Pulmonary hypertension associated with liver cirrhosis: an echocardiographic study

Conflicting results about the prevalence of pulmonary hypertension, ranging from 0.25% to 20%, in liver patients with portal hypertension, have previously been reported. The aim of this study was to evaluate pulmonary arterial pressure in a consecutive series of cirrhotic patients, using a noninvasive method. A complete clinical, laboratory, ultrasonographic, and endoscopic evaluation were performed in 83 consecutive liver patients assessed according to Child's classification and Pugh's score and according to evidence of ultrasonographic and/or endoscopic signs of portal hypertension. A complete echocardiographic evaluation was also performed and pulmonary arterial systolic pressure (PASP) was estimated by measuring tricuspidal regurgitation, using the modified Bernoulli equation. These same evaluations were performed by the same observers in a group of 60 healthy volunteers. The results showed a surprisingly high prevalence (about 20%) of pulmonary hypertension. Patients with more severe liver damage and portal hypertension showed a high prevalence for pulmonary hypertension. A progression in the frequency of portopulmonary hypertension (PPH) was found in Child's classification A to C, and in patients without to patients with evidence of portal hypertension. However, increased PASP was detected in some patients belonging to Child's class A, without evidence of portal hypertension. In conclusion, the echocardiographic examination (a noninvasive technique), appears suitable for detecting pulmonary hypertension in patients with compensated liver cirrhosis, and can elucidate some aspects of the clinical course of the so-called PPH syndrome.     

Asymptomatic primary pulmonary hypertension associated with liver cirrhosis

We report a case of asymptomatic primary pulmonary hypertension associated with liver cirrhosis (type B) and portal hypertension found by chance during a preoperative Swan-Gantz catheterization study. Our experience suggests that the actual prevalence of primary pulmonary hypertension associated with liver cirrhosis may be greater than that previously reported. During the follow-up of liver cirrhosis with portal hypertension, we should consider primary pulmonary hypertension, even if the patient is free of symptoms, and a chest X-ray check may be necessary.     

Comparison of portacaval shunts in patients with alcoholic and nonalcoholic liver disease

A series of 46 patients with alcoholic and nonalcoholic cirrhosis and who underwent portasystemic decompressive procedures were analyzed and compared. The two groups were similar in age and degree of dysfunction according to liver function tests and functional classification. For both emergency and elective procedures operative mortality was significantly less in the nonalcoholic group: For emergency procedures it was 20% in the nonalcoholic group, 57% in the alcoholic; for elective procedures it was 5.3% in the nonalcoholic, 20% in the alcoholic. Long-term survival and frequency of severe encephalopathy were nearly the same for both groups although 60-year-old or older nonalcoholics did better, with no operative deaths and only 1 case of severe encephalopathy. The older patients with alcoholic cirrhosis faired poorly—4 of 5 died of hepatic failure, 2 during the operative period. Three patients had active chronic hepatitis and cirrhosis. One died during the operative period; the other 2 have suffered from chronic encephalopathy requiring continuous medical therapy for control.At the VA Hospital Woodard is a Research and Education Trainee in Gastroenterology and Webster is a Medical Investigator.     

肝积合剂对肝硬化门静脉高压的影响

目的：探讨肝积合剂对肝硬化门脉高压的影响。方法：将60例肝硬化门脉高压症患者随机分为两组，治疗组患者口服中药肝积合剂，对照组患者口服心得安，共3个月。用彩色双功能多普勒超声诊断仪测量门静脉内径、脾静脉内径、门静脉血流速度、脾静脉血流速度。采用胃镜观察食管静脉曲张部位、条数、形态、颜色及胃底有无静脉曲张。结果：肝积合剂可显著缩小肝硬化患者门静脉及脾静脉内径、加快血流速度、减少血流量。尤其在加快门静脉、脾静脉血流速度及减少血流量上与心得安比较，差异有显著性意义（P〈0．05）。结论：肝积合剂对肝硬化门脉高压症有一定的防治作用。     

