胰源性区域性门脉高压症——附14例报告

本文报告了14例胰源性区域性门脉高压症。本病为肝外型门脉高压症中罕见的一种，对未发现肝脏疾病，而有胃底，食管静脉曲张，脾肿大的病人，应考虑本病。门静脉造影可以确立诊断，经皮脾穿刺门静脉造影是确诊本症的三种简单可行的方法。本病可经脾切除治愈。     

Endoscopic sclerotherapy for bleeding esophageal varices secondary to extrahepatic portal vein obstruction

Portal hypertension and variceal bleeding secondary to extrahepatic portal vein obstruction continue to present a therapeutic challenge. We performed endoscopic injection sclerotherapy in eight patients with extrahepatic portal vein obstruction and bleeding esophageal varices. In contrast to other reported series, all but one of our patients were adults at the time sclerotherapy was initiated. Six had episodes of continued bleeding after a variety of surgical procedures. After sclerotherapy, five had no further bleeding with a mean follow-up of 26 months. Three patients had episodes of bleeding prior to variceal obliteration; two of these patients underwent surgical intervention after emergency sclerosis to stabilize their condition. Transfusion requirements were less after sclerosis (p = 0.035), although the follow-up has been relatively short (mean, 24 months) compared to the duration of bleeding. Our results suggest that endoscopic sclerotherapy is an effective therapeutic alternative, and perhaps the initial treatment of choice, in patients with extrahepatic portal vein obstruction and bleeding esophageal varices.     

Impact of portal vein thrombosis on the efficacy of endoscopic variceal band ligation

BackgroundInfluence of portal vein thrombosis on efficacy of endoscopic variceal banding in patients with cirrhosis or extrahepatic portal vein obstruction has never been evaluated. Aim of the study was to assess influence of thrombosis on rate and time to eradication in cirrhosis and extrahepatic portal vein obstruction undergoing banding, compared to cirrhotic patients without thrombosis.MethodsRetrospective analysis of 235 consecutive patients (192 with cirrhosis without thrombosis, 22 cirrhosis and thrombosis and 21 extrahepatic portal vein obstruction) who underwent banding. Banding was performed every 2–3 weeks until eradication; endoscopic follow-up was performed at 1, 3, 6 months, then annually.ResultsEradication was achieved in 233 patients. Median time to eradication in cirrhotic patients with portal vein thrombosis vs. cirrhotic patients without thrombosis was 50.9 days (12–440) vs. 43.4 days (13–489.4); log-rank: 0.04; patients with extrahepatic portal vein obstruction vs. cirrhotic patients without thrombosis 63.9 days (31–321.6) vs. 43.4 days (13.0–489.4); log-rank: 0.008. Thrombosis was shown to be the only risk factor for longer time to eradication.ConclusionsPortal vein thrombosis per se appears to be the cause of a longer time to achieve eradication of varices but, once eradication is achieved, it does not influence their recurrence.     

Sclerotherapy in extrahepatic portal venous obstruction.

One hundred and twenty two patients who presented with variceal bleeding as a result of extrahepatic portal vein obstruction (EHPO) were entered into the sclerotherapy programme with a mean follow up of 23.69 months (range four to 60 months). Eighteen (14.7%) patients were lost to follow up, three (2.4%) patients underwent surgery, and six (4.9%) patients died. Variceal obliteration was achieved in the remaining 95 patients requiring 5.4 (2.4) sessions of sclerotherapy (range 2-18). Seventeen episodes of upper gastrointestinal bleed occurred in 15 patients during sclerotherapy. Recurrence of oesophageal varices was seen in 15 patients. Ten patients developed bulbous gastric varices after obliteration. Major complications including perforation and strictures were seen more commonly in children. Sclerotherapy was associated with a significant reduction in the bleeding rate (bleeds/month/patient) as compared with the presclerotherapy period (p less than 0.001). Endoscopic sclerotherapy is an effective and safe modality in the prevention of variceal bleeds in patients with extrahepatic portal vein obstruction.     

