Cardiac Cirrhosis


Article Author:
Mary Rodriguez Ziccardi


Article Editor:
Mayank Singhal


Editors In Chief:
Silvio de Melo Jr.
Vittorio Giuliano
Truptesh Kothari


Managing Editors:
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Frank Smeeks
Kristina Soman-Faulkner
Benjamin Eovaldi
Radia Jamil
Sobhan Daneshfar
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Hajira Basit
Phillip Hynes


Updated:
4/8/2019 11:11:13 PM

Introduction

Cardiac cirrhosis is a term used to include the spectrum of hepatic disorders that occur secondary to hepatic congestion due to cardiac dysfunction, especially the right heart chambers. Cardiac cirrhosis can be caused by any right sided pathology that can generate right heart failure that causes an increase in venous congestion and increase of pressure in the hepatic sinusoids. Common causes of cardiac abnormalities that can cause cardiac cirrhosis are (1) valvular disease, (2) severe pulmonary hypertension, (3) cor pulmonale, (4) biventricular heart failure (5) Pericardial diseases (6) Cardiac tamponade and (7) constrictive pericarditis. [1]

Etiology

Cardiac cirrhosis is the consequence of the hemodynamic rearrangements caused by heart failure. Right-sided heart dysfunction can due to left-sided heart failure, valvular diseases (tricuspid regurgitation, mitral stenosis) or decreases in relaxation or filling of the right sided heart as in the setting of constrictive pericarditis. It produces an increase in the preload and back pressure to the hepatic system producing hepatic congestion. Prolonged hepatic congestion eventually leads to liver cirrhosis.[2]

Epidemiology

The prevalence of cardiac cirrhosis is hard to determine because this condition can be asymptomatic or the diagnosis can be missed because of other causes of liver injury. Usually in cardiac cirrhosis, the mortality is related more to the underlying cardiac disease  rather than the hepatic congestion and injury. Age of presentation for cardiac cirrhosis is unclear as it depends on onset of decompensated heart failure. As for cardiac diseases, the risk of cardiac cirrhosis increases with age. Similarly, it is more frequent in males as they also have an increased risk of developing heart failure and subsequently cardiac cirrhosis. [3][4]

Pathophysiology

There are several mechanisms which create unbalance in the circulatory system and will cause hepatic injury. The main mechanism causing hepatic dysfunction due to heart dysfunction is either an increase in cardiac filling pressures or low cardiac output and impaired perfusion. An increase in the preload or central venous pressure due to right ventricular dysfunction may cause direct liver damage due to increasing retrograde pressure to the venous and capillaries into the liver. This generates an elevation in liver enzymes. The elevated pressure is transmitted from the right heart chambers (right ventricle and atrium) to the hepatic veins and sinusoids leading to intrahepatic edema, decreased perfusion and oxygen diffusion as well as hemorrhagic injury and modification on the hepatocyte architecture and atrophy with associated collagen deposition, and fibrosis to the hepatic veins and sinusoids.

Another mechanism that can cause hepatic injury, especially when the left side of the heart is affected, is impaired perfusion and tissue hypoxia from decreased cardiac output. This may be associated with acute hepatocellular necrosis with marked elevation of serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), lactic dehydrogenase (LDH), and prolongation of coagulation studies as thrombin and prothrombin time. [5]

History and Physical

Patients with cardiac cirrhosis are usually asymptomatic. This condition is usually suggested from abnormal liver function tests. Common symptoms are due to heart failure like shortness of breath, leg swelling, paroxysmal nocturnal dyspnea and orthopnea. When symptoms are present, these are gradual but may present abruptly in cases of acute right ventricular decompensation, constrictive pericarditis or acute decompensation of a valve disease. Jugular venous distention is also present. Symptomatic patients due to liver dysfunction typically present with abdominal pain that can be localized in right upper quadrant (secondary to hepatic capsule dilation), nausea and vomiting, jaundice, hepatomegaly, abdominal distention, and ascites. These findings are easily confused with biliary disease. Jaundice is not commonly reported. Liver injury can present in the setting of heart failure exacerbation with liver enzymes that are massively elevated after 24 to 72 hours after the cardiac decompensation.

