Perioperative management of constrictive pericarditis with cardiac cirrhosis: A case report.

INTRODUCTION: The interactions between the heart and liver have been known for a long time, pericarditis constrictive could cause congestive hepatopathy via right-sided heart failure. Liver cirrhosis correlates with a high risk of mortality so perioperative management greatly influences outcomes. CASE PRESENTATION: An Indonesian man, 50 years old, complained of breath shortness. The patient had a history of pulmonary tuberculosis and was declared cured 30 years ago. The patient began experiencing fatigue 14 years ago, and the patient was diagnosed with constrictive pericarditis 5 years ago. Currently, the patient has an increased jugular venous pressure of 9 cmH2O and abnormal laboratory indicators, including a platelet count of 121,000/µL, albumin count of 3.41 g/L, direct bilirubin count of 0.7 mg/dL, total bilirubin count of 1.4 mg/dL, and INR of 1.4. Echocardiography revealed left ventricle hypertrophy, diastolic dysfunction, and right ventricle failure. Cardiac CT scan showed pericardial calcification. Abdominal ultrasound showed liver congestive and splenomegaly. Transient elastography showed severe fibrosis in liver and stiffness in spleen. The patient underwent pericardiectomy with CTP score of 6 and MELD of 12. The surgery was successful, and the complaint was reduced. The patient experienced an improvement in his condition and able to carry out activities well after 2 years post-surgery. DISCUSSION: The patient has no contraindications to pericardiectomy, CTP class A (5-6) and MELD score <13.5 has a low risk of mortality. CONCLUSION: CTP and MELD scores predict life expectancy in post-surgery cardiac cirrhosis patients.

Shear-wave elastography for monitoring Fontan-associated liver disease: A prospective cohort study.

The spectrum of Fontan-associated liver disease (FALD) varies from abnormal liver function tests to fibrosis and even cirrhosis. In this prospective study, we evaluated the role of shear-wave elastography (SWE) in predicting the presence of advanced FALD. Forty-eight patients (30 males, 13.9 [6-21] years) with a Fontan circulation were evaluated at 8.3 (2.1-18.7) years since the Fontan surgery. The median liver stiffness measurement (LSM) value was higher than values in normal children at 15.4 (9.5-38.7) kPa. The LSMs had a weak but significant correlation with age at the time of LSM (r = 0.25, p = 0.01) and duration post-Fontan surgery (r = 0.31, p = 0.02). It had a poor correlation with the concomitant aspartate transaminase-to-platelet ratio index (r = 0.1, p = 0.39). No difference in the elastography values between children with and without ultrasound evidence of advanced liver disease (17.7 [interquartile range, IQR: 4] vs. 16.1 [IQR: 6], p = 0.62] was observed. Further studies are required to determine the precise role of SWE as a noninvasive marker of liver fibrosis in FALD.

Hepatic encephalopathy due to cardiac cirrhosis.

Right-sided heart failure, including tricuspid regurgitation (TR), can cause cardiac cirrhosis. The pathophysiology is reduced arterial perfusion and passive congestion secondary to increased systemic venous pressure. However, hepatic encephalopathy due to cardiac cirrhosis is rare. This is the first case of hepatic encephalopathy with cardiac cirrhosis caused by persistent TR.

Clinical course of congestive hepatopathy pre/post heart transplantation.