Laparoscopic splenectomy for variceal bleeding with non-cirrhotic portal vein thrombosis: a case report

A 57-year-old man was referred to our hospital for treatment of refractory gastric bleeding from gastric varices secondary to portal vein thrombosis. The patient's liver function tests and coagulation profile were normal. The venous phase of the superior mesenteric arteriogram, on the other hand, showed superior mesenteric vein-portal vein occlusion with surrounding hepatopetal variceal collaterals. The venous phase of the splenic arteriogram additionally showed splenic vein occlusion and collateral vessels from the gastric and retroperitoneal regions flowing into a portal cavernous transformation. Gastroscopy confirmed that the patient had gastric varices in the cardia. We performed laparoscopic splenectomy to treat refractory gastric bleeding from varices and symptomatic hypersplenism. The postoperative course was uneventful; the patient's gastric varices were less prominent on follow-up gastroscopy and the hematologic profile returned to normal. Extrahepatic portal vein thrombosis is the leading cause of variceal hemorrhage in patients with healthy livers. There is a consensus in the literature that splenectomy alone is of minimal value in preventing variceal bleeding in portal vein thrombosis. Splenectomy is, however, indicated in cases in which the patient has hepatopetal collaterals from the mesenteric vein system and whose hemorrhagic gastric varices are related to splenic vein thrombosis as in our case.     

Prevalence, classification and natural history of gastric varices: a long-term follow-up study in 568 portal hypertension patients.

To determine the prevalence and natural history of gastric varices, we prospectively studied 568 patients (393 bleeders and 175 nonbleeders) with portal hypertension (cirrhosis in 301 patients, noncirrhotic portal fibrosis in 115 patients, extrahepatic portal vein obstruction in 117 patients and hepatic venous outflow obstruction in 35 patients). Primary (present at initial examination) gastric varices were seen in 114 (20%) patients; more were present in bleeders than in non-bleeders (27% vs. 4%, respectively; p < 0.001). Secondary (occurring after obliteration of esophageal varices) gastric varices developed in 33 (9%) patients during follow-up of 24.6 +/- 5.3 mo. Gastric varices (compared with esophageal varices) bled in significantly fewer patients (25% vs. 64%, respectively). Gastric varices had a lower bleeding risk factor than did esophageal varices (2.0 +/- 0.5 vs. 4.3 +/- 0.4, respectively) but bled more severely (4.8 +/- 0.6 vs. 2.9 +/- 0.3 transfusion units per patient, respectively). Once a varix bled, mortality was more likely (45%) in gastric varix patients. Gastric varices were classified as gastroesophageal or isolated gastric varices. Type 1 gastroesophageal varices (lesser curve varices) were the most common (75%). After obliteration of esophageal varices, type 1 gastroesophageal varices disappeared in 59% of patients and persisted in the remainder; bleeding from persistent gastroesophageal varices was more common than it was from gastroesophageal varices that were obliterated (28% vs. 2%, respectively; p < 0.001). Type 2 gastroesophageal varices, which extend to greater curvature, bled often (55%) and were associated with high mortality. Type 1 isolated gastric varices patients had only fundal varices, with a high (78%) incidence of bleeding.(ABSTRACT TRUNCATED AT 250 WORDS)     

Diagnosis and management of ectopic varices

Abstract   Ectopic varices are dilated portosystemic venous collateral vessels that may occur anywhere in the gastrointestinal tract. Ectopic varices account for approximately 5% of all hemorrhages from varices. Ectopic varices may occur as a result of portal hypertension from any cause but are more common (particularly duodenal and biliary varices) in patients with extrahepatic portal vein thrombosis. Ectopic varices may also develop following successful endoscopic obliteration of gastroesophageal varices. With the exception of isolated gastric fundal varices, ectopic varices have relatively low risk for bleeding. Diagnosis is often made by endoscopy; however, computed tomography, magnetic resonance imaging and portal venography may be needed in some cases. Endoscopic treatment is successful in many cases and is the safest option provided bleeding is definitively controlled. Surgical options are now reserved for treatment of life-threatening bleeding or for shunt insertion in patients who are not candidates for transjugular intrahepatic portosystemic shunt (TIPS) as a result of portal vein thrombosis. Portal decompression using TIPS, in spite of the risk for encephalopathy, is the treatment of choice for bleeding from ectopic varices that cannot be successfully managed endoscopically.     