Evaluation

At the time of presentation, the cause of liver injury and cardiac decompensation needs to be evaluated. A careful history and physical examination should be done to evaluate possible causes of liver injury like hepatitis, medications, gallbladder disease and others. An initial evaluation of liver function should also be done. The most common finding is the elevation of the total bilirubin due to increasing indirect bilirubin. This finding has been found in 70% of patients with cardiac cirrhosis, and the bilirubin levels are rarely greater than 3 mg/dl. Alteration of the synthetic function of the liver presents with prolongation of the coagulation times. Despite this, increases in the international normalized ratio are not that common but can be elevated in severe cases of ischemic injury. Serum albumin levels can be decreased in a 40% of patients. Severe elevation in LDH and ALT/LDH ratio of less than 1.5 helps to differentiate perfusion injury from other causes of acute hepatitis.[6][7][8][9]

Evaluation of causes that can explain exacerbation of heart failure is important. Cardiac enzymes and brain peptide natriuretic peptide (BNP) may be elevated. An electrocardiogram can demonstrate arrhythmias and ischemic changes as well as suggest right ventricular hypertrophy. An echocardiogram is an important test to evaluate the underlying cause of congestive hepatopathy. Echo evaluates for valvular disease, wall motion abnormalities, pulmonary artery systolic pressure, mitral inflow, atrial and ventricular size, and evaluation for pericardial effusion or constrictive pericarditis. Inferior vena cava (IVC) evaluation would help to define volume status and pressure between the right atrium and the hepatic circulation. IVC respiratory variation (normally greater than or equal to 50% narrowing during inspiration) or IVC diameter greater than or equal to 2.3 cm suggests right-sided cardiac disease with increased right atrial filling pressures. CT and MRI evaluations of cardiac and hepatic conditions also can be used to determine etiology of the clinical presentation. Due to common signs and symptoms of the biliary disease, imaging evaluation should be considered. Abdominal ultrasound for evaluation of the biliary tract and gallbladder can rule out acute obstructive disease or hepatic vein thrombosis. In the presence of ascites, paracentesis should be performed. This fluid typically reveals a high protein content, commonly > 2.5g/dl. This increased in protein can be related to hepatic lymphatic ruptured with leakage of protein-rich fluid. Serum to ascites albumin gradient (SAAG) greater than 1.1 reflects portal hypertension.

Treatment / Management

The treatment of cardiac cirrhosis is based on management of the underlying cardiac condition causing hemodynamic imbalance. Determination of the volume status and adequate management of fluids for these patients is important. Clinical features of cardiac cirrhosis may improve dramatically with adequate diuresis.  Close monitoring of cardiac output is important to prevent ischemic hepatitis. Usual management of liver cirrhosis as indicated in the setting of liver dysfunction irrespective of the etiology such as beta blocker to reduce portal hypertension, diuretics for recurrent ascites, spontaneous bacterial peritonitis prophylaxis in the setting of GI bleed etc.[10][11]

Differential Diagnosis

Differential diagnosis for cardiac cirrhosis are

  • Alcoholic liver cirrhosis
  • Primary biliary cirrhosis
  • Chronic viral hepatitis
  • Wilson disease
  • Venoocclusive disease
  • Nonalcoholic Steatohepatitis (NASH)
  • Autoimmune hepatitis
  • Ischemic hepatitis

Prognosis

The prognosis of cardiac cirrhosis depends on the prognosis of the underlying cardiac abnormality.

Complications

The complications of cardiac cirrhosis depend on the complications of the underlying cardiac abnormality and liver dysfunction.It can lead to portal hypertension, hepatic encephalopathy, variceal bleeding, recurrent ascites, hepatorenal syndrome, spontaneous bacterial peritonitis and hepatopulmonary syndrome. An advanced cases, it causes coma and death also.

Consultations

Following consultations are usually recommended.

  • Cardiology
  • Gastroenterology

Enhancing Healthcare Team Outcomes

Cardiac cirrhosis is a serious disorder with very high morbidity and mortality. The condition is best managed by a multidisciplinary team that includes a cardiologist, liver specialist, nephrologist, internist, cardiac nurses, and radiologist. The treatment of cardiac cirrhosis is based on management of the underlying cardiac condition causing hemodynamic imbalance. Determination of the volume status and adequate management of fluids for these patients is important. Clinical features of cardiac cirrhosis may improve dramatically with adequate diuresis.  Close monitoring of cardiac output is important to prevent ischemic hepatitis. Usual management of liver cirrhosis as indicated in the setting of liver dysfunction irrespective of the etiology such as beta blocker to reduce portal hypertension, diuretics for recurrent ascites, spontaneous bacterial peritonitis prophylaxis in the setting of GI bleed.

