Modification of cardiac function in cirrhotic patients with and without ascites

OBJECTIVES: Abnormalities in cardiac function have been reported in liver cirrhosis, suggesting a latent cardiomyopathy in these patients. In this study we investigated cardiac function in cirrhotic patients and in controls. METHODS: A total of 20 cirrhotic patients without previous or ongoing ascites, 20 cirrhotic patients with moderate-to-severe ascites, and 10 healthy controls were studied by two-dimensional Doppler echocardiography. Cardiac dimensions and left and right ventricular function were evaluated. The left ventricular geometric pattern was calculated according to Ganau's criteria. Diastolic function was evaluated by the peak filling velocity of E wave and A wave, E/A ratio, and deceleration time of E wave. The pulmonary systolic arterial pressure was also estimated in patients with tricuspid insufficiency. RESULTS: Right and left atrium and right ventricle diameters were significantly enlarged in cirrhotic patients versus controls. E/A ratio was decreased (p < 0.05) in patients with ascites (0.9 +/- 0.2) versus those without ascites (1.3 +/- 0.4) and controls (1.3 +/- 1). The estimated pulmonary systolic arterial pressure was slightly elevated in patients with ascites (35 +/- 5 mm Hg, six patients) versus those with no ascites (28 +/- 5, 10 patients) and controls (27 +/- 8, 6 controls, analysis of variance, p < 0.05). The pattern of left ventricular geometry was normal in the majority of patients. Nitrite and nitrate levels were increased in cirrhotics irrespective of the presence of ascites. CONCLUSIONS: Liver cirrhosis is associated with enlarged right cardiac chambers. Diastolic dysfunction and mild pulmonary hypertension are evident in cirrhotic patients with ascites. These changes do not depend on variations in the left ventricular geometry.     

Non-cirrhotic portal hypertension versus idiopathic portal hypertension

Portal hypertension occurs in a number of disorders other than cirrhosis and they are collectively called non-cirrhotic portal hypertension (NCPH). The common causes of NCPH include idiopathic portal hypertension (IPH), non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous thrombosis (EHPVT). Other causes include schistosomiasis, hepatic venous outflow tract obstruction, veno-occlusive disease and congenital hepatic fibrosis. Patients with IPH and EHPVT present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation and jaundice due to portal biliopathy. The diagnosis is usually made by abdominal ultrasound, upper gastrointestinal endoscopy, normal liver function tests and normal liver histology. Variceal bleeding in NCPH has lower mortality as compared with cirrhosis because of better liver functions in NCPH. Treatment for NCPH includes primary prophylaxis for variceal bleeding and prevention of repeat bleeding using drugs like beta-blockers, endoscopic sclerotherapy and endoscopic band ligation of varices. In patients with uncontrolled variceal bleeding or symptomatic hypersplenism, porto-systemic shunt surgery or splenectomy are required.     

Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis

BackgroundThe knowledge of natural history of patients with portal hypertension (PH) not due to cirrhosis is less well known than that of cirrhotic patients.AimTo describe the clinical presentation and the outcomes of 89 patients with non-cirrhotic PH (25 with non-cirrhotic portal hypertension, INCPH, and 64 with chronic portal vein thrombosis, PVT) in comparison with 77 patients with Child A cirrhosis.MethodsThe patients were submitted to a standardized clinical, laboratory, ultrasonographic and endoscopic follow-up. Variceal progression, incidence of variceal bleeding, portal vein thrombosis, ascites and survival were recorded.ResultsAt presentation, the prevalence of varices, variceal bleeding and ascites was similar in the 3 groups. During follow-up, the rate of progression to varices at risk of bleeding (p < 0.0001) and the incidence of first variceal bleeding (p = 0.02) were significantly higher in non-cirrhotic then in cirrhotic patients. A PVT developed in 32% of INCPH patients and in 18% of cirrhotics (p = 0.02).ConclusionsIn the patients with non-cirrhotic PH variceal progression is more rapid and bleeding more frequent than in cirrhotics. Patients with INCPH are particularly prompt to develop PVT. This observational study suggests that the management of patients with non-cirrhotic PH should take into consideration the natural history of portal hypertension in these patients and cannot be simply derived by the observation of cirrhotic patients.     