Extrahepatic Portal Vein Obstruction and Portal Vein Thrombosis in Special Situations: Need for a New Classification

Extrahepatic portal vein obstruction is a vascular disorder of liver, which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein, splenic vein, or superior mesenteric vein. Portal vein obstruction due to chronic liver disease, neoplasm, or postsurgery is a separate entity and is not the same as extrahepatic portal vein obstruction. Patients with extrahepatic portal vein obstruction are generally young and belong mostly to Asian countries. It is therefore very important to define portal vein thrombosis as acute or chronic from management point of view. Portal vein thrombosis in certain situations such as liver transplant and postsurgical/liver transplant period is an evolving area and needs extensive research. There is a need for a new classification, which includes all areas of the entity. In the current review, the most recent literature of extrahepatic portal vein obstruction is reviewed and summarized.     

The epidemiology and pathogenesis of gastrointestinal varices

Gastrointestinal varices are a consequence of portal hypertension that can occur in the setting of cirrhosis or extrahepatic portal vein obstruction. Increased intrahepatic vascular resistance, a hyperdynamic circulation, and increased flow through the portal and collateral venous system lead to persistently elevated portal pressures that result in angiogenesis and formation of collaterals between the portal and systemic circulation. Despite this physiological attempt at decompression, portal hypertension persists as collateral vessels have higher resistance than the normal liver. Variceal wall tension is the main factor that determines vessel rupture and bleeding occurs when tension in the wall exceeds the limit of elasticity of the vessel. Progressive distension leads to increasing resistance to flow and hemorrhage ensues when the limits of resistance to further dilation are surpassed. Gastroesophageal varices are present in 50% of patients with cirrhosis and progress in size at a rate of 8%-10% per year. Hemorrhage occurs at a rate of approximately 12% per year and large esophageal varices carry a higher risk of rupture. Gastric varices occur in 20% of patients with portal hypertension and bleed less frequently, but more severely. Cardiofundal varices have a complex vascular anatomy that is important to consider as it pertains to the effectiveness of strategies used for management. Ectopic varices make up 2%-5% of all variceal bleeding, occur more frequently in patients with extrahepatic portal hypertension, and their identification should prompt assessment of the intra-abdominal vasculature. Varices in the setting of splenic vein thrombosis should be considered a distinct entity owing to their disparate etiologic basis and treatment approach.     

The natural history of portal hypertensive gastropathy: influence of variceal eradication

OBJECTIVE: The natural history and likelihood of bleeding from portal hypertensive gastropathy (PHG) present in patients with portal hypertension before endoscopic variceal obliteration may differ from that in patients who develop PHG during or after variceal eradication. METHODS: A total of 967 variceal bleeders who had achieved variceal eradication by endoscopic sclerotherapy in the recent past were prospectively studied. In all, 88 (9.1%) patients (cirrhosis in 54, noncirrhotic portal fibrosis in 18, and extrahepatic portal vein obstruction in 16) had distinct mucosal lesions. PHG alone was present in 78, PHG with gastric antral vascular ectasia (GAVE) in eight, and GAVE alone in two patients. PHG was graded as mild or severe and according to whether present before (group A) or after endoscopic intervention (group B). Patients underwent regular endoscopy at follow-up to see if the PHG was transitory (disappearing within 3 months), persistent (no change), or progressive. Bleeding from PHG lesions was defined as acute or chronic. RESULTS: Twenty-two (26%) patients had PHG before (group A) and 64 (74%) developed PHG after variceal eradication (group B). During a mean follow-up of 25.1 +/- 14.2 months, PHG lesions disappeared in group A in only two patients (9%), but in group B in 28 (44%) patients (p < 0.05). PHG lesions more often progressed in the former as compared to the latter (18% vs 9.4%, p = NS). The incidence of bleeding was higher in group A than group B (32% vs 4.7%, p < 0.02). Bleeding from PHG occurred in 10 patients (11.6%); seven of them were from group A, and all had either progressive (n = 3) or persistent (n = 4) lesions. CONCLUSIONS: PHG developing after variceal eradication is often transitory and less severe. If PHG is pre-existing, endoscopic therapy for varices could worsen the PHG, with a likelihood of bleeding. Such patients may be benefited by concomitant beta-blocker therapy.     

