Passive Hepatic Congestion Research Articles

Abstract 11296: Non-Invasive Assessment of Liver Stiffness in Pulmonary Hypertension Patients - A New Marker for Right Ventricular Dysfunction

INTRODUCTION - Right ventricular dysfunction (RVD) is an important indicator of a poor Pulmonary Hypertension (PH) prognosis. Cardio-hepatic interaction in left heart failure (LHF) is discreet, however, research is lacking in right heart failure (RHF). The study rationale is that PH patients can evolve to RHF with increased pressure in right chambers and passive hepatic congestion, consequently leading to an increase in liver stiffness (LS). OBJECTIVE - Assess LS in precapillary patients compared to a healthy control group. METHODOLOGY - A transversal pilot study conducted from January 2020 to April 2022 with precapillary PH patients over age 18, diagnosed by right heart catheterization. Postcapillary PH secondary to schistosomiasis, liver disease and alcoholism were excluded. Results were paired with a healthy control group. Both groups underwent two-dimensional speckle tracking with strain echocardiogram (ECO) and shear wave hepatic elastography (HE) on a Phillips ultrasound. HE measurements were taken at two points; standard (SHE), supported by literature and widely used, and a central (CHE), located near the hepatic thread. In addition, the PH group underwent laboratory exams: liver function, BNP dose, complete blood count and kidney function. Results: PH group 31 patients: 26 women (84%) and 5 men (16%); average age 51.7 (24-81 years). PH group etiology: group I (63%), group IV (22.6%) and group III (16%). Degree of dyspnea: III (74%), II (16%) and I (6%). Control group 15 individuals: 10 women (67%) and 5 men (33%): 45.7 average age (27-61 years). PH group SHE median 5.93Kpa, IQR: 5.87, versus 3.69kpa for the control group (p<0.001) with IQR 0.82kpa. While PH group CHE was 8.03Kpa: IQR 6.10Kpa (p<0,001) versus 5.15Kpa, IQR 1.60Kpa in the control group. ECO parameters in PH patients, only the right ventricle free wall strain presented a moderate correlation with SHE, Pearson coefficient of -0.427 p<0.019. There is a high correlation between SHE and CHE, Pearson coefficient 0.887 p<0.001. PH group lab results did not indicate correlation with ECO nor HE. CONCLUSION: HE can be used as a new marker for RVD in PH patients.

Fontan-associated liver disease.

Fontan-associated liver disease.

Liver magnetic resonance relaxometry can provide useful markers for the assessment of right heart failure in dilated cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Introduction In dilated cardiomyopathy (DCM) patients at risk of developing right heart failure (RHF), early depiction of congestive heart failure (CHF) is pivotal to inform about the hemodynamic status and tailor medical therapy. Purpose We hypothesized that increased liver relaxation times measured at routine cardiovascular magnetic resonance (CMR), reflecting passive hepatic congestion, may be a valuable imaging biomarker to depict CHF. Methods The study cohort included DCM patients with (n = 48) and without (n = 46) right ventricular dysfunction (RVD), defined as a right ventricular ejection fraction <35%, and >45%, respectively, and a control group (n = 40). Native T1, T2, and extracellular volume (ECV) liver values were measured on routinely acquired cardiac maps. Results DCM with RVD patients had higher C-reactive protein, troponin I and NT-pro BNP values, and worse LV functional parameters than DCM without RVD patients (all p < 0.001). T1, T2 and ECV liver values were significantly higher in DCM with, compared to DCM without, RVD patients and also compared to controls [T1: 675 ± 88ms vs. 538 ± 39ms and 540 ± 34ms; T2: 54 ± 8ms vs. 45 ± 5ms and 46 ± 4ms; ECV: 36 ± 7% vs. 29 ± 4% and 30 ± 3%, respectively (all p < 0.001)]. Gamma glutamyltranspeptidase (γGT) correlated moderately but significantly with liver native T1 (r2 =0.34), T2 (r2 =0.27), and ECV (r2 =0.23) (all p < 0.001). Using right atrial pressure (RAP > 5 mmHg), as a surrogate measure of RHF, liver native T1 yielded at ROC analysis the highest AUC (0.906), significantly higher than liver ECV (0.813), γGT (0.806), liver T2 (0.797), total bilirubin (0.737) and alkaline phosphatase (0.561) [Figure 1]. A liver native T1 value of 617 ms showed a sensitivity of 79.5% and a specificity of 91.0% in identifying RHF. Density plots to discriminate between presence and absence of RHF are demonstrated at Figure 2. Excellent intra-/inter-observer agreement was found for assessment of native T1/T2/ECV liver values. Conclusion In DCM patients, assessment of liver relaxation times acquired on a CMR exam, may provide valuable information with regard to the presence of RHF.