The outlook for patients with cardiac cirrhosis is guarded. If the primary condition is not managed well, the prognosis is grave. Even those who survive are often left with residual multiorgan dysfunction.[12]

 


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Cardiac Cirrhosis - Questions

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Select the correct statement about cardiac cirrhosis.



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Which finding is not usually seen in cardiac cirrhosis?



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Which of the following medications would be least useful for cardiac cirrhosis?



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Which is not a sign of cardiac cirrhosis?



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A 65-year-old female presents with orthopnea, anorexia, and bilateral lower extremity edema. Physical exam reveals cachexia, jugular venous distention, a holosystolic, high-pitched, blowing murmur, bilateral rales, and hepatomegaly. Blood work shows an elevated total bilirubin, aspartate aminotransferase, and alanine aminotransferase. What is the best treatment for the suspected condition?



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Cardiac Cirrhosis - References

References

Wells ML,Venkatesh SK, Congestive hepatopathy. Abdominal radiology (New York). 2018 Aug     [PubMed]
Koehne de Gonzalez AK,Lefkowitch JH, Heart Disease and the Liver: Pathologic Evaluation. Gastroenterology clinics of North America. 2017 Jun     [PubMed]
Houghton D,Zalewski P,Hallsworth K,Cassidy S,Thoma C,Avery L,Slomko J,Hardy T,Burt AD,Tiniakos D,Hollingsworth KG,Taylor R,Day CP,Masson S,McPherson S,Anstee QM,Newton JL,Trenell MI, The degree of hepatic steatosis associates with impaired cardiac and autonomic function. Journal of hepatology. 2019 Feb 13;     [PubMed]
Yuan W,Lu HZ,Mei X,Zhang YY,Zhang ZG,Zou Y,Wang JF,Qian ZP,Guo HY, Cardiac health in patients with hepatitis B virus-related cirrhosis. Medicine. 2019 Mar;     [PubMed]
Te H,Doucette K, Viral Hepatitis: Guidelines by the American Society of Transplantation Infectious Disease Community of Practice. Clinical transplantation. 2019 Feb 28;     [PubMed]
Ayllón MD,Ciria R,Gómez-Luque I,Rodriguez-Perálvarez M,Padial A,Luque A,López-Cillero P,de la Mata M,Briceño J, Use of Peritoneovenous Shunt for the Management of Refractory Ascites. Transplantation proceedings. 2019 Jan - Feb;     [PubMed]
Artzner T,Michard B,Besch C,Levesque E,Faitot F, Liver transplantation for critically ill cirrhotic patients: Overview and pragmatic proposals. World journal of gastroenterology. 2018 Dec 14;     [PubMed]
van Cleemput J,Sonaglioni A,Wuyts WA,Bengus M,Stauffer JL,Harari S, Idiopathic Pulmonary Fibrosis for Cardiologists: Differential Diagnosis, Cardiovascular Comorbidities, and Patient Management. Advances in therapy. 2019 Feb;     [PubMed]
Lee SK,Song MJ,Kim SH,Ahn HJ, Cardiac diastolic dysfunction predicts poor prognosis in patients with decompensated liver cirrhosis. Clinical and molecular hepatology. 2018 Dec;     [PubMed]
Lanier GM,Fallon JT,Naidu SS, Role of Advanced Testing: Invasive Hemodynamics, Endomyocardial Biopsy, and Cardiopulmonary Exercise Testing. Cardiology clinics. 2019 Feb;     [PubMed]
Ufere NN,Brahmania M,Sey M,Teriaky A,El-Jawahri A,Walley KR,Celi LA,Chung RT,Rush B, Outcomes of in-hospital cardiopulmonary resuscitation for patients with end-stage liver disease. Liver international : official journal of the International Association for the Study of the Liver. 2019 Feb 27;     [PubMed]
Hollenberg SM,Waldman B, The Circulatory System in Liver Disease. Critical care clinics. 2016 Jul     [PubMed]

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