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).     

Combined liver vein and spleen pulp pressure measurements in patients with portal or splenic vein thrombosis

BACKGROUND: Patients with thrombosis of the portal or splenic vein may develop portal hypertension with bleeding from oesophageal or gastric varices. The relevant portal pressure cannot be measured by liver vein catheterization or transhepatic puncture of the portal vein because the obstruction is peripheral to the accessible part of the portal system. METHODS: Liver vein catheterization was combined with percutaneous splenic pressure measurement in 10 patients with portal or splenic vein thrombosis and no cirrhosis, and 10 cirrhotic patients without thrombosis. The splenic pressure was measured by percutaneous puncture below the curvature of the ribs with an angle of the needle to skin of 30 degrees in order to minimize the risk of cutting the spleen if the patient took a deep breath. RESULTS: None of the patients in whom the described procedure was followed had complications. Pressure measurements in the spleen pulp and splenic vein were concordant. The pressure gradient across the portal venous system (splenic-to-wedged hepatic vein pressure) was -1.3 to 8.5 mmHg (median, 2.8 mmHg) in cirrhosis patients and 0-44 mmHg (median, 18 mmHg) in thrombosis patients, the variation reflecting various degrees of obstruction to flow in the portal venous system. Peripheral portal pressure (splenic-to-free liver vein pressure gradient) was 1.1-28 mmHg (median, 17 mmHg) in cirrhotic patients and 11-52 mmHg (median, 23 mmHg) in thrombosis patients. CONCLUSIONS: Liver vein catheterization combined with percutaneous splenic pressure measurement is feasible in quantifying pressure gradient across a thrombosis of the portal/splenic vein and in quantifying portal pressure peripheral to this kind of thrombosis.     

Sinistral portal hypertension. A case report

Sinistral portal hypertension is a clinical syndrome of gastric variceal hemorrhage in the setting of splenic vein thrombosis due to a primary pancreatic pathology. The distinguishing features from other forms of portal hypertension are preserved liver function and a patent extrahepatic portal vein. The important causes include acute and chronic pancreatitis, pancreatic pseudocysts and pancreatic carcinomas. Benign pancreatic neoplasms only rarely cause sinistral portal hypertension. Splenic vein thrombosis complicates 7-20% of patients having pancreatitis or a pancreatic pseudocyst; however, bleeding occurs in only approximately 5% of patients. The diagnosis of sinistral portal hypertension is achieved by a combination of gastroscopy, liver function tests, ultrasound examination (with Doppler) and/or contrast-enhanced CT scan of the abdomen. A mere demonstration of sinistral portal hypertension does not warrant intervention. An expectant management is justifiable in asymptomatic patients with pancreatitis. However, concomitant splenectomy may be considered in patients undergoing operative treatment of symptomatic chronic pancreatitis if sinistral portal hypertension and gastroesophageal varices are present. In patients presenting with gastric variceal hemorrhage, splenectomy (with treatment for the primary pancreatic pathology, e.g. distal pancreatectomy) is curative with excellent long term results.     