Assessment of Right-Sided Heart Failure in Patients with Dilated Cardiomyopathy using Magnetic Resonance Relaxometry of the Liver

In non-ischemic dilated cardiomyopathy (DC) patients at risk of developing right heart failure (RHF), early depiction of congestive heart failure (CHF) is pivotal to inform about the hemodynamic status and tailor medical therapy. We hypothesized increased liver relaxation times measured at routine cardiovascular magnetic resonance (CMR), reflecting passive hepatic congestion, may be a valuable imaging biomarker to depict congestive heart failure. The study cohort consisted of DC patients with LV dysfunction (i.e., ejection fraction <35%) with (n = 48) and without (n = 46) right ventricular dysfunction (RVD), defined as a right ventricular ejection fraction <35%, and >45%, respectively, and a control group (n = 40). Native T1, T2, and extracellular volume (ECV) liver values were measured on routinely acquired cardiac maps. DC+RVD patients had higher C-reactive protein, troponin I and NT-pro BNP values, and worse LV functional parameters than DC-RVD patients (all p <0.001). T1, T2 and ECV Liver values were significantly higher in DC+RVD compared to DC-RVD patients and controls, that is, T1: 675 ± 88 ms verses 538 ± 39 ms and 540 ± 34 ms; T2: 54± 8 ms versus 45 ± 5 ms and 46 ± 4 ms; ECV: 36 ± 7% versus 29 ± 4% and 30 ± 3% (all p <0.001). Gamma-glutamyltranspeptidase (GGT) correlated moderately but significantly with native T1 (r2 = 0.34), T2 (r2 = 0.27), and ECV liver (r2 = 0.23) (all p <0.001). Using right atrial (RA) pressure, as surrogate measure of RHF (i.e., RA pressure >5 mm Hg), native T1 liver yielded at ROC analysis the highest area under the curve (0.906), significantly higher than ECV liver (0.813), GGT (0.806), T2 liver (0.797), total bilirubin (0.737) and alkaline phosphatase (0.561)(p = 0.04). A T1 value of 617 ms yielded a sensitivity of 79.5% and specificity of 91.0% to depict RHF. Excellent intra-/inter-observer agreement was found for assessment of native T1/T2/ECV liver values. In conclusion, in DC patients, assessment of liver relaxation times acquired on a cardiovascular magnetic resonance exam, may provide valuable information with regard to the presence of RHF.

Abstract 17140: Impact of Atrial Fibrillation on the Outcomes of Heart Failure With Preserved Ejection Fraction- A National Inpatient Sample Based Study

Introduction: Atrial fibrillation (AF) adversely affect the outcomes in the patients of heart failure (HF) with reduced ejection fraction, however there are limited data exploring such an association in HF with preserved ejection fraction (HFpEF). Hypothesis: AF is associated with worse outcomes in HFpEF. Methods: We included all the patients with the primary diagnosis of HFpEF from the national inpatient sample (NIS) database (2012-2014) using ICD-9 codes. Exposure of interest was AF. Primary outcome was in-hospital mortality and secondary outcomes were rates of sudden cardiac arrest (SCA), syncope, cardiogenic shock, embolic stroke, acute myocardial infarction (AMI), acute kidney injury (AKI), passive hepatic congestion, ventricular fibrillation (V fib) and flutter, ventricular assist device (VAD), AICD, cardiac resynchronization therapy (CRT), intra-aortic balloon placement (IABP) placement and heart transplantation. Hospitalization cost was also studied. Results: Our study cohorts included 26,51,970 patients of HFpEF with AF and 37,44,101 patients of HFpEF without AF. AF cohort had more numbers of older patients and less female representation. In-hospital mortality was more in AF cohort. Similarly, the odds of SCA, cardiogenic shock, embolic stroke, passive hepatic congestion, Vfib and flutter, AICD and CRT placement were higher in AF cohort. The odds of syncope, AMI and AKI were lower in AF cohort as compared to non-AF cohort. While the odds of heart transplantation and VAD and IABP use remained comparable between the study cohorts, AF cohort incurred greater of cost of hospitalization. Conclusion: AF in HFpEF patients is associated with increased in-hospital mortality and cardiogenic shock and should be aggressively treated for improved outcomes.