Immunological profile of patients with non-cirrhotic portal fibrosis

The aetiopathogenesis of non-cirrhotic portal fibrosis (NCPF), a common cause of portal hypertension in India, is not known. To study the immune status of NCPF patients and to see whether immunological mechanisms have a role to play, humoral and cell-mediated immunological studies were carried out in 43 patients with NCPF and compared with equal number of matched healthy controls and 31 patients with compensated liver cirrhosis. Serum immunoglobulin A (IgA) and complement (C3, C4) levels were significantly (P less than 0.001) lower in NCPF patients compared with controls and cirrhotics. There was no significant difference between the total or the relative concentration of the immunoglobulins and complements between NCPF patients and healthy controls, but, in patients with cirrhosis, concentration of all the immunoglobulins was higher. The cutaneous response to dinitrochlorobenzene was poorer in patients with NCPF, but the difference between cirrhotics and controls was not significant. A decrease in the suppressor/cytotoxic (T8) phenotype of lymphocytes in the peripheral blood and an increase in the ratio of helper/inducer (T4) and T8 lymphocytes was seen in patients with NCPF and cirrhosis. Although these results indicate definite immunological abnormalities in NCPF patients, their role in the pathogenesis of NCPF remains to be investigated.     

Measurement and correlation of wedged hepatic, intrahepatic, intrasplenic and intravariceal pressures in patients with cirrhosis of liver and non-cirrhotic portal fibrosis.

In order to examine the relationship of various haemodynamic parameters in two different liver diseases, 10 patients with cirrhosis of liver and 14 patients with non-cirrhotic portal fibrosis were studied. In cirrhotics, mean (+/- SD) wedged hepatic (25.8 +/- 6.4 mmHg), intrahepatic (24.5 +/- 6.2 mmHg) and intrasplenic (25.0 +/- 5.6 mmHg) pressures correlated significantly (p less than 0.001) with intravariceal (25.2 +/- 6.7) pressure measurements. In patients with NCPF, mean (+/- SD) wedged hepatic (9.1 +/- 3.7 mmHg) and intraphepatic (15.4 +/- 5.8 mmHg) pressures were significantly (p less than 0.01) lower than the intrasplenic (24.5 +/- 4.2 mmHg) and intravariceal (23.96 +/- 5.6 mmHg) pressures. Two independent pressure gradients, one between intrasplenic and intrahepatic pressure (8.9 +/- 6.5 mmHg) and another between intrahepatic and wedged hepatic venous pressure (6.2 +/- 5.6 mmHg) were seen in non-cirrhotic portal fibrosis patients, indicating the likelihood of both pre- and perisinusoidal resistance to flow of portal venous blood in these patients. A highly significant (p less than 0.001) correlation between intravariceal and intrasplenic pressures was found in patients with cirrhosis of liver (r = 0.93), as well as in patients with non-cirrhotic portal fibrosis (r = 0.85). No correlation was found between the size of oesophageal varices and wedged hepatic and intrahepatic pressures. Patients with grade 4 varices had significantly higher intravariceal (p less than 0.01) and intrasplenic (p less than 0.05) pressure than patients with grade 2 varices. It can be concluded that intravariceal pressure is representative of portal pressure in patients with cirrhosis of liver as well as in non-cirrhotic portal fibrosis patients and it can be recommended as the single haemodynamic investigation in patients with portal hypertension and oesophageal varices.     

Gastric secretion in cirrhosis and non-cirrhotic portal fibrosis

Studies on basal and pentagastrin-stimulated gastric secretion were carried out in 20 patients with cirrhosis, 20 with non-cirrhotic portal fibrosis (NCPF) and 20 control subjects. There was no significant difference in the basal volume and acid output between the three groups. However, maximal volume and acid output were significantly lower in patients with cirrhosis and NCPF compared with the control group. There was no correlation between the acid secretion and the degree of hepatocellular dysfunction in patients with cirrhosis. Moreover, the gastric hyposection was as marked in the patients with NCPF as in those with cirrhosis, although the former did not suffer from any hepatic decompensation. It is concluded that gastric hyposecretion is not due to a derangement of hepatocyte function but may be secondary to portal hypertension and collateral circulation.     

Circulating endogenous cannabinoid anandamide and portal, systemic and renal hemodynamics in cirrhosis.