经皮经肝曲张静脉栓塞治疗断流术后食管胃底静脉破裂出血的疗效

目的 研究以组织胶为主要栓塞材料,采用经皮经肝曲张静脉栓塞术(PTVE)治疗和预防门奇静脉断流术后食管胃底静脉曲张破裂出血的临床疗效.方法 2006年11月至2008年9月,对22例曾行断流术再发食管胃底静脉曲张破裂出血的患者行PTVE组织胶栓塞(n=10)或内镜下硬化剂(EIS,n=12)治疗,随访两组患者治疗后再出血率、死亡率、治疗前后静脉曲张和肝功能以及PTVE治疗组患者在曲张侧支静脉栓塞前后门静脉压力的变化.结果 ①在平均12.5个月的随访期内,PTVE治疗组患者再出血率和死亡率分别为1/10和0;EIS治疗组随访13.4个月,患者再出血率和死亡率分别为7/12和3/12,两组问差异有统计学意义(P<0.05).②PTVE和EIS治疗均可显著减轻食管和胃底静脉曲张程度.③对有门静脉血栓患者,PTVE联合门静脉球囊成形术,可以改善肝脏门静脉血供.④PTVE和EIS治疗均未加重肝功能损伤.结论 对门奇静脉断流术后食管胃底静脉破裂出血的患者,采用以组织胶为主要栓塞材料的PTVE治疗的疗效优于EIS治疗.     

Portal vein thrombosis： Etiology and clinical outcome of cirrhosis and malignancy-related non-cirrhotic, non-tumoral extrahepatic portal venous obstruction

The etiology and pathogenesis of portal vein thrombosis are unclear. Portal venous thrombosis presentation differs in cirrhotic and tumor-related versus non-cirrhotic and non-tumoral extrahepatic portal venous obstruction (EHPVO). Non-cirrhotic and non-tumoral EHPVO patients are young and present with well tolerated bleeding.Cirrhosis and tumor-related portal vein thrombosis patients are older and have a grim prognosis. Among the 118 patients with portal vein thrombosis, 15.3% had cirrhosis, 42.4% had liver malignancy (primary or metastatic), 6% had pancreatitis (acute or chronic), 5% had hypercoagulable state and 31.3% had idiopathy,12% had hypercoagulable state in the EHPVO group.     

孤立性胃静脉曲张的病因和临床特征

目的 分析孤立性胃静脉曲张的病因和临床特征.方法 选择2003年1月至2008年1月收治的31例孤立性胃静脉曲张患者,回顾性分析其病因、临床表现、影像学检查及治疗情况.结果 31例占同期胃食管静脉曲张患者的7.38%(31/420).病因依次为左侧门脉高压(14例,45.2%)、肝硬化(8例,25.8%)、不明原因(6例,19.4%)、原发性肝癌(2例,6.5%)、门静脉海绵样变性(1例,3.2%).曲张静脉破裂出血21例(67.7%).脾肿大21例(67.7%),其中脾功能亢进10例(32.3%).18例患者有明确的受侵静脉,其中脾静脉阻塞9例(50%),门静脉受侵9例(50%).外科手术8例,硬化剂治疗3例,其余20例内科保守治疗.结论 孤立性胃静脉曲张大部分由左侧门脉高压引起,部分病因是肝硬化.手术是治疗出血和预防再出血的有效措施.     

Splenogastrorenal shunt in portal hypertension: a little known entity. Study of 6 cases and review of the literature