BACKGROUND AND AIMS: Heart failure (HF) might lead to increased hepatic venous pressure, thereby impairing hepatic blood outflow and subsequently inducing congestive hepatopathy. We aimed to evaluate prevalence of congestive hepatopathy in patients undergoing heart transplantation (HTX) as well as their post-transplant course. METHODS: Patients undergoing HTX from 2015-2020 at the Vienna General Hospital were included (n = 205). Congestive hepatopathy was defined by hepatic congestion on abdominal imaging and hepatic injury. Laboratory parameters, ascites severity, and clinical events were assessed and post-HTX outcomes evaluated. RESULTS: At listing, 104 (54%) patients showed hepatic congestion, 97 (47%) hepatic injury, and 50 (26%) had ascites. Congestive hepatopathy was diagnosed in 60 (29%) patients, who showed more often ascites, lower serum sodium and cholinesterase activity, and higher hepatic injury markers. Mean albumin-bilirubin (ALBI)-score as well as (modified)-model for end-stage liver disease (MELD)-scores were higher in patients with congestive hepatopathy. Median levels of laboratory parameters/scores normalised after HTX, and ascites resolved in most patients with congestive hepatopathy (n = 48/56, 86%). The post-HTX (median follow-up 55.1 months) survival was 87% and liver-related events were rare (3%). Severe ascites, low cholinesterase, and MELD/MELD-XI were associated with ascites persistence/death 1­year after HTX. Age, male sex, and severe ascites were the only independent predictors of post-HTX mortality. Both ALBI and MELD-scores were robust indicators of post-HTX survival when measured 4 weeks after HTX (ALBI log-rank test p < 0.001; MELD log-rank test p = 0.012). CONCLUSION: Congestive hepatopathy and ascites were mostly reversible after HTX. Liver-related scores and ascites improve prognostication in patients after HTX.

Right-Sided Heart Failure and the Liver.

Currently, there are 6.2 million people with heart failure (HF) in the United States with 1 million new HF cases being diagnosed annually. Twenty to 30% of patients with acute heart failure also have liver dysfunction. The dual diagnoses of chronic heart and liver disease has significant prognostic implications.

Cirrhotic Cardiomyopathy: The Interplay Between Liver and Heart.

Cardiac vascular dysfunction was described years ago in alcohol-associated liver cirrhosis and recently became known as cirrhotic cardiomyopathy (CCM) in 2005. Cirrhotic cardiomyopathy is a specific cardiac dysfunction estimated to be prevalent in half of the liver cirrhosis patient population; it comprises a triad of impaired myocardial contractile responses to stress (systolic dysfunction), inadequate ventricular relaxation, and electrophysiological abnormalities. This review describes the various pathophysiological mechanisms connecting liver cirrhosis to the alterations seen in CCM and briefly mentions the role of the cardiovascular system in connecting the pathophysiology of hepatorenal syndrome (HRS). Insertion of the transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation exacerbates the underlying cardiac dysfunction leading to signs and symptoms of heart failure. This article also focuses on the clinical importance of diagnosing CCM and the limitations existing around traditional diagnostic criteria based on transmitral flow parameters. It highlights newer parameters proposed by the Cirrhotic Cardiomyopathy Consortium to obtain a diagnosis of CCM. Liver transplantation is the only treatment available to cure CCM.

Congenital cardiac liver cirrhosis with combined hepatocellular-cholangiocarcinoma-a case report.

Background: Cardiac liver cirrhosis secondary to Fontan procedure has been associated with hepatocellular carcinoma at a younger age. However, Fontan associated liver disease and combined hepatocellular-cholangiocarcinoma has not been previously reported. Combined hepatocellular-cholangiocarcinoma is a rare cancer that accounts for 2-5% of primary liver tumors and poses significant diagnostic and treatment challenges. This case highlights these needs and potential screening and treatment considerations. Herein we describe a case of combined hepatocellular-cholangiocarcinoma in a patient with autism, congenital heart disease, and Fontan procedure. Case Description: The patient is a 27-year-old male who presented with a liver mass detected on MRI performed in the context of a rising alpha-fetoprotein during a screening visit. Biopsy of the mass revealed a combined hepatocellular-cholangiocarcinoma which was staged as localized. Due to the COVID-19 pandemic and subsequent halt of all elective surgeries, the patient received local therapy with chemoembolization followed by pembrolizumab. The disease progressed though, and therapy was changed to gemcitabine plus cisplatin. Patient received 2 cycles of therapy, after which he and his family decided to transfer medical care to Memorial Sloan Kettering. Next generation sequencing of the tumor revealed TP53 and FGFR2 mutations. By then patient was also found to have lung metastasis. To help address the hepatocellular carcinoma, lenvatinib was added. Patient had sustainable disease control for about a year, yet eventually developed thrombocytopenia complicated by an episode of gastrointestinal bleeding. With a worsening performance status, adverse events of the treatment, and recurrent hospitalizations, a goals of care discussion with his family led to the discontinuation of active cancer therapy and patient was started on best supportive care. Patient remained in active follow-up until the time of this report and passed away less than a year from initiating best supportive care alone. Conclusions: This challenging case raises awareness towards screening and monitoring all patients with Fontan procedure for Fontan associated liver disease and liver cancers, including combined hepatocellular-cholangiocarcinoma. To the best of our knowledge, this is the first description of combined hepatocellular-cholangiocarcinoma occurring in the context of cardiac cirrhosis. The management difficulties that led to altering the goals of care, is another reminder of the dynamic nature of the care oncologists would provide.