BACKGROUND: Endocannabinoids may participate in the homeostasis of arterial pressure. Recently, anandamide, the most extensively studied endocannabinoid, has been proposed as a key mediator in the peripheral arterial vasodilation of cirrhosis. OBJECTIVES: To determine if circulating levels of anandamide are related to the extent of the peripheral arterial vasodilation, the severity of portal hypertension and the degree of liver and renal dysfunction of patients with cirrhosis. METHODS: Plasma levels of anandamide and several systemic, portal and renal hemodynamic parameters were determined in 18 patients with cirrhosis and eight healthy subjects (control group). RESULTS: Plasma levels of anandamide were elevated in patients compared to the control group (P<0.05), nevertheless, no differences between patients with ascites and well-compensated patients were found. There was no correlation between anandamide concentration and arterial pressure, cardiac output and systemic vascular resistance, Child-Pugh's score, portal pressure, renal vascular resistance, plasma renin activity or plasma aldosterone concentration. CONCLUSIONS: Circulating levels of anandamide are increased in cirrhotic patients. However, this elevation was unrelated to the extent of arterial vasodilation, the severity of portal hypertension or the degree of hepatic and renal dysfunction. Although a local hormonal action cannot be excluded, our results do not support a relevant contribution of this system in the hemodynamic disturbance of cirrhosis.     

Variceal pressure is a strong predictor of variceal haemorrhage in patients with cirrhosis as well as in patients with non-cirrhotic portal hypertension

BACKGROUND: Variceal pressure is a strong predictor for a first variceal bleed in patients with cirrhosis. AIMS: To evaluate whether variceal pressure is also a determinant of the risk of a first variceal bleed in patients with non-cirrhotic portal hypertension. METHODS: Variceal pressure was measured non-invasively in 25 patients with non-cirrhotic portal hypertension and large varices while receiving a stable therapeutic regimen. Factors predictive of bleeding were compared with those observed in 87 cirrhotics. RESULTS: The one year incidence of variceal bleeding was 32% (n=28) for the cirrhotic and 20% (n=5) for the non-cirrhotic patients. There was no difference in factors predicting the risk of bleeding between the groups, except for variceal pressure. For the same level of variceal pressure, the risk of variceal bleeding was lower in patients with non-cirrhotic portal hypertension. Multiple logistic regression analysis revealed the following variables as having a significant predictive power: variceal pressure (p=0.0001), red spots (p=0.004), and the time interval between the first observation of the varices and the moment of variceal pressure measurement (p=0. 0046). For the non-cirrhotics the risk of bleeding increased with higher Child-Pugh score (p=0.0024); this was not the case for the cirrhotic patients (p=0.9521). CONCLUSION: Variceal pressure is a major predictor of variceal bleeding in patients with cirrhosis as well as in patients with non-cirrhotic portal hypertension. The risk of bleeding in non-cirrhotics is less than in cirrhotics for the same level of variceal pressure. In patients with non-cirrhotic portal hypertension the risk of variceal bleeding increases more with advancing disease.     

肝硬化门脉高压症患者心脏功能影响因素及其状况评价*

目的 研究肝硬化门脉高压症患者心脏功能状态,探讨其与肝功能的关系,并分析影响左心室舒张功能不全的相关危险因素。方法 我院消化科住院的肝硬化门脉高压症患者126例,其中 Child-Pugh A级76例、B级41例、C级9例,MELD分级≤9分64例和>9分62例。行心电图和超声心动图（包括脉搏波多普勒和组织多普勒成像）检测。采用回归分析影响舒张功能不全的相关因素。结果 在126例患者中,有1例（0.8%）被诊断为左心收缩功能不全,77例（61%）被诊断为左心室舒张功能不全,心电图异常率为65%,其中QTc延长发生率为41%;不同Child-Pugh分级和MELD评分患者左心室舒张功能不全、心电图异常率和QTc延长发生率差异无统计学意义（P>0.05）;Logistic回归分析发现年龄（OR=1.163,95%CI：1.086~1.244）和心率（OR=1.106,95%CI：1.018~1.201）是影响舒张功能不全的相关危险因素。结论 肝硬化门脉高压症患者心功能改变主要以舒张功能减退和电生理异常为主,而与肝病严重程度和大量腹水并无显著相关性。对于年长和心率增快的肝硬化门脉高压症患者,要加强心脏舒张功能的评估,尽早诊断和及时干预可能能改善肝硬化患者的预后。     

Duplex Doppler ultrasound of the ligamentum teres and portal vein: A clinically useful adjunct in the evaluation of patients with known or suspected chronic liver disease or portal hypertension

