Portopulmonary hypertension(PPHTN)is a known complication of cirrhosis.Moderate-to-severe PPHTN implies an extremely poor prognosis.It occurs in 5%-10%of patients referred for liver transplantation(LT),and probably wi...Portopulmonary hypertension(PPHTN)is a known complication of cirrhosis.Moderate-to-severe PPHTN implies an extremely poor prognosis.It occurs in 5%-10%of patients referred for liver transplantation(LT),and probably with an higher incidence in patients with large portosystemic shunts.Patients with moderate-tosevere pulmonary hypertension have been previously excluded from LT because of the extremely high surgical risk and since the post-transplant outcome reported was poor.Recently,new perspectives in the management of patients with portopulmonary hypertension are emerging.In fact,some pulmonary vasoactive drugs have become routine in the treatment of patients with idiopathic pulmonary hypertension.These drugs,particularly epoprostenol,have been recently introduced in the treatment of patients with PPHTN,and have been shown to be effective in reducing pulmonary artery pressure as well as pulmonary vascular resistances.Furthermore,recent studies seem to demonstrate that treatment with pulmonary vasoactive drugs could allow liver transplantation with acceptable surgical risks and excellent survival.Although there are not large series nor prospective studies addressing this topic,the clinical scenario of patients with PPHTN seems to be positively changing.展开更多
BACKGROUND:Cirrhosis is associated with several extrahepatic manifestations including portopulmonary hypertension (PPHT).Recent data suggest that endothelins (ETs) are related to the pathophysiology of PPHT.The study ...BACKGROUND:Cirrhosis is associated with several extrahepatic manifestations including portopulmonary hypertension (PPHT).Recent data suggest that endothelins (ETs) are related to the pathophysiology of PPHT.The study aimed to measure serum ET levels in hospitalized cirrhotic patients and to determine their association with PPHT and patient outcome.METHODS:Fifty-seven cirrhotic patients [43 males;median age 58 (28-87) years] underwent Doppler echocardiography.Patients with systolic pulmonary arterial pressure ≥40 mmHg and pulmonary acceleration time <100 ms were deemed to have PPHT.ET-1,2,and 3 serum levels were measured with an ELISA assay.All-cause mortality was recorded over a median period of 24 months.RESULTS:Nine out of 57 patients (15.8%) had PPHT.Among various clinical variables,only autoimmune hepatitis was associated with PPHT (OR=11.5;95% CI,1.58-83.4;P=0.01).ET-1 levels [9.1 (1.6-20.7) vs 2.5 (1.4-9.2) pg/mL,P=0.02] and the ET-1/ET-3 ratio [4.73 (0.9-22.4) vs 1.6 (0.3-10.7),P=0.02] were significantly higher in patients with PPHT than in those without.ET-2 and ET-3 levels did not differ between the two groups.There was no difference in survival between the two groups,although ET-1 levels were associated with an adverse outcome in Cox regression analysis (HR=1.11;95% CI,1.02-1.22;P=0.02 per unit increase in ET-1).CONCLUSION:Our data suggest that ET-1 and the ET-1/ET-3 ratio are elevated in patients with PPHT and that ET-1 is associated with a poor outcome irrespective of PPHT.展开更多
BACKGROUND: Portopulmonary hypertension (PPH) is difficult to recognize in the early and middle stages because it is frequently asymptomatic. As right ventricular function is impaired in patients with moderate and sev...BACKGROUND: Portopulmonary hypertension (PPH) is difficult to recognize in the early and middle stages because it is frequently asymptomatic. As right ventricular function is impaired in patients with moderate and severe PPH, any dramatic hemodynamic changes in liver transplantation or other procedures may result in death from pulmonary and cardiac events. In this study, we investigated the prevalence of PPH in patients with portal hypertension (PHT) mainly caused by hepatitis B virus, and evaluated the effect of 2-dimensional Doppler echocardiography (2D-ECHO) in screening for PPH. METHODS: One hundred and five PHT patients received transthoracic 2D-ECHO preoperatively, systolic pulmonary arterial pressure (SPAP, normal range <30 mmHg) and pulmonary acceleration time (PAT, normal range >= 120 msec) were measured to screen for PPH (positive result: SPAP >= 30 mmHg and/or PAT <100 msec). Subsequently, pulmonary hemodynamic parameters were measured by right heart catheterization (RHC) for definitive diagnosis of PPH. The results of the two methods were compared to assess the screening effect of 2D-ECHO. RESULTS: The prevalence of PPH in this study was 3.8% (4/105). About 90% (95/105) of patients had a detectable tricuspid regurgitation by 2D-ECHO and the mean SPAP was 27.7 +/- 5.9 mmHg. Twenty-two of these 95 patients had an SPAP >30 mmHg. The mean PAT of all patients was 140 23 msec and 5 were <100 msec. Twenty-two patients were screened out by 2D-ECHO and 4 were diagnosed by RHC. A positive significant correlation (r=0.55, P<0.01) was found between SPAP measured by 2D-ECHO and mean pulmonary artery pressure (MPAP) measured by RHC, and a weak but significant negative correlation (r=-0.27, P=0.005) existed between PAT and pulmonary vascular resistance (PVR). The sensitivity, specificity, agreement rate, positive predictive value and negative predictive value of the screening test were 100%, 82%, 83%, 18% and 100%, respectively. CONCLUSIONS: The prevalence of PPH in this study is lower than in Western countries. As a screening test, 2D-ECHO has very high sensitivity and negative predictive value. A negative test result can directly be used to exclude PPH, while a positive result should be confirmed by RHC.展开更多
