Cases reported "Hypertension, Portal"

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1/112. Improvement of pulmonary hypertension after liver transplantation.

    Pulmonary hypertension at the end stage of chronic liver disease is not an uncommon situation. This association termed portopulmonary hypertension raises the question of the feasibility of performing orthotopic liver transplantation (OLT). In the case reported herein, there was a favorable outcome after OLT, even though the mean pulmonary artery pressure (MPAP) before transplantation was increased to 45 mm Hg. Before OLT, the cardiac index (CI) was considerably elevated (7.69 L/min/m2), giving evidence of a marked hyperdynamic circulatory state. The CI decreased significantly after OLT (3.38 L/min/m2), and this produced a significant decrease in the MPAP. Our observation suggests that portopulmonary hypertension due to a marked increase in the CI can be managed successfully by OLT.
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2/112. Progressive splenomegaly after epoprostenol therapy in portopulmonary hypertension.

    patients with end-stage liver failure, portal hypertension, and associated pulmonary artery hypertension (portopulmonary hypertension [PPHTN]) have a high mortality when undergoing liver transplantation. Successful transplantation in these patients may depend on efforts to reduce pulmonary artery pressure (PAP). To this end, a number of centers are using a continuous intravenous (IV) infusion of epoprostenol, which has been shown to improve symptoms, extend life span, and reduce PAP in patients with primary pulmonary hypertension. We report four cases in which treatment of patients with PPHTN with continuous IV epoprostenol was followed by the development of progressive splenomegaly, with worsening thrombocytopenia and leukopenia. This finding may limit the usefulness of epoprostenol in PPHTN and influence the timing of transplantation in such patients.
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3/112. Massive esophageal variceal hemorrhage triggered by complicated endotracheal intubation.

    Esophageal variceal hemorrhage is frequently a catastrophic event. The specific events that trigger variceal rupture are not well understood. Acute elevations in systemic blood pressure and increased splanchnic blood flow, however, may lead to increased intravariceal pressure followed by variceal rupture and hemorrhage. This report describes a strong temporal association between complicated endotracheal intubation and abrupt onset of life-threatening variceal hemorrhage. A 52-year-old man with a history of portal hypertension was intubated emergently for airway protection because of respiratory insufficiency due to sepsis. intubation was complicated by initial inadvertent esophageal intubation and by a peak mean arterial blood pressure of 155 mmHg. At the conclusion of the procedure, the patient sustained large volume hematemesis due to esophageal variceal rupture. This case suggests a risk of triggering variceal hemorrhage as a result of intubation-induced increase in blood pressure. A number of agents, including fentanyl, have been shown to be effective in attenuating the cardiovascular response to intubation. This case report provides strong evidence in support of administering fentanyl, or a suitable alternative adjunctive medication, before intubation of patients with documented portal hypertension and a history of esophageal variceal hemorrhage.
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4/112. isosorbide-5-mononitrate in the treatment of pulmonary hypertension associated with portal hypertension.

    Pulmonary hypertension is uncommonly associated with portal hypertension. The current approach for the management of pulmonary hypertension involves the use of vasodilators in patients who show vascular responsiveness during an acute challenge. Since the association of portal hypertension with pulmonary hypertension is very seldomly presented, its optimal therapy has not been defined. Moreover, calcium-channel blockers, which are usually used in pulmonary hypertension treatment, may exert a deleterious effect on portal hypertension. Therefore, the search for drugs that may be active under both conditions has important clinical implications. This report presents the case of a patient with portal hypertension-associated pulmonary artery hypertension that was effectively treated with isosorbide-5-mononitrate (Is-5-Mn). The patient had severe portal hypertension (hepatic venous pressure gradient=14.5 mmHg) and pulmonary hypertension (mean pulmonary artery pressure (PAP)=50 mmHg). Acute administration of prostacyclin and nitric oxide elicited a significant reduction in both PAP and pulmonary vascular resistance (PVR), an effect that was also achieved with Is-5-Mn. The patient was treated with 40 mg Is-5-Mn twice daily and a haemodynamic study performed 6 months later showed that the reduction in both PAP and PVR persisted.
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5/112. Use of aerosolized inhaled epoprostenol in the treatment of portopulmonary hypertension.

    BACKGROUND: Portopulmonary hypertension is a known complication in the liver transplant candidate. Intravenous epoprostenol has been demonstrated to decrease pulmonary artery pressures and possibly remodel right ventricle geometry. methods: In this report, we document the efficacy of inhaled aerosolized epoprostenol in a patient with portopulmonary hypertension. The effect was of rapid onset and offset. RESULTS: After 10 min of delivery, mean pulmonary artery pressure decreased 26%; cardiac output increased by 22%; pulmonary vascular resistance decreased by 42%; and the transpulmonary gradient decreased by 29%. There were no untoward side effects. CONCLUSION: The inhaled route of delivery of epoprostenol is potential alternative for the acute therapy of portpulmonary hypertension.
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6/112. Idiopathic splenic vein stenosis: a cause of gastric variceal hemorrhage.

