1/50. hepatopulmonary syndrome in gaucher disease with right-to-left shunt: evaluation and measurement using Tc-99m MAA.hepatomegaly is a common manifestation in gaucher disease. In some patients with the disease, hepatic fibrosis and portal hypertension are observed. A patient with gaucher disease with the hepatopulmonary syndrome associated with severe cyanosis and hypoxemia was examined for intrapulmonary right-to-left shunt using Tc-99m MAA. Quantitative evaluation revealed an approximately 50% right to left shunt as indicated by Tc-99m MAA activity in the lungs and systemic organs.- - - - - - - - - - ranking = 1keywords = hepatic (Clic here for more details about this article) |
2/50. Severe intrapulmonary shunting associated with metastatic carcinoid.A 37-year-old woman with a 10-year history of metastatic carcinoid presented to her oncologist with increased dyspnea. Further evaluation revealed hypoxemia and intrapulmonary vasodilatation. We describe a case of hepatopulmonary-like physiology associated with metastatic carcinoid in a patient with intact liver function. To our knowledge, this is the first documented case of intrapulmonary shunting and hepatopulmonary-like physiology associated with metastatic carcinoid.- - - - - - - - - - ranking = 16.0771956919keywords = liver (Clic here for more details about this article) |
3/50. hepatopulmonary syndrome associated with cardiorespiratory disease.BACKGROUND/AIMS: hepatopulmonary syndrome is defined as a clinical triad including chronic liver disease, abnormal pulmonary gas exchange resulting ultimately in profound arterial hypoxaemia, and evidence of intrapulmonary vascular dilatations. We report five patients with liver cirrhosis diagnosed with hepatopulmonary syndrome who had associated chronic obstructive or restrictive respiratory diseases. methods: Clinical, radiographic and constrast-enhanced echocardiographic findings, and systemic and pulmonary haemodynamic and gas exchange, including ventilation-perfusion distributions, measurements were assessed in all five patients. RESULTS: echocardiography was consistent with the presence of intrapulmonary vasodilation without intracardiac abnormalities, and high resolution computed tomographic scan features were compatible with clinical (3 cases) or histopathological diagnoses (2 cases) of associated respiratory disorders. The most common prominent functional findings were moderate to severe arterial hypoxaemia, caused by moderately to severely increased intrapulmonary shunting and/or mild to moderate low ventilation-perfusion areas, and hypocarbia along with an increased cardiac output and a low pulmonary artery pressure and vascular resistance. CONCLUSIONS: These functional characteristics, classically reported in the setting of clinically stable, uncomplicated hepatopulmonary syndrome, conform to a distinctively unique, chronic gas exchange pattern. Equally important, these pulmonary haemodynamic-gas exchange hallmarks are not influenced by the co-existence of chronic cardiorespiratory disease states. These data may have clinical relevance for elective indication of hepatic transplantation in patients with life-threatening hepatopulmonary syndrome.- - - - - - - - - - ranking = 577.88642490269keywords = liver cirrhosis, cirrhosis, liver, hepatic (Clic here for more details about this article) |
4/50. Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis.We report the case of a patient with liver cirrhosis who successively developed hepatopulmonary syndrome and portopulmonary hypertension. Initially, the patient presented with severe dyspnea and hypoxemia at rest. technetium-99 macroaggregated albumin lung perfusion scan demonstrated right-to-left shunt, and hemodynamic study revealed a hyperdynamic state with low pulmonary vascular resistance, thus confirming the diagnosis of hepatopulmonary syndrome. More than 2 years after the onset of pulmonary symptoms, a marked improvement in dyspnea and gas exchange was observed. lung perfusion scan did not disclose any right-to-left shunt and right-sided heart catheterization showed evidence of severe pulmonary hypertension. We conclude that hepatopulmonary syndrome and portopulmonary hypertension are not mutually exclusive. We hypothesize that, by reversing the phenomenon of intrapulmonary vasodilatation, the development of portopulmonary hypertension interfered with each of the potential causes of hypoxemia in hepatopulmonary syndrome (ventilation-perfusion inequalities, intrapulmonary shunting, oxygen diffusion limitation) and, as a result, led to a correction of hypoxemia.- - - - - - - - - - ranking = 1492.8059596157keywords = liver cirrhosis, cirrhosis, liver (Clic here for more details about this article) |
5/50. Late recurrence and rapid evolution of severe hepatopulmonary syndrome after liver transplantation.recurrence of hepatopulmonary syndrome (HPS) after orthotopic liver transplantation (OLT) in an adult has never been reported. We describe a 23-year-old woman who initially underwent OLT because of debilitating and severe HPS associated with nonalcoholic steatohepatitis (NASH). Although the clinical resolution of HPS was well documented day 117 post-OLT, the reappearance of NASH was documented by liver biopsy. Severe hypoxemia because of recurrent HPS rapidly evolved beginning approximately day 700 post-OLT. Retransplantation was attempted, but the patient died post-OLT of sepsis and/or multiorgan failure.- - - - - - - - - - ranking = 96.4631741514keywords = liver (Clic here for more details about this article) |