A window into portal hemodynamics in adult fontan patients?

BackgroundFontan circulation alters portal venous hemodynamics, causing chronic passive hepatic congestion and fibrosis. This congestion increases liver stiffness (LS) leading to overestimates of liver fibrosis as measured by ultrasound shear wave elastography (SWE) of the liver. We evaluated whether Fontan circulation has a similar effect on spleen stiffness (SS) and SS/LS ratio as measured by SWE. MethodsWe retrospectively compared the SS of adult Fontan patients to age and gender matched, control patients without congenital heart disease. We correlated SS measurements to LS measurements and also performed a limited subgroup analysis of SS in Fontan patients with various manifestations of Fontan Associated Liver Disease. ResultsSS in Fontan patients was similar to healthy controls (1.43 vs. 1.36 m/s, p = 0.26). LS was elevated in 78% of the Fontan patients (mean 1.68 m/s, SD 0.31, 95% CI 1.53–1.85). The correlation between LS and SS was modest (Pearson's correlation coefficient, r = 0.5) but did not reach statistical significance (p = 0.06). The mean SS/LS ratio was 0.85 (95% CI 0.77–0.94). ConclusionBased on our study cohort, SS in Fontan patients is similar to age and gender matched control patients without congenital heart disease. The SS/LS ratio, however, is frequently less than 1, which is lower than that reported in both healthy patients and those with other forms of non-cardiac liver disease. SS and SS/LS ratio may be a useful indicator of portal hemodynamics in Fontan patients.

Between a Lift and a Hard Place

Between a Lift and a Hard Place

3182 Spontaneous Bacterial Peritonitis in Cardiac Ascites: A Rare but Deadly Occurrence

INTRODUCTION: Spontaneous bacterial peritonitis (SBP) is frequently described in cirrhotic patients who develop infected ascitic fluid. The phenomenon of SBP in cardiac ascites is rare. Here we present a unique case of a patient who was admitted for advanced cardiorenal syndrome in the setting of a gastroenteritis that likely promoted a bacterial translocation resulting in SBP. CASE DESCRIPTION/METHODS: An 85M presented from a nursing facility with lethargy, confusion and non-bloody diarrhea. Medical history was significant for congestive heart failure (CHF) and chronic kidney disease. No history of alcohol use. Upon arrival to the hospital, he was noted to be confused with a mildly distended abdomen, asterixis and two plus lower extremity pitting edema. The patient was admitted for a CHF exacerbation and metabolic encephalopathy. Initial labs revealed a BNP 3915, WBC 11,500, BUN 89, creatinine 3.5, total bilirubin 2, AST 22, ALT 9, and INR 1.27. An abdominal CT scan showed a moderate amount of intraperitoneal fluid. A diagnostic paracentesis was performed. The aspirated fluid was dark yellow and opaque with a serum ascites albumin gradient of 1.9 g/dL, total fluid protein 3.3 g/dL, 1,115 PMNs cells/mm3, 49% polymorphonuclear leukocytes. To much surprise, these findings demonstrated cardiac ascites resulting in SBP. There was no evidence of cirrhosis. Antibiotic treatment was initiated. Unfortunately, the patient exhibited a rapid decline renal function, and expired shortly afterwards. DISCUSSION: Due to the liver's extensive vascular supply it is prone to hemodynamic disturbances which can result in passive hepatic congestion from right sided heart failure. Recent studies have supported the diagnostic utility of serum BNP levels to distinguish ascites from cirrhosis or heart failure. It has been generally well accepted that the low occurrence of SBP in cardiac ascites may be related to the protein rich ascitic fluid that has both opsonic and bactericidal activity. Interestingly, the “gut hypothesis” suggests that intestinal hypoperfusion and congestion can lead to altered gut morphology, permeability, and the growth of microbiota that may ultimately stimulate bacterial translocation and endotoxin release. This case tends to shed light on a few quintessential points for clinicians to be aware of, including the potential intersection between the microbiota and metabolic effects of CHF and the necessity to lower the diagnostic threshold for SBP cardiac ascites in patient's presenting for a CHF exacerbation.