The authors report 6 cases of portal hypertension with gastrorenal shunt. This shunt did not arise from the left gastric vein, but from the splenic vein. Portal hypertension was related to alcoholic cirrhosis in 3 cases, to extensive portal thrombosis in 2 cases, and to nodular regenerative hyperplasia of the liver in one case. A gastrointestinal hemorrhage revealed portal hypertension and the liver disease in the 3 cases of alcoholic cirrhosis and complicated the course of the disease in the other cases. Hemorrhage was either massive and life-threatening or often recurred. It was related to a rupture of fundic varices in all cases. The fundic varices were not associated with esophageal varices in the 3 cases of cirrhosis. The degree of portal hypertension was above 20 mm Hg, as assessed by the portohepatic gradient (one case), or the pressure gradient between a tributary portal system vein and the inferior vena cava during laparotomy (5 cases). Definitive control of hemorrhage could not be achieved by endoscopic variceal sclerotherapy (2 cases) or percutaneous transhepatic embolization (one case). Portacaval shunt or splenectomy was performed in 5 cases. These findings suggest that spontaneous splenogastrorenal shunt is a clinical and hemodynamic entity which requires specific treatment when associated with gastric variceal bleeding.     

Bleeding isolated gastric varices: a retrospective analysis.

OBJECTIVE: Isolated gastric varices (IGV) are rare and are believed to be associated with left-sided portal hypertension. We studied patients presenting with bleeding from IGV and compared them with those bleeding from both esophageal and gastric varices. METHODS: A retrospective analysis of 14 patients with bleeding from IGV was carried out. Portovenography findings (pattern of collateralization and natural shunts) in these patients were compared with a matched group of 69 patients with both esophageal and gastric varices. RESULTS: Of 14 patients with IGV, 2 had isolated splenic vein thrombosis and 12 had generalized portal hypertension. Portovenograms in 11 of the latter 12 revealed predominantly 'left-sided' collateralization in 8 patients as compared to 17 of 69 (25%) patients with esophageal and gastric varices (p = 0.004); natural shunts were seen in 6 of 11 cases and 15 of 69 (22%) patients in the two groups, respectively (p = 0.05). Abdominal devascularization operation gave good short- and long-term control of bleeding. CONCLUSIONS: Contrary to belief most patients with isolated gastric varices may have generalized portal hypertension rather than splenic vein obstruction as the cause and hence should be treated by a more extensive procedure than just splenectomy. The IGV could be a result of predominant collateralization to the retroperitoneal area (left-sided collateralization and natural shunts) rather than the usual pattern to the azygos system which results in esophageal varices.     

The effects of chronic endoscopic variceal sclerotherapy on portal pressure in cirrhotics

The effect of obliterating esophageal varices by endoscopic sclerotherapy on portal pressure was prospectively studied in 11 cirrhotic patients with variceal hemorrhage. Portal venous pressure gradient, determined as the difference between transhepatic portal and hepatic vein pressure, increased by a mean of 31.1% +/- 14.5% in 8 (73%) and decreased by a mean of 30.1% +/- 11.7% in 3 (27%) patients, with no statistically significant change overall (P = 0.1). These changes in portal venous pressure gradient occurred despite an improvement in the laboratory and clinical parameters of hepatic function. Deep abdominal sonography with color flow imaging at variceal obliteration showed patent paraumbilical veins in 6 (55%) patients, 3 of whom had decreases in portal venous pressure gradient (29%, 19%, 42.5%) at variceal obliteration. In 5 (45%) patients without patent paraumbilical veins, a statistically significant increase in portal venous pressure gradient between initial endoscopic variceal sclerotherapy and variceal obliteration was noted (P = 0.008). Rebleeding (single episode in all 4 patients, before obliteration in 3 patients) occurred in those with an increase in portal venous pressure gradient; all patients with portal venous pressure gradient decreases were nonbleeders. No correlation between changes in portal venous pressure gradient and time to variceal obliteration, number of sclerotherapy treatments, or rebleeding episodes was observed. Thus, an increase in portal venous pressure gradient was noted in the majority of patients at variceal obliteration. Although the portal venous pressure gradient decrease may be explained by a patent paraumbilical vein, the mechanism of portal venous pressure gradient increase is not clear. It is speculated that this portal venous pressure gradient increase may be caused by an increase in collateral resistance or flow or a combination of both, resulting from obliteration of esophageal varices by endoscopic sclerotherapy.     

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.