Repeated TACE in HCC after Fontan surgery and situs viscerum inversus: A case report.

We describe the case of a 32-year-old man who developed a liver neoplasm due to previous Fontan surgery (FS) for a single ventricle anomaly and situs viscerum inversus. He was admitted to our hospital for suspected hepatocellular carcinoma during an Ultrasound (US) follow up. Computed tomography (CT) showed features of chronic liver disease and 7 cm hepatic nodule with arterial enhancement. Laboratory analyses documented preserved liver function and increased levels of alpha-fetoprotein. Trans-arterial-chemoembolization (TACE) was performed obtaining complete necrosis at 4 weeks of follow up and significant reduction of alpha-fetoprotein. The patient is currently in follow-up, being evaluated for further treatments and/or combined liver-heart transplantation. TACE is a therapeutic option for the treatment of patients with unresectable hepatocellular carcinoma (HCC) and with severe heart disease, like those submitted to FS and with also other vascular abnormalities like those correlated to situs viscerum inversus.

Transarterial chemoembolization for hepatocellular carcinoma in Fontan surgery patient.

We describe the case of a 41-year-old woman who developed a liver neoplasm due to previous Fontan surgery for a single ventricle anomaly and pacemaker implantation. She was admitted to our hospital for moderate ascites and she was affected by hepatocellular carcinoma treated by trans-arterial chemoembolization (TACE). Computed tomography showed features of chronic liver disease and 4 cm hepatic nodules with arterial enhancement. Laboratory analyses documented preserved liver function and increased levels of alpha-fetoprotein. TACE was performed obtaining complete necrosis at 4 weeks of follow up and significant reduction of alpha-fetoprotein after 2 months. The patient is currently in follow-up, being evaluated for further treatments and/or combined liver/heart transplantation. TACE is a therapeutic option for the treatment of patients with unresectable HCC and with severe heart disease, like those submitted to FS.

Liver histopathological findings in advanced heart failure: a reappraisal of cardiac cirrhosis concept.

Cardiogenic liver disease is a common yet poorly characterized complication of advanced heart failure (HF), and may impact clinical management in the setting of heart transplant evaluation. In this retrospective study, we describe clinical and histopathological features of liver injury in advanced HF, with a focus on the role of liver biopsy. Included were 45 HF patients, assessed for possible heart transplant, who underwent liver biopsy for suspected liver disease. Median duration of HF symptoms was 5 years. Most patients had stiff hepatomegaly and elevated bilirubin. Viral hepatitis (19 patients, 42.2%) was the most common cause of prior known liver disease. Sinusoidal dilatation was detected in the majority of patients (64.4%). Median necroinflammatory index was 3 and median fibrosis was 1, consistent with a small burden of histologically proven liver disease. Viral hepatitis was the only variable associated with a higher grade of necroinflammation and fibrosis. Nine of the 14 (64.3%) advanced fibrosis/cirrhosis patients had a viral hepatitis infection. Fibrosis was significantly associated with splenomegaly. The MELD score was not correlated with cardiac index. A coarse liver echo-pattern had a 29% positive and 63% negative predictive value for advanced fibrosis/cirrhosis. Severe liver disease is uncommon in patients with advanced HF in the absence of splenomegaly or primary causes of liver disease. Ultrasound data need to be carefully evaluated, as it may overstate the severity of liver disease. Liver biopsy may be needed to accurately stage liver disease before excluding patients from advanced treatment strategies.

