A CASE OF BUDD CHIARI SYNDROME IN 16 YEARS OLD MALE

Budd chiari syndrome is an uncommon disorder dened as hepatic vein outow tract obstruction, which is independent of the level and mechanism of obstruction( except pericardial disease and cardiac cirrhosis and sinusoidal obstruction syndrome)

Overview on Cardiac Cirrhosis and Congestive Hepatopathy - A Review

Cardiac cirrhosis (congestive hepatopathy) refers to a group of hepatic abnormalities that develop as a result of right-sided heart failure. Cirrhosis of the liver can be induced by any right-sided pathology that leads to right-sided heart failure, which leads to increased venous congestion and pressure in the hepatic sinusoids. Because cardiac cirrhosis might be asymptomatic or diagnosed incorrectly due to other types of liver disease, determining its prevalence is difficult. The underlying heart disease, rather than the hepatic congestion and damage, is usually the cause of death in cardiac cirrhosis. The control of the underlying cardiac disease, as well as the optimization of cardiac output, are the mainstays of congestive hepatopathy treatment. Diuresis can help with hepatic congestion, but it must be used with caution to avoid causing hepatic ischemia. Hemodynamic therapy may be able to reverse the early stages of congestive hepatitis. The widespread use of heart transplantation (HT) and considerable breakthroughs in medical and surgical treatments have drastically altered the profile of CH patients. In this overview we will be looking at the disease cause, epidemiology, diagnosis, and treatment.

Constrictive Pericarditis Leading to Cardiac Cirrhosis: A Rare Cause of Chronic Liver Disease

not available DS (Child) H J 2020; 36(1) : 71-74

Decompensated Acute Heart Failure Accompanied with Cardiac Cirrhosis and Chronic Disease Anemia in Child

Continuous heart failure can lead to complications to other systems, one of which is the hepatic system. Heart failure results in venous congestion in the portal vein so that the portal vein pressure increases, which is called portal hypertension. Portal hypertension causes fluid to pass through the walls of blood vessels and into the tissues. Management of decompensatory heart failure accompanied by comorbid disease in cardiac cirrhosis includes drugs management that does not aggravate the liver, namely angiotensin converting enzyme inhibitors and loop diuretic agents. Also, non-pharmacological management such as resting position and a diet sufficient in protein and low salt help improve the patient's general condition. This case report aims to present a decompensated heart failure accompanied by cardiac cirrhosis and anaemia of chronic disease in a child.

Abstract 16533: Heterogeneity of Fibrosis in Patients Undergoing Combined Heart-Liver Transplant

Introduction: Combined heart-liver transplant (CHLT) is indicated for patients with end-stage heart failure and concomitant irreversible liver injury. Given that liver dysfunction from congestive hepatopathy is common in patients with end-stage heart failure, it can be difficult to determine reversible from irreversible liver injury. Transjugular liver biopsy (TJLB) is frequently used to evaluate the severity of parenchymal injury, but is limited by heterogeneity of fibrosis across biopsy specimens. We sought to compare the fibrosis evaluation of the TJLB specimens as compared to the pathology of the liver explant at the time of CHLT. Methods: All CHLT cases at CSMC between 2007 and 2017 were included. Demographic and liver ultrasound (US) or abdominal computed tomography (CT) imaging was retrieved by chart review. TJLB was performed prior to transplant to determine the severity of liver fibrosis and was compared to the pathology of the liver explant. A biopsy was considered to show heterogenous fibrosis if there was at least a 2 stage difference between the predominant and secondary patterns. Results: Thirteen CHLTs were performed at our center during the study period. The median follow-up was 59 months (IQR 48-67). Mean age at transplant was 53 years (SD +/- 14.9) and 77% of patients were male. Indications for CHLT included cardiac cirrhosis (7), amyloidosis (2), HCV cirrhosis (2), and congenital heart disease (2). By imaging, 3/7 patients (43%) had US and CT evidence, 2/7 (29%) had US or CT evidence, and 2/7 (29%) had no evidence of liver nodularity US or CT. All patients diagnosed with cardiac cirrhosis had both TJLB and explant pathology for review. All TJLBs were graded as stage 4 fibrosis. Fibrosis on analysis of the liver explant was variable: stage 4 (2), stage 3-4 (3), stage 2-4 (1), and stage 1-4 (1). Thus, 2/7 patients (29%) showed heterogeneity of fibrosis in their explant as compared to their TJLB. Conclusions: We found heterogeneity of fibrosis in liver explants of patients that had stage 4 fibrosis (cirrhosis) by TJLB and underwent CHLT. Further work is needed to identify which heart transplant candidates will most benefit from CHLT.