Pediatric Fontan associated liver disease: Non-invasive evaluation with serologic markers and acoustic radiation force impulse (ARFI) elastography

Hepatic fibrosis is a significant complication in adult Fontan patients suggesting development as a function of time since the surgery. Children with Fontan circulation are not routinely assessed for development of liver disease. We aimed to evaluate the effectiveness of serologic biomarkers and acoustic radiation force impulse (ARFI) elastography to detect liver disease in pediatric Fontan patients. Patients ≥1 year after Fontan operation prospectively had hepatic US with acoustic radiation force impulse and laboratory testing. Clinical cardiac data (echocardiograms, cardiac catheterizations) were reviewed. Statistical analysis was performed using Pearson's correlation coefficient, Wilcoxon rank-sum test and Kruskal-Wallis test. Forty patients were enrolled with median age of 11 years and median time since Fontan of 6.5 years. Platelet count negatively correlated with years since Fontan (p < 0.000). Thrombocytopenia was noted in 15% of patients with the lowest platelet count of 78 K/cu mm, in a patient >10 years from the Fontan (DORV) operation. Alanine transaminase (ALT, p = 0.034) and aspartate aminotransferase (AST, p = 0.009) were higher in patients with Extracardiac Conduit Fontan compared to those with Extracardiac Pericardial and Lateral Fontan operations. Heterogeneous echotexture on liver ultrasound correlated with years since Fontan (p = 0.022), however all acoustic radiation force impulse values were elevated (>1.34 m/s) and did not correlate with age, years since Fontan, labs or imaging. FibroSure values did not correlate with years since Fontan. This suggests that ARFI may be elevated due to passive hepatic congestion, limiting its value in this patient population. Additional testing is necessary to identify reliable non-invasive screening modalities for hepatic fibrosis in Fontan patients. Our study is the largest pediatric study to evaluate ARFI in patients after the Fontan operation and showed increased shear wave speed for all patients with no correlation with time since palliation. Decreasing platelet count may indicate the development of liver fibrosis.

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.

910 - Passive Hepatic Congestion Leads to Fibrosis Through Sinusoidal Endothelial Cell Responses to Mechanical Forces that Lead to Generation of Neutrophil Extracellular Traps (NETS)

910 - Passive Hepatic Congestion Leads to Fibrosis Through Sinusoidal Endothelial Cell Responses to Mechanical Forces that Lead to Generation of Neutrophil Extracellular Traps (NETS)

Imaging Findings of Congestive Hepatopathy.

Congestive hepatopathy (CH) refers to hepatic abnormalities that result from passive hepatic venous congestion. Prolonged exposure to elevated hepatic venous pressure may lead to liver fibrosis and cirrhosis. Liver dysfunction and corresponding clinical signs and symptoms typically manifest late in the disease process. Recognition of CH at imaging is critical because advanced liver fibrosis may develop before the condition is suspected clinically. Characteristic findings of CH on conventional images include dilatation of the inferior vena cava and hepatic veins; retrograde hepatic venous opacification during the early bolus phase of intravenous contrast material injection; and a predominantly peripheral heterogeneous pattern of hepatic enhancement due to stagnant blood flow. Extensive fibrosis can be seen in chronic or severe cases. Hyperenhancing regenerative nodules that may retain hepatobiliary contrast agents are often present. Magnetic resonance (MR) elastography can show elevated liver stiffness and may be useful in evaluation of fibrosis in CH because it can be incorporated easily into routine cardiac MR imaging. Preliminary experience with MR elastography suggests its future use in initial evaluation of patients suspected of having CH, for monitoring of disease, and for assessment after therapy. To facilitate appropriate workup and treatment, radiologists should be familiar with findings suggestive of CH at radiography, ultrasonography, computed tomography, MR imaging, and MR elastography. In addition, knowledge of underlying pathophysiology, comparative histologic abnormalities, and extrahepatic manifestations is useful to avoid diagnostic pitfalls and suggest appropriate additional diagnostic testing. (©)RSNA, 2016.

Vacuolar myopathic lesion in a Holstein-Friesian dairy cow affected with bovine dilated cardiomyopathy: a preliminary report