Cirrhotic cardiomyopathy: the liver affects the heart

Cirrhotic cardiomyopathy historically has been confused as alcoholic cardiomyopathy. The key points for diagnosis of cirrhotic cardiomyopathy have been well explained, however this entity was neglected for a long time. Nowadays the diagnosis of this entity has become important because it is a factor that contributes significantly to morbidity-mortality in cirrhotic patients. Characteristics of cirrhotic cardiomyopathy are a hyperdynamic circulatory state, altered diastolic relaxation, impaired contractility, and electrophysiological abnormalities, particularity QT interval prolongation. The pathogenesis includes impaired function of beta-receptors, altered transmembrane currents and overproduction of cardiodepressant factors, such as nitric oxide, cytokines and endogenous cannabinoids. In addition to physical signs of hyperdynamic state and heart failure under stress conditions, the diagnosis can be done with dosage of serum markers, electrocardiography, echocardiography and magnetic resonance. The treatment is mainly supportive, but orthotopic liver transplantation appears to improve this condition although the prognosis of liver transplantation in patients with cirrhotic cardiomyopathy is uncertain.

Transcatheter Aortic Valve Replacement to Treat Liver Decompensation.

•Critical aortic stenosis can cause severe biventricular disease.•Painless jaundice can present secondary to severe biventricular failure.•TAVR treats high-risk critical aortic stenosis.•TAVR can reverse clinical and biochemical congestive hepatopathy.•High-risk TAVR can be performed using circulatory support.

Congestive hepatopathy.

Passive hepatic congestion may result from a variety of distinct cardiovascular conditions. Injury to the liver caused by congestion is often asymptomatic and may not be recognized clinically. Diagnosis of congestive hepatopathy is important as it has the potential to cause complications including hepatic fibrosis and development of benign and malignant liver masses. This review will summarize the pathophysiologic mechanisms of congestive hepatopathy and provide both description and examples of its multimodality imaging findings. Examples of alternative disease which may have a similar imaging appearance will be provided. Knowledge regarding the characteristic imaging findings of congestive hepatopathy will allow for its accurate identification. Reviewing both the benefits and limitations of imaging performed to evaluate congestive hepatopathy and its complications will help to avoid pitfalls and enable recommendation of appropriate next steps in diagnostic evaluation.

Anesthetic management during the first combined heart-liver transplant performed in Korea: a case report.

Herein, we describe the anesthetic management during the first combined heart-liver transplant (CHLT) performed in Korea. Though CHLT is a rare procedure, accumulating evidence suggests that it is a feasible option for patients with coexisting heart and liver failure. A 45-year-old female patient presented with severe cardiac dysfunction requiring extracorporeal membrane oxygenation (ECMO) support and secondary congestive hepatopathy. The patient underwent consecutive heart and liver transplantation using extracorporeal circulatory devices-heart transplant with cardiopulmonary bypass, and liver transplant with peripheral ECMO. In this case report, we focus on the specific anesthetic considerations for CHLT pertaining to the challenges associated with dual pathophysiology.

Characterization of ascites in cardiac cirrhosis: the value of ascitic fluid protein to screen for concurrent cardiac cirrhosis.

Abstracts Objectives: Cardiogenic ascites has been well described regarding its pathophysiology and fluid characteristics in prior literatures. However, ascites in patients with cardiac cirrhosis has not been characterized as a separate entity despite its unique pathophysiology and clinical aspects. This study aims to describe the fluid profile of ascites of cardiac cirrhosis and explore the utility of ascitic fluid protein (AFP) to predict concurrent cardiac cirrhosis. METHODS AND MATERIALS: We retrospectively selected and reviewed samples from the patients with cardiogenic ascites with and without concurrent cardiac cirrhosis. Epidemiologic characters, serum laboratory values, and fluid characteristics were directly compared between the groups. RESULTS: We analyzed 20 samples of ascitic fluid from the patients of cardiac cirrhosis and compared with 48 samples of non-cirrhotic cardiac ascites. The AFP was significantly lower in patients with cardiac cirrhosis (3.66g/dl) as compared to non-cirrhotic patients (4.31g/dl, p < .01); while there was no difference in serum-ascites albumin gradient (1.48g/dl vs. 1.47g/dl, p = .95). AFP equal to or less than 4.3g/dl predicted cirrhosis with a sensitivity of 95% and negative likelihood ratio of 0.10; the corresponding ROC curve of AFP has an AUC of 0.777, higher than AUC of other noninvasive prediction models. CONCLUSIONS: We presented the first fluid characterization of ascites in patients with cardiac cirrhosis. AFP was significantly lower than that from non-cirrhotic cardiac ascites, likely secondary to decreased serum protein level. AFP equal to or less than 4.3g/dl could be utilized to screen for concurrent cardiac cirrhosis with high sensitivity in patients with cardiogenic ascites without other predisposing factors for liver injury.