The prevalence and potential value of the detection of signs of portal hypertension by duplex Doppler ultrasound (DDU) of the ligamentum teres and portal vein in patients with known or suspected chronic liver disease and/or portal hypertension was studied in 136 consecutive patients undergoing clinical assessment including that of liver histopathology. Portal hypertension was considered to be present when any of the following DDU signs, previously demonstrated to be specific for portal hypertension, were present: an enlarged and/or patent para-umbilical vein, portal vein obstruction or hepatofugal flow in the portal vein. Of 123 patients with parenchymal liver disease, eighty-three had cirrhosis and, of these, portal hypertension was detected on DDU criteria in 86% of alcoholic cirrhotics and 67% of non-alcoholic cirrhotics. Of the 42 patients with non-cirrhotic liver disease, 1 of 7 patients with metastatic liver disease and 3 of 5 patients with alcoholic hepatitis had DDU signs of portal hypertension. Thus, in patients with parenchymal liver disease, DDU had a sensitivity of 73%, specificity of 90% and predictive values of 94 and 62% for positive and negative studies respectively for the detection of cirrhosis. In all 14 patients with portal hypertension secondary to vascular occlusive diseases, DDU examination of the ligamentum teres, portal vein and hepatic vein gave an accurate guide to the site of the occluding lesion. The high positive predictive value of DDU and its ability to aid in localizing the site of increased resistance to flow through the liver suggest that DDU of the ligamentum teres and portal vein is a potentially useful non-invasive adjunct in the assessment of patients with suspected or known liver disease or portal hypertension.     

Outcome of injection sclerotherapy using absolute alcohol in patients with cirrhosis, non-cirrhotic portal fibrosis, and extrahepatic portal venous obstruction

In order to assess the comparative efficacy and safety of endoscopic injection sclerotherapy in patients with portal hypertension of different etiology, i.e., cirrhosis, non-cirrhotic portal fibrosis, and extrahepatic portal venous obstruction, 87 patients with variceal bleeding were initiated on sclerotherapy using absolute alcohol. There was no significant difference in the success rate of sclerotherapy as well as in the number of sessions and volume of alcohol required for variceal obliteration between the three groups. Major complications included esophageal ulcers (30.0%), symptomatic strictures (18.6%), and interval re-bleed (17.1%) with similar complication rates for the three groups (p greater than 0.05). There was no difference between patients with Child's class A cirrhosis compared with classes B and C together with respect to efficacy and complications of sclerotherapy. Fifty patients (25 cirrhosis, 11 non-cirrhotic portal fibrosis, and 14 extrahepatic portal venous obstruction) with complete variceal obliteration were followed up for a mean period of 16.5 months. Sixteen patients (32%) had variceal recurrence, but bleeding due to recurrent varices occurred in only one case. There was no difference among the three groups for overall variceal recurrence, although recurrence tended to be somewhat later in extrahepatic portal venous obstruction (9.4 +/- 4.0 months) compared with that in cirrhosis (5.1 +/- 3.6 months) and non-cirrhotic portal fibrosis (4.8 +/- 2.6 months).     

Efficacy of endoscopic sclerotherapy on long-term management of oesophageal varices: a comparative study of results in patients with cirrhosis of the liver, non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous obstruction (EHO)

A prospective study was conducted to compare the results of long-term endoscopic variceal sclerotherapy in patients with different aetiologies of portal hypertension. A total of 404 consecutive patients were included. There were 234 patients with hepatic cirrhosis, 83 with non-cirrhotic portal fibrosis (NCPF) and 87 with extrahepatic portal venous obstruction (EHO). The mean follow-up for patients with cirrhosis, NCPF and EHO was 25, 37 and 28 months. A total of 73 (31%) patients with cirrhosis, 19 (23%) with NCPF and 10 (11.5%) with EHO rebled (P less than 0.05) on follow-up, prior to eradication of varices. Irrespective of the aetiology, 40 (17%) patients of Child's A class, 42 (33%) of Child's B and 20 (50%) of Child's C class rebled (P less than 0.01). The median bleeding free period (BFP) was longer (P less than 0.05) in patients with EHO than in cirrhotics. Patients in Child's A class had significantly longer BFP than those in Child's B, and the latter had a longer BFP than those in Child's C class (P less than 0.01). The probability of 7-year survival was also better with EHO (97.5%) and NCPF (73.6%) than cirrhotics (41%). Survivals in patients with EHO and NCPF were comparable (P less than 0.1). Similarly 7-year survival irrespective of aetiology in Child's A patients (90.7%) was longer than in Child's B (28.8%), and longer in Child's B than Child's C patients (0%). Success of eradication was greater (P less than 0.05) in EHO (92%) and NCPF (87%) than cirrhotic patients (75%).(ABSTRACT TRUNCATED AT 250 WORDS)     

Hepatopulmonary syndrome: prevalence and clinical profile.