Occurrence of vacuolar myopathy caused by factors other than inherited glycogen storage condition is uncommon in domestic animals. This preliminary report describes the detection of a vacuolar myopathic lesion in a 13-year-old Holstein- Friesian dairy cow which showed an 8-day history of cardiac disease. Gross inspection after slaughter revealed edema in a variety of tissues, dilatation and hypertrophy of the cardiac ventricles, and hepatic congestion. Histopathology revealed degenerative myocardial lesions and passive hepatic congestion, which were identical to those previously documented in cases of bovine dilated cardiomyopathy. Skeletal muscles represented a vacuolar myopathic lesion characterized by the presence of distinctive vacuoles in atrophic myofibers, which occurred singly in the central zone of each myofiber on transverse sections and showed an irregularly elongated appearance on longitudinal sections. Such vacuoles frequently contained a small amount of weakly eosinophilic, granular, debris-like, or amorphous substances, displaying a feature of autophagic vacuoles. These vacuolated myofibers were more prominently observed in some muscle fascicles of the longissimus thoracis muscle than other muscles examined. The vacuolar myopathic lesion recognized in this cow was similar to some degree to that of autophagic vacuolar myopathy in man. Further studies are necessary to clarify a causal relationship between this vacuolar myopathic lesion and the disorder of bovine dilated cardiomyopathy.

Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces.

Chronic passive hepatic congestion (congestive hepatopathy) leads to hepatic fibrosis; however, the mechanisms involved in this process are not well understood. We developed a murine experimental model of congestive hepatopathy through partial ligation of the inferior vena cava (pIVCL). C57BL/6 and transgenic mice overexpressing tissue factor pathway inhibitor (SM22α-TFPI) were subjected to pIVCL or sham. Liver and blood samples were collected and analyzed in immunohistochemical, morphometric, real-time polymerase chain reaction, and western blot assays. Hepatic fibrosis and portal pressure were significantly increased after pIVCL concurrent with hepatic stellate cell (HSC) activation. Liver stiffness, as assessed by magnetic resonance elastography, correlated with portal pressure and preceded fibrosis in our model. Hepatic sinusoidal thrombosis as evidenced by fibrin deposition was demonstrated both in mice after pIVCL as well as in humans with congestive hepatopathy. Warfarin treatment and TFPI overexpression both had a protective effect on fibrosis development and HSC activation after pIVCL. In vitro studies show that congestion stimulates HSC fibronectin (FN) fibril assembly through direct effects of thrombi as well as by virtue of mechanical strain. Pretreatment with either Mab13 or Cytochalasin-D, to inhibit β-integrin or actin polymerization, respectively, significantly reduced fibrin and stretch-induced FN fibril assembly. Chronic hepatic congestion leads to sinusoidal thrombosis and strain, which in turn promote hepatic fibrosis. These studies mechanistically link congestive hepatopathy to hepatic fibrosis.

What does the liver tell us about the failing heart?

This editorial refers to ‘Liver function abnormalities, clinical profile, and outcome in acute decompensated heart failure’, by M. Nikolaou et al. , doi:10.1093/eurheartj/ehs332 The high metabolic activity of the liver results in a high perfusion rate of ∼1 mL/g/min. Under resting conditions, this is about a quarter of the bodýs total blood supply. The oxygen-rich blood of the hepatic artery contributes to about a quarter of the total liver perfusion that may rise substantially under conditions of excessive oxygen demand. The complex blood supply makes the liver extraordinarily vulnerable to acute circulatory disturbances. Both the severity and the pattern of hepatic injury depend on the relative contribution of passive congestion and diminished perfusion.1 Increased central venous pressure results in passive hepatic congestion and causes elevations of alkaline phosphatase (AP), γ-glutamyltransferase (GGT), and direct and indirect serum bilirubin. This ‘congestive hepatic injury’ is known as nutmeg liver on pathology. Decreased cardiac output with impaired organ perfusion is associated with acute centrilobular (zone 3 of the acinus) hepatocellular damage and necrosis. ‘Hepatic ischaemic injury’ results in elevations in serum aminotransferases ( Figure 1 ).2,3 Figure 1 Haemodynamic disturbances in heart failure and mechanisms resulting in different patterns of elevated liver enzymes. Congestive hepatic injury (left panel) and ischaemic hepatic injury (right panel). In contrast to acute perfusion abnormalities, prolonged and chronic haemodynamic disturbances may result in bridging fibrosis, ‘cardiac’ cirrhosis, and impaired hepatic function with decreased synthesis of coagulation factors and albumin. Hepatic congestion occurs without particular symptoms in most cases. Some patients may suffer from jaundice, stretching of the liver capsule with right upper quadrant discomfort, and ascites. Rare cases of congestive heart failure (HF) associated with fulminant hepatic failure have been reported. However, most such cases occur in the setting of superimposed cardiogenic shock and consecutive hepatic ischaemic injury.4 …