    We report the case of a patient with isolated gastric variceal bleeding. obesity precluded the use of noninvasive means for assessing splenic vein patency. splenic vein stenosis was diagnosed by transhepatic portal and splenic venography with pressure measurements. A cause for the stenosis could not be found. splenectomy was used as a curative measure.
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7/112. Successful use of continuous intravenous prostacyclin in a patient with severe portopulmonary hypertension.

    INTRODUCTION: Portopulmonary hypertension, defined by a mean pulmonary artery pressure > 25 mm Hg in the presence of normal pulmonary capillary wedge pressure and portal hypertension, is a known complication of end-stage liver disease that has been associated with high morbidity and mortality at the time of liver transplantation. DESIGN: Descriptive case report. PATIENT: A 32 year old male patient suffering from end-stage hepatitis c liver cirrhosis presented with severe portopulmonary hypertension. At presentation the following pulmonary hemodynamics were measured: systolic pulmonary artery pressure (PAP) 76 mm Hg, mean PAP 42 mm Hg, pulmonary vascular resistance index (PVRI) 931, pulmonary capillary wedge pressure (PCWP) 9 mm Hg, and cardiac output (CO) 4.03 l/min. INTERVENTION: After acute hemodynamic testing the patient received 8 ng/kg/min epoprostenol (prostacyclin) by continuous intravenous infusion with an infusion pump. Hemodynamic evaluation was performed monthly by transthoracic echocardiography and right heart catheterisation after 5 months. RESULTS: After 5 months of continuous therapy right heart catheterisation revealed the following hemodynamics: systolic pulmonary artery pressure (PAP) 59 mm Hg, mean PAP 32 mm Hg, pulmonary vascular resistance index (PVRI) 561, pulmonary capillary wedge pressure (PCWP) 7 mm Hg, and cardiac output (CO) 6.95 l/min. This presents a decrease in systolic pulmonary artery pressure of approximately 22%, a decrease in mean pulmonary artery pressure of approximately 30%, a decrease in pulmonary vascular resistance of approximately 40% and an increase in cardiac output of approximately 73%. echocardiography demonstrated a decrease in estimated systolic pulmonary artery pressure of about 37% after 8 months of therapy. No complications were observed during epoprostenol therapy. CONCLUSION: In this adult patient suffering from end-stage liver disease and portopulmonary hypertension, administration of continuous intravenous epoprostenol resulted in significant reduction of pulmonary hypertension and therefore in acceptance for orthotopic liver transplantation. Utilisation of this new therapeutic strategy might be a helpful pharmacological tool for patients with portopulmonary hypertension to make them acceptable for orthotopic liver transplantation.
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8/112. Transjugular intrahepatic portosystemic shunt performed in a 2-year-old infant with uncontrollable intestinal bleeding.

    Although transjugular intrahepatic portosystemic shunt (TIPS) is widely accepted in adults, there have been few successful reports in infants. The authors describe a 2-year-old boy with postoperative biliary atresia who underwent TIPS for uncontrollable lower intestinal bleeding and achieved hemostasis. Massive melena developed, which was not controlled by conservative therapy and devascularization. A shunt was created between the right hepatic vein and the right portal vein with a 6- x 50-mm Wallstent. After TIPS, the porto-systemic pressure gradient decreased from 15 to 11 mm Hg, and collaterals markedly reduced. He has not had recurrent bleeding for over 7 months. TIPS may be a technically feasible and effective treatment to control intestinal bleeding in infants. However, further experience and long-term follow-up will be required.
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9/112. Progressive portopulmonary hypertension after liver transplantation treated with epoprostenol.

    Portopulmonary hypertension (PPHTN) is an uncommon complication of advanced liver disease. epoprostenol has been effective in the treatment of PPHTN and has been used as a bridge to orthotopic liver transplantation (OLT). The role of OLT in the reversal of PPHTN is unclear. We report a case of severe PPHTN (mean pulmonary artery pressure of 45 mm Hg) that progressed after OLT. Acute dosing with epoprostenol improved the pulmonary vascular resistance by 55% and the cardiac index by 134%. Hemodynamic and symptomatic improvements were maintained after 18 months of long-term treatment with epoprostenol. This is the first reported case of a successful favorable outcome after treatment for progressive PPHTN after OLT. Our case report complements previous reports by highlighting the potential effective use of epoprostenol as a definitive treatment for PPHTN.
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10/112. Disappearance of "pseudocholangiocarcinoma sign" in a patient with portal hypertension due to complete thrombosis of left portal vein and main portal vein web after web dilatation and transjugular intrahepatic portosystemic shunt.

    The main portal vein web is probably a consequence of portal vein thrombosis, which is a very rare cause of portal hypertension. Principal manifestations are related to the degree of portal hypertension. In the literature, no data has been found for the treatment modality of portal vein web. We report, herein, the clinical and laboratory findings of a 38-year-old woman with angiographically proven incomplete main portal vein web and complete thrombotic occlusion of the left portal vein causing pseudocholangiocarcinoma sign (PCCS) on the common bile duct. She was treated by transjugular intrahepatic portosystemic shunt (TIPS) and membrane dilatation, which resulted in complete disappearance of collaterals and PCCS. It appears that TIPS and balloon dilatation of the portal vein web via transjugular approach was effective in decreasing portal pressure and its consequences.
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