BACKGROUND: The hepatopulmonary syndrome (HPS) is defined as a triad of liver dysfunction, intrapulmonary vascular dilatations (IPVD) and arterial hypoxemia. There is paucity of Indian studies regarding the prevalence of IPVD and arterial hypoxemia particularly amongst patients with non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO), where liver dysfunction is not a feature. METHODS: All patients with portal hypertension and esophageal varices seen at a tertiary care hospital during 1995-98 were studied. Ultrasonography of abdomen, contrast-enhanced echocardiography (CEE), arterial blood gas analysis and assessment of alveolar-arterial oxygen gradient were done. RESULTS: Of 138 patients with portal hypertension seen during the study period, 88 fulfilled the inclusion and exclusion criteria. These included 63 with cirrhosis, 15 with NCPF and 10 with EHPVO. CEE showed IPVD in 17 (27%) patients with cirrhosis, of which 11 (17.5%) fulfilled the criteria for HPS. IPVD were also noted in 4 (26.6%) cases of NCPF and 3 (30%) of EHPVO, though only 2 (13.3%) and 1 (10%) respectively had elevated alveolar-arterial gradient and liver dysfunction in addition. Age and sex distribution and duration of symptoms were not different in patients with HPS. Patients with HPS had higher incidence of dyspnea, platypnea, clubbing and spider nevi. CONCLUSIONS: Hepatopulmonary syndrome is present in 17.5% of cirrhotics, 13.3% of patients with NCPF and 10% with EHPVO. Patients with HPS had significantly higher incidence of dyspnea, platypnea, clubbing and spider nevi.     

Successful treatment of noncirrhotic portal hypertension with eculizumab in paroxysmal nocturnal hemoglobinuria: A case report

BACKGROUND Idiopathic non-cirrhotic portal hypertension(INCPH) is mainly associated with thrombophilia in Western countries. Paroxysmal nocturnal hemoglobinuria(PNH) is a rare hematologic disease that manifests with hemolytic anemia,thrombosis, and peripheral blood cytopenias. Portal and hepatic venous thrombosis were reported in PNH. A rare case of INCPH complicating PNH is described.CASE SUMMARY A 63-year old woman with a 2-year past medical history of PNH without treatment was admitted because of jaundice and refractory ascites requiring large volume paracentesis. Liver histology revealed portal venopathy with portal fibrosis and sclerosis, nodular regenerative hyperplasia, parenchymal ischemic changes, and focal sinusoidal and perivenular fibrosis without bridging fibrosis or cirrhosis, all indicative of INCPH. The flow cytometry confirmed PNH diagnosis and eculizumab treatment was initiated. Her condition was improved gradually, bilirubin was normalized 6 months following initiation of eculizumab,and 1 year later diuretics were stopped.CONCLUSION Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria.     

Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal hepatic fibrosis

Non-cirrhotic perisinusoidal hepatic fibrosis is a process of imprecise pathogenesis involving collagenization of the space of Disse. Exposure to chemicals, auto-immunity, thrombophilia and/or infections are suspected primary agents. Here, we present the case of a patient who developed severe portal hypertension with histological features suggesting a non-cirrhotic perisinusoidal hepatic fibrosis. A 52-year-old man was hospitalized for oesophageal variceal haemorrhage. Liver cirrhosis or portal vein thrombosis were absent as attested by laboratory tests, duplex sonography, computed tomography scan and histological examination of a liver biopsy specimen. Presinusoidal portal hypertension was suggested by a normal wedge-free hepatic vein gradient. Only electron microscopy examination of a liver biopsy specimen could disclose perisinusoidal fibrosis. This was most probably secondary to a combined chemotherapy received 4 years earlier for non-Hodgkin large-cell lymphoma. As variceal ligation failed to control oesophageal varices while liver function tests were normal, a transjugular intrahepatic portosystemic shunt (TIPS) was performed. This dramatically improved the signs of portal hypertension. This case illustrates the use of TIPS in the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal fibrosis.     