Liver Abnormalities in Cardiac Diseases and Heart Failure

Heart failure (HF) is characterized by the inability of systemic perfusion to meet the body's metabolic demands and is usually caused by cardiac pump dysfunction and may occasionally present with symptoms of a noncardiac disorder such as hepatic dysfunction. The primary pathophysiology involved in hepatic dysfunction from HF is either passive congestion from increased filling pressures or low cardiac output and the consequences of impaired perfusion. Passive hepatic congestion due to increased central venous pressure may cause elevations of liver enzymes and both direct and indirect serum bilirubin. Impaired perfusion from decreased cardiac output may be associated with acute hepatocellular necrosis with marked elevations in serum aminotransferases. Cardiogenic ischemic hepatitis ("shock liver") may ensue following an episode of profound hypotension in patients with acute HF. We discuss pathophysiology and identification of liver abnormalities that are commonly seen in patients with HF.

"Playboy Bunny" Sign of Congestive Heart Failure

In emergency, ultrasound has been widely used as a noninvasive and effective examination to evaluate congestive heart failure. We highlight “Playboy Bunny” sign as a reliable marker and an important clue to the diagnosis of passive hepatic congestion, caused by congestive heart failure.

Congestive Heart Failure in 6 African Grey Parrots (Psittacus e erithacus)

Six African grey parrots (Psittacus e erithacus) were diagnosed with cardiomyopathy and congestive heart failure based on gross and microscopic findings. Ages ranged from 15 days to 8 years, and 5 of 6 parrots were either neonates or juveniles at the time of diagnosis. Two neonates and 2 juveniles came from the same breeding aviary; the 2 juveniles were born to the same breeding pair. The 2 other parrots were kept as pets. Clinical signs included distention of the coelomic cavity (4 of 6), rales (3 of 6), weakness (4 of 6), bradyarrhythmia (1 of 6), growth retardation (1 of 6), crop stasis (1 of 6), and regurgitation (1 of 6). Three parrots were euthanized and 3 died. Gross findings included cardiomegaly due to biventricular, right-, or left-sided cardiomyopathy (6 of 6); coelomic effusion (6 of 6); whitish or yellow foci in the liver (6 of 6); atrophy of the liver (particularly, the left lobe; 5 of 6); reddened or grey lungs (5 of 6); subcutaneous edema (2 of 6); hydropericardium (1 of 6); and bilateral thyroid gland enlargement (1 of 6). Relevant microscopic findings included passive hepatic congestion (6 of 6) and pulmonary congestion (2 of 6), lymphocytic thyroiditis (2 of 6), and diffuse thyroid follicular hyperplasia (2 of 6). Microscopically, the heart was unremarkable (2 of 6) or had mild lymphocytic myocarditis (2 of 6), mild multifocal cytoplasmic vacuolation of cardiomyocytes (2 of 6), mild lymphocytic myocardial (Purkinje cell) ganglioneuritis (1 of 6), and mild multifocal interstitial fibrosis and nuclear hypertrophy of cardiomyocytes (1 of 6). One parrot had concurrent proventricular dilatation disease (systemic ganglioneuritis). The cause of cardiomyopathy in these parrots was not determined.

Hepatocellular carcinoma in two patients with cardiac cirrhosis

Hepatocellular carcinoma has been commonly associated with multiple etiologic factors including hepatitis B and C, alcoholic liver disease, and more rarely congenital metabolic liver diseases. 'Cardiac cirrhosis' is the cirrhosis resulting from prolonged passive liver venous congestion secondary to right-sided congestive heart failure; hepatocellular carcinoma is a rarely reported outcome. In this study we present two female patients with congenital heart defects treated with the Fontan procedure who survived into their third decade, and developed hepatocellular carcinoma in the setting of cardiac cirrhosis. The Fontan procedure diverts blood from the inferior vena cava and superior vena cava to the pulmonary arteries, thereby increasing survival in infants born with a single effective ventricle. As such patients live longer, however elevated pulmonary and right-sided heart pressures cause chronic passive liver congestion and eventual cardiac cirrhosis. The two patients in this study had no risk factors for hepatocellular carcinoma other than cardiac cirrhosis secondary to their prolonged survival after their Fontan procedure. In conclusion, we suggest that cardiac cirrhosis may be a risk factor for developing hepatocellular carcinoma and recommend close follow-up and hepatocellular carcinoma screening for patients with known right heart failure and passive hepatic congestion.

Bilateral Sequential Lung Transplantation

Bilateral Sequential Lung Transplantation