Heart Disease and the Liver: Pathologic Evaluation.

Liver injury due to acute and chronic heart failure has long been recognized. This article discusses the concepts of acute cardiogenic liver injury (ACLI) and cardiac or congestive hepatopathy (CH) along with their clinical manifestations and sequelae. Histologically, ACLI manifests as centrilobular hepatocellular necrosis, whereas CH is associated with centrilobular hepatocyte atrophy, dilated sinusoids, and perisinusoidal fibrosis, progressing to bridging fibrosis and ultimately cirrhosis. ACLI is associated with marked increases in aminotransferase levels, whereas CH is associated with a cholestatic pattern of laboratory tests. Certain cardiac medications have also been implicated as a cause of liver fibrosis.

Anesthetic management during the first combined heart-liver transplant performed in Korea: a case report / 대한마취과학회지

Herein, we describe the anesthetic management during the first combined heart-liver transplant (CHLT) performed in Korea. Though CHLT is a rare procedure, accumulating evidence suggests that it is a feasible option for patients with coexisting heart and liver failure. A 45-year-old female patient presented with severe cardiac dysfunction requiring extracorporeal membrane oxygenation (ECMO) support and secondary congestive hepatopathy. The patient underwent consecutive heart and liver transplantation using extracorporeal circulatory devices—heart transplant with cardiopulmonary bypass, and liver transplant with peripheral ECMO. In this case report, we focus on the specific anesthetic considerations for CHLT pertaining to the challenges associated with dual pathophysiology.

Transarterial embolization for pediatric hepatocellular carcinoma with cardiac cirrhosis.

We describe the case of a 15-year-old boy with a history of Fontan operation and multiple intrahepatic tumors. Computed tomography showed multiple hepatic nodules with arterial enhancement. Because hepatocellular carcinoma (HCC) was not detected on biopsies and tumor markers were normal, progress was monitored on imaging. One hepatic tumor increased greatly in size during follow up. At 15 years of age, tumor markers rose rapidly, and he had upper abdominal swelling. Therefore, transarterial embolization (TAE) was performed for the largest tumor, suspected to be a HCC due to cardiac cirrhosis. This tumor had not increased at follow up 4 months later. The patient died from hepatic failure at the age of 17 years, and HCC was diagnosed at autopsy. Although pediatric HCC is rare, its incidence is likely to increase. TAE, with or without anticancer agents, is a therapeutic option for unresectable pediatric HCC, as it is for adult HCC.

Hepato-cardiac disorders.

Understanding the mutual relationship between the liver and the heart is important for both hepatologists and cardiologists. Hepato-cardiac diseases can be classified into heart diseases affecting the liver, liver diseases affecting the heart, and conditions affecting the heart and the liver at the same time. Differential diagnoses of liver injury are extremely important in a cardiologist's clinical practice calling for collaboration between cardiologists and hepatologists due to the many other diseases that can affect the liver and mimic haemodynamic injury. Acute and chronic heart failure may lead to acute ischemic hepatitis or chronic congestive hepatopathy. Treatment in these cases should be directed to the primary heart disease. In patients with advanced liver disease, cirrhotic cardiomyopathy may develop including hemodynamic changes, diastolic and systolic dysfunctions, reduced cardiac performance and electrophysiological abnormalities. Cardiac evaluation is important for patients with liver diseases especially before and after liver transplantation. Liver transplantation may lead to the improvement of all cardiac changes and the reversal of cirrhotic cardiomyopathy. There are systemic diseases that may affect both the liver and the heart concomitantly including congenital, metabolic and inflammatory diseases as well as alcoholism. This review highlights these hepatocardiac diseases.