerontology been a procedure performed in cirrhotic patients for Acute Care Nurse Practitioner, Lake Tahoe Regional Hospitalist, Carson City, Nevada. more than 20 years to treat the often-deadly complica- The author declares no conflict of interest. tions of portal hypertension ( Boyer & Haskal, 2009). Correspondence to: Jacqueline Modock, BSN, RN, Case Western Reserve Although successful outcomes have been achieved with University, 1685 Murrieta Ct., Reno, NV 89521 ( [email protected] ). the TIPS procedure, the American Association for the DOI: 10.1097/SGA.0000000000000016 Study of Liver Disease continues to have very narrow VOLUME 37 | NUMBER 1 | JANUARY/FEBRUARY 2014 33 Copyright © 2014 Society of Gastroenterology Nurses and Associates. Unauthorized reproduction of this article is prohibited. GGNJ-D-12-00039R1.inddNJ-D-12-00039R1.indd 3333 11/23/14/23/14 88:02:02 AAMM Acute Pulmonary Hypertension After Transjugular Intrahepatic Portosystemic Shunt indications for the performance of a TIPS procedure Although a pulmonary embolism (PE) could not be ( Boyer & Haskal , 2009). It is only after medical man- fully ruled out, the patient’s risk factors for PE were agement has failed for abdominal ascites and variceal minimal given that he was ambulating prior to the bleeding that the morbidity and mortality associated procedure, had no history of blood clots, did not with a TIPS procedure no longer outweighs the bene- smoke, and was receiving low-dose heparin for deep fits of the procedure ( Boyer & Haskal , 2009). As the vein thrombosis prophylaxis while hospitalized. number of patients with HCV cirrhosis increases, the Consideration of his potential for coagulopathy related proportion of TIPS procedures performed will also to his cirrhosis was noted. The decision was made to increase. Therefore, it is becoming even more impera- intubate the patient, place a Swan-Ganz catheter, and tive for clinicians to be able to recognize the complica- start him on inhaled epoprostenol. tions associated with a TIPS procedure and then be After the patient was intubated, readings from the able to respond with appropriate interventions. Swan-Ganz catheter were obtained. The readings revealed an elevated right ventricular systolic pressure Case Study and pulmonary artery pressure consistent with a diag- This case study focuses on a 52-year-old Caucasian nosis of pulmonary hypertension. Epoprostenol was man with end-stage liver disease secondary to Hepa- then delivered via aerosol by the endotracheal tube. titis C infection who presented for an elective TIPS Serial measurements were obtained from the pulmo- procedure due to refractory abdominal ascites, which nary artery catheter and the epoprostenol was able to required three paracenteses per week. The Model for be titrated off after 2 days and the patient was success- End Stage Liver Disease score is a model that helps fully extubated on Day 5. predict survival of patients with end-stage liver dis- ease. The model scores a patient based on the Pathogenesis of Hepatitis C patient’s risk of dying while waiting for a liver trans- Cirrhosis–Pulmonary Hypertension plant. Originally developed at the Mayo Clinic, the and the TIPS Procedure model currently approved by the Organ Procurement The development of cirrhosis from Hepatitis C infec- and Transplant Network and the United Network tion is thought to be a complex process with the virus for Organ Sharing has been modified to include the itself not believed to be cytopathic ( Pawlotsky , 2004). patient’s international normalized ratio, bilirubin, The exact role of the virus factors remains unclear in creatinine, and whether the patient has had dialysis the disease progression from fibrosis to cirrhosis, but twice in the past week. certain exogenous factors have significant influences At the time of the procedure, the patient’s Model for with the most important factor being alcohol con- End Stage Liver Disease score was 17. Prior to the sumption. Other factors include co-infection by HIV procedure, the patient underwent an outpatient tran- or other hepatitis viruses, diabetes, obesity, and vari- sthoracic echocardiogram that was unrevealing for any ous causes of immunosuppresion ( Pawlotsky , 2004). heart disease. The patient’s medical history was sig- The liver damage seen in HCV infection is thought nificant for diabetes mellitus and cirrhosis. Following to be mediated by inflammation and fibrosis. The the TIPS procedure, the patient was admitted to the inflammation is caused by a local immune response hepatology floor at the hospital for overnight observa- that results in portal lymphoid infiltration, necrosis, tion and discharge was planned for the next day. Over and degenerative lesions primarily composed of CD4 + the course of his stay, the patient developed shortness T cells ( Pawlotsky , 2004). Pawlotsky states that “fibro- of breath and required transfer to the medical intensive sis progression appears to result directly from chronic care unit for closer observation. The patient remained inflammation of the liver, which is associated with stable over the next several hours, requiring only 2 L chronic destruction of liver cells and local production of oxygen support via a nasal cannula when he acutely of cytokines and growth factors.” developed worsening dyspnea. Fibrosis leads to increases in collagen and other At this time, an arterial blood gas (ABG) was extracellular matrix products in the liver parenchyma. obtained and electrocardiogram (ECG) was ordered. A Throughout the entire process, the remaining hepato- prior chest x-ray demonstrated no pulmonary edema cytes are stimulated to regenerate as spherical nodules or pleural effusions, but a slightly enlarged cardiac within the fibrous covering of the liver. The overall silhouette was noted. Data obtained from the ABG effect is severely compromised delivery of blood to the revealed a partial pressure of oxygen in arterial blood hepatocytes and reciprocal reduction in secretion of (Pa o 2 ) of 60 mmHg. The ECG showed a normal sinus substances by the hepatocytes into the blood (Kumar, rhythm. The thoughts of the intensive care team were Abbas, Fausto, & Aster, 2010). that the patient was experiencing an acute decompen- Resistances to portal flow and hyperdynamic circu- sation post-TIPS related to pulmonary hypertension. lation interact in the complex process of portal 34 Copyright © 2014 Society of Gastroenterology Nurses and Associates Gastroenterology Nursing Copyright © 2014 Society of Gastroenterology Nurses and Associates. Unauthorized reproduction of this article is