6/50. hepatopulmonary syndrome: a rare complication of chronic liver disease in children.An 11-year-old boy with congenital hepatic fibrosis presented with cyanosis at the National University Hospital. Echocardiogram revealed a structurally normal heart with good ventricular function. A pulmonary cause of his cyanosis was suggested on macroaggregated albumin scan and selective pulmonary artery angiogram. Arterial hypoxaemia secondary to intrapulmonary arteriovenous shunting in chronic liver cirrhosis can lead to permanent cyanosis. The potential for a complete reversal of this condition after liver transplantation indicates that arterial hypoxaemia, rather than being a contraindication, should be a reason for early liver transplantation.- - - - - - - - - - ranking = 658.27240336219keywords = liver cirrhosis, cirrhosis, liver, hepatic (Clic here for more details about this article) |
7/50. Hypoxaemia--think of the liver! Every internist should be aware of the hepatopulmonary syndrome.hepatopulmonary syndrome is characterised by arterial hypoxaemia, liver disease, and intrapulmonary vascular dilatation. A case is reported in which severe hypoxaemia, detected by chance, led to the diagnosis of liver disease and hepatopulmonary syndrome.- - - - - - - - - - ranking = 96.4631741514keywords = liver (Clic here for more details about this article) |
8/50. Severe portopulmonary hypertension after liver transplantation in a patient with preexisting hepatopulmonary syndrome.BACKGROUND: Portopulmonary hypertension and hepatopulmonary syndrome have been considered mutually exclusive pulmonary vascular disorders in liver disease states. methods: This current report describes a middle-aged patient, a candidate for liver transplantation, diagnosed with hepatopulmonary syndrome on the basis of clinical, echocardiographic and gas exchange criteria. Unusually high pulmonary pressures were observed at liver transplantation, performed 6 months after the initial diagnosis of hepatopulmonary syndrome. Three months later, the patient developed severe pulmonary hypertension and died of right ventricular failure during a second attempted liver transplantation. Postmortem histologic findings in the lung confirmed the presence of plexogenic pulmonary arteriopathy. CONCLUSION: This case illustrates the potential occurrence of hepatopulmonary syndrome and portopulmonary hypertension in the same patient, suggesting that the presence of hepatopulmonary syndrome may not preclude the development of portopulmonary hypertension.- - - - - - - - - - ranking = 128.6175655352keywords = liver (Clic here for more details about this article) |
9/50. hepatopulmonary syndrome and venous emboli causing intracerebral hemorrhages after liver transplantation: a case report.Increasing experience has fostered the acceptance of liver transplantation as a treatment for patients with hepatopulmonary syndrome. morbidity and mortality is most commonly attributed to progressive arterial hypoxemia postoperatively. A cerebral hemorrhage has been reported in one patient with hepatopulmonary syndrome after transplantation. However, a postmortem examination of the brain was not performed and the pathogenesis or type of cerebral hemorrhage was undefined. We report on a patient with severe hepatopulmonary syndrome who developed multiple intracranial hemorrhages after transplantation. The intracerebral hemorrhages were most consistent with an embolic etiology on postmortem examination. We postulate that venous embolization, caused by the manipulation of a Swan Ganz catheter in a thrombosed central vein, resulted in pulmonary emboli that passed through dilated intrapulmonary vessels into the cerebral microcirculation. Special attention to central venous catheters and avoidance of manipulation may be warranted in subjects with severe hepatopulmonary syndrome after liver transplantation.- - - - - - - - - - ranking = 96.4631741514keywords = liver (Clic here for more details about this article) |
10/50. hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty.Reports show that hepatopulmonary syndrome mostly occurs in the setting of advanced hepatic dysfunction, with the associated vasoactive substance imbalance believed to be responsible for its pathogenesis. However, hepatopulmonary syndrome has also been reported in cases of mild hepatic dysfunction or noncirrhotic portal hypertension, indicating that portal hypertension also plays a part in the pathogenesis. liver transplantation remains the only therapeutic option of proven benefit. We describe 2 cases of hepatopulmonary syndrome in the setting of inferior vena cava (suprahepatic) obstruction, but with minimal hepatic dysfunction. After balloon cavoplasty, 1 patient showed, in addition to improvement of the features of hepatic outflow obstruction, significant reduction of dyspnea, cyanosis, and hypoxemia with arterial blood gas normalization within 2 weeks and intrapulmonary shunt reversal within 8 weeks. This implies that hemodynamic alterations (such as portal hypertension) independently contribute to the pathogenesis of hepatopulmonary syndrome in at least some of the cases. Therapies aimed at correcting these abnormal hemodynamics may be important in the treatment of this condition, especially when the hepatic functional status by itself does not warrant a liver transplant.- - - - - - - - - - ranking = 22.0771956919keywords = liver, hepatic (Clic here for more details about this article) |
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