Glucose tolerance after portacaval shunt in liver cirrhosis

The liver plays a key role in glucose homeostasis and insulin metabolism. Altered glucose and insulin levels in peripheral blood are common findings in chronic liver disease. The aim of the present study was to investigate the effect of surgical portosystemic shunt on plasma glucose and insulin responses to glucose administration in a group of cirrhotic patients. For this purpose 10 cirrhotic subjects (8 males and 2 females) aged 42 to 65 years underwent an oral glucose tolerance test (OGTT, 75 g), and an intravenous glucose tolerance test (IVGTT, 0.33 g/kg) before and after undergoing a side-to side portocaval anastomosis (PCS). 6 noncirrhotic, nondiabetic patients matched for sex, age and body weight who underwent abdominal vascular surgery served as controls. In cirrhotic subjects, the PCS resulted in: increased plasma glucose and insulin levels during OGTT; decreased C-peptide level during OGTT; unmodified plasma glucose and insulin concentrations during IVGTT. In control subjects the abdominal surgery did not affect plasma glucose and insulin responses to oral or intravenous glucose loads. These results suggest that in cirrhotic subjects surgical portocaval shunt results in: deterioration of oral but not intravenous glucose tolerance, due to an escape of ingested glucose from the liver; increased peripheral insulin response to oral glucose administration as a consequence of reduction in hepatic removal of the hormone; and decreased pancreatic response to oral glucose due possibly to a greater feed back inhibition of beta-cell. These events seem to be a consequence of the shunt per se and not of a deterioration of hepatocellular function.     

The effect of arterialization of the liver on gastric secretion of dogs with portacaval shunts

Summary Arterialization of the liver in dogs with portacaval shunts and Heidenhain pouches did not reduce the hypersecretion that usually occurs following the establishment of a portacaval shunt alone.The work described here was supported by the Donald J. Cowling and Jay and Rose Phillips Fund for Surgical Research and the U.S.P.H.S. Peptic Ulcer Grant A-2150.     

Pulmonary hypertension associated with primary biliary cirrhosis in the absence of portal hypertension: a case report.

Pulmonary hypertension is well described in association with portal hypertension of any cause including end stage primary biliary cirrhosis (PBC). The essential feature of this association is the presence of portosystemic shunting, including surgically created shunts. A patient with primary pulmonary hypertension and PBC without portal hypertension is reported. This suggests that primary pulmonary hypertension may be associated with PBC in the absence of portal hypertension. Decisions regarding appropriate organ transplantation may depend on whether pulmonary hypertension is primary or secondary to portal hypertension.     

经颈静脉肝内门体分流术治疗食道静脉曲张破裂出血

采用经颈静脉肝内门体分流术(TIPSS)治疗5例肝硬化门静脉高压症(CPH)食管静脉反复破裂出血病人,平均门静脉压力由术前3.6±0.7kPa,降至术后1.73±0.35kPa,术后24小时全部病例出血均停止,门静脉血流阻力系数降低,曲张静脉消失或减轻。TIPSS 治疗门脉高压食管静脉反复性出血具有损伤小、合并症少,近期疗效确切等特点,是治疗门脉高压食道静脉破裂出血的首选方法。     

Pulmonary hypertension associated with COPD

Although the prevalence of pulmonary hypertension (PH) in individuals with chronic obstructive pulmonary disease (COPD) is not known precisely, approximately 10%–30% of patients with moderate to severe COPD have elevated pulmonary pressures. The vast majority of PH associated with COPD is mild to moderate and severe PH occurs in <5% of patients. When COPD is associated with PH, both mortality and morbidity are increased. There are no clinical or physical examination findings that accurately identify patients with underlying PH. Radiographic imaging findings are specific but not sensitive indicators of PH. Echocardiography is the principle noninvasive diagnostic test but may be technically limited in a significant proportion of patients with COPD. Right heart catheterization is required for accurate measurement of pulmonary pressures. The combined effects of inflammation, endothelial cell dysfunction, and angiogenesis appear to contribute to the development of PH associated with COPD. Systemic vasodilators have not been found to be effective therapy. Selective pulmonary vasodilators including inhaled nitric oxide and phosphodiesterase inhibitors are promising treatments for patients with COPD associated PH but further evaluation of these medications is needed prior to their routine use.     

Pulmonary hypertension associated with long-standing thrombocytosis

A case of thromboembolic pulmonary hypertension associated with long-standing thrombocytosis is presented. In this patient we found a significant local pulmonary platelet activation and thrombin generation as indicated by the existence of a transpulmonary gradient for thromboxane A2, beta thromboglobulin and fibrinopeptide A. Prolonged heparin and acetylsalicylic acid treatment resulted in improvement of clinical and hemodynamic conditions. These findings support the usefulness of anticoagulating and antiaggregating therapy in selected cases of pulmonary hypertension.     

Pulmonary hypertension associated with nonketotic hyperglycinaemia

Nonketotic hyperglycinaemia (NKH) is an autosomal recessive disorder characterized by defective glycine degradation by the mitochondrial glycine cleavage system. The clinical features include lethargy, hypotonia, apnoea, seizures and severe psychomotor retardation, all attributed to the accumulation of glycine in the nervous system. Pulmonary hypertension (PHN) has not been reported in NKH. We describe four patients with NKH who had PHN in addition to the characteristic manifestations of NKH. This newly recognized association might provide additional insight into the underlying pathophysiology of PHN.