Histological abnormalities of the small bowel mucosa in cirrhosis and portal hypertension

AIM： To study the small bowel （SB） mucosa on biopsy in cirrhotic patients with portal hypertension and in non-cirrhotic controls and grade findings according to the Marsh criteria. METHODS： We prospectively enrolled 51 consecutive patients undergoing an upper endoscopy for their routine medical care. Twenty five patients with cirrhosis and portal hypertension were compared to 26 controls. We obtained coeliac serology and multiple upper small bowel biopsies on all 51 patients. A GI pathologist interpreted biopsies and graded findings according to the Marsh criteria. We assessed equivalence in Marsh grade between cirrhotic and non-cirrhotic controls using the Mann-Whitney test for equivalence. RESULTS： Gender, ethnicity and age were similar between both groups. Marsh grades were equivalent between the groups. Grade of 0 was present in 96% and grade of 1 was present in 4% of both groups and there was no villus atrophy or decrease in villus/crypt ratio in patients with portal hypertension. CONCLUSION： This study provides evidence for the lack of villus atrophy in patients with cirrhosis and portal hypertension, and supports the continuous reliance on the Marsh criteria when the diagnosis of coeliac disease is to be made in the presence of cirrhosis.     

Long-term follow-up study of adult patients with non-cirrhotic obstruction of the portal system: comparison with cirrhotic patients.

Thirty-two patients with non-cirrhotic portal system obstruction and oesophageal varices of non-malignant etiology were recruited over 13 years. Diagnosis was based on the presence of oesophageal varices at endoscopy, minor alterations in liver function tests and liver histology, a low hepatic venous pressure gradient, and pertinent angiographic patterns. Twenty-three had portal vein thrombosis, nine had splenic vein thrombosis. Twenty-one had idiopathic portal vein obstruction, 11 had secondary obstruction. The outcome was compared with a group of 32 patients with cirrhosis and portal hypertension, matched for age, Child-Pugh class, previous history of gastrointestinal bleeding, and size of oesophageal varices. Patients with non-cirrhotic obstruction of the portal system were followed for up to 171 months (mean 94 months). During follow-up ten patients had gastrointestinal bleeding, and eight died (five of gastrointestinal bleeding). After 6 years of follow-up, the cumulative risk of gastrointestinal bleeding was 24%, the cumulative risk of death was 17%, and the cumulative risk of death from gastrointestinal bleeding was 14%. Cumulative probability of death by any cause and the probability of gastrointestinal bleeding were significantly lower in patients with non-cirrhotic obstruction of the portal system than in patients with cirrhosis comparable for liver function and portal hypertension (p = 0.04 for both). The cumulative probability of death by gastrointestinal bleeding was not significantly different. In conclusion, the prognosis for non-cirrhotic obstruction of the portal system is significantly better than for patients with cirrhosis with comparable levels of liver function impairment and severity of portal hypertension.     

Systemic and pulmonary hemodynamics in patients with extrahepatic portal vein obstruction is similar to compensated cirrhotic patients

Background  Patients with cirrhosis and portal hypertension exhibit a hyperdynamic circulation manifesting as increased cardiac output, heart rate and plasma volume; and decreased arterial blood pressure, systemic vascular resistance, and pulmonary vascular resistance. It is believed that these changes are related to both hepatocellular dysfunction and portal hypertension. However, the role of portal hypertension per se in producing these changes in circulation has not been clear. Extrahepatic portal vein obstruction (EHPVO), a vascular disorder of the liver characterized by cavernomatous transformation of the main portal vein, is an excellent model to study the role of portal hypertension per se in producing these changes because there is no hepatic dysfunction in EHPVO. The main aim of our study was, therefore, to evaluate alterations of systemic and pulmonary vascular systems in patients with EHPVO and compare them with patients with compensated cirrhosis. Patients and methods  Consecutive patients of EHPVO, 15 years or older, and past variceal bleeders were studied. For comparison, consecutive patients with compensated cirrhosis and history of variceal bleed, matched for variceal status, and body surface area were included. The hemodynamic studies included the measurements of cardiac index (by Fick’s oxygen method), and systemic and pulmonary vascular resistance indices. Results  Fifteen patients of EHPVO and same number of controls (compensated cirrhotics) were included in the study. The baseline parameters in the two groups were comparable. Both EHPVO patients and cirrhotics had similar values in all the measured systemic and pulmonary hemodynamic parameters. The median (range) cardiac index in EHPVO was 3.8 (2.3–7.7) l min⁻¹ m⁻², whereas it was 4.4 (2.8–8.9) l min⁻¹ m⁻² in cirrhosis (P = 0.468). The median (range) systemic vascular resistance index in EHPVO was 1,835 (806–3400) dyne s cm⁻⁵ m⁻², which was similar to that in cirrhotic patients (1,800 [668–3022], P = 0.520). Similarly, the values of median (range) pulmonary vascular resistance index were comparable in the two groups (71 [42–332] vs. 79 [18–428], P = 0.885). A subgroup analysis was done for 8 patients of EHPVO and 8 age-matched compensated cirrhotic patients, which also revealed similar values of cardiac index, cardiac output, systemic vascular resistance index, systemic vascular resistance, pulmonary vascular resistance index, and pulmonary vascular resistance in the two groups. Conclusions  EHPVO patients have hyperdynamic circulation manifested by high cardiac index and low systemic and pulmonary vascular resistance indices. These hemodynamic changes are comparable with compensated cirrhotic patients who have similar grade of portal hypertension. This suggests a predominant role of portal hypertension per se in the genesis of systemic and pulmonary hemodynamic alterations.     

Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non-cirrhotic portal fibrosis are presented. METHODS: The disease is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction (EHPVO). Retrospective analysis of records of 151 patients with NCPF was analyzed for the clinical presentation, physical findings, laboratory tests, radiological and histological findings, and for the outcome of treatment. RESULTS: The disease is characterized by massive splenomegaly with anemia, preserved liver function and benign prognosis in a majority of patients. Splenoportovenography (SPV) showed massive dilatation of the portal and splenic veins, and the presence of collaterals. Twenty-four (15.9%) patients showed evidence of natural/spontaneous shunts (splenorenal 15, umbilical nine) on SPV; these patients had a lower incidence of variceal bleeding. Liver histology demonstrated maintained lobular architecture, portal fibrosis of variable degree, sclerosis and obliteration of small-sized portal vein radicles, and subcapsular scarring with the collapse of the underlying parenchyma. Piecemeal or hepatocytic necrosis was absent in all histology specimens. Three patients showed nodular transformation along with abnormal liver functions, and may represent late manifestation of NCPF where features are similar to those seen in patients with incomplete septal cirrhosis. In the initial part of the study, surgery (side-to-side lieno-renal shunt) was the preferred modality of treatment, however, endoscopic sclerotherapy or variceal ligation has now become the preferred first line of management of variceal bleeding. CONCLUSIONS: The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.     

Observation of thoracic duct morphology in portal hypertension by endoscopic ultrasound

Background:Thoracic duct dilation has been demonstrated in portal hypertension and hepatic cirrhosis by lymphangiography and laparotomy and at autopsy. It is thought to be secondary to increased hepatic lymph flow and has been described in the absence of ascites or esophageal varices. The aim of the present study was to observe thoracic duct morphology by endoscopic ultrasound in various subsets of patients with portal hypertension and hepatic cirrhosis and also to validate existing radiologic/surgical data. Methods:The thoracic duct of 33 patients with cirrhosis and portal hypertension was studied by endoscopic ultrasound. Patients were divided into four groups: 1, patients with ascites and esophageal varices; 2, esophageal varices without ascites; 3, without esophageal varices or ascites; 4, extrahepatic portal hypertension due to pancreatic malignancy. The thoracic duct diameter was also measured in 14 control subjects (group 5). Results:When the thoracic duct diameter for the five groups was compared with analysis of variance, significance was p < 0.0001; by pairwise comparison, group 1 differed from the other four groups (p < 0.05). Thoracic duct dilation (5.61 mm) was seen in group 1 patients, whereas no dilation was present in groups 2 through 4. Additionally, thoracic duct diameter in 33 portal hypertensive and/or cirrhotic patients was significantly different from that in the 14 control subjects (p = 0.003). Conclusion:The thoracic duct can be reliably identified by EUS in patients with hepatic cirrhosis and portal hypertension. Dilation of the duct is seen only in patients with hepatic cirrhosis, ascites, and esophageal varices. No thoracic duct dilation is present in extrahepatic portal hypertension. Contrary to existing radiologic/surgical data, thoracic duct dilation is not seen in all patients with hepatic cirrhosis and portal hypertension signifying advanced disease. A dilated thoracic duct by endoscopic ultrasound should be considered yet another sign of portal hypertension. (Gastrointest Endosc 1998;48:588-92.)