Pulmonary vascular disorders including portopulmonary hypertension(PoPHT) are among the common complications of liver disease and are prognostically significant. Survival is very low without medical treatment and live...Pulmonary vascular disorders including portopulmonary hypertension(PoPHT) are among the common complications of liver disease and are prognostically significant. Survival is very low without medical treatment and liver transplantation. With advances in medical therapy for elevated pulmonary artery pressure(PAP) and liver transplant surgery, survival of patients with Po PHT and advanced liver disease is significantly improved. Because of the prognostic significance of Po PHT and the limited donor pool, a comprehensive preoperative cardio-pulmonary assessment is of great importance in cirrhotic patients prior to transplant surgery. Therefore, a detailed transthoracic Doppler echocardiographic examination must be an essential component of this evaluation. Patients with mild Po PHT can safely undergo liver transplant surgery. In cases of moderate to severe Po PHT, right heart catheterization(RHC) should be performed. In patients with moderate to severe Po PHT on RHC(mean PAP 35-45 mm Hg), vasodilator therapy should be attempted. Liver transplantation should be encouraged in cases that demonstrate a positive response. Bridging therapy with specific pulmonary arterial hypertension treatment agents should be considered until the transplant surgery and should be continued during the peri- and post-operative periods as needed.展开更多
BACKGROUND Portopulmonary hypertension(PoPH) is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for LT...BACKGROUND Portopulmonary hypertension(PoPH) is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for LT.Many patients with liver disease require but cannot receive LT due to severe PoPH and eventually died.We report a patient with severe PoPH who underwent successful LT and had near normal pulmonary pressure without drug treatment.CASE SUMMARY A 39-year-old woman was hospitalized with the chief complaint of jaundice and exertional dyspnea and fatigue.Caroli disease and liver cirrhosis was diagnosed 6 years previously.Her liver disease met the criteria for LT.However, right heart catheterization showed that her mean pulmonary artery pressure was increased at 50 mmHg, pulmonary vascular resistance was 460 dyn?s/cm^5 and pulmonary artery wedge pressure was 20 mmHg, which may have been the reasons for her chief complaint.The patient was diagnosed with severe PoPH and was not listed for LT immediately.After 5 mo of pharmacotherapy, her severe PoPH was moderate, and she underwent successful LT.Pulmonary artery pressure steadily decreased according to post-operative echocardiographic monitoring and drugs have been discontinued for a month.CONCLUSION The safety of LT can be greatly improved by reducing mean pulmonary artery pressure to a low level, and LT may cure PoPH.展开更多
Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are two frequent complications of liver disease, with prevalence among liver transplant candidates of 6% and 10%, respectively. Both conditions res...Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are two frequent complications of liver disease, with prevalence among liver transplant candidates of 6% and 10%, respectively. Both conditions result from a lack of hepatic clearance of vasoactive substances produced in the splanchnic territory. Subsequently, these substances cause mainly pulmonary vascular remodeling and some degree of vasoconstriction in POPH with resulting elevated pulmonary pressure and right ventricular dysfunction. In HPS the vasoactive mediators cause intrapulmonary shunts with hypoxemia. Medical treatment is disappointing overall. Whereas liver transplantation (LT) results in the disappearance of HPS within six to twelve months, its effect on POPH is highly unpredictable. Modern strategies in managing HPS and POPH rely on a thorough screening and grading of the disease’s severity, in order to tailor the appropriate therapy and select only the patients who will benefit from LT. The anesthesiologist plays a central role in managing these high-risk patients. Indeed, the important hemodynamic and respiratory modifications of the perioperative period must be avoided through continuation of the preoperatively initiated drugs, appropriate intraoperative monitoring and proper hemodynamic and respiratory therapies.展开更多
BACKGROUND: Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or withou...BACKGROUND: Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH. DATA SOURCES: An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension. RESULTS: Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH. CONCLUSIONS: After an accurate diagnosis of PPH treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.展开更多
Intravenous epoprostenol is recommended for World Health Organization functional class(WHO-FC) Ⅳ patients with pulmonary arterial hypertension(PAH) in the latest guidelines. However, in portopulmonary hypertension(Po...Intravenous epoprostenol is recommended for World Health Organization functional class(WHO-FC) Ⅳ patients with pulmonary arterial hypertension(PAH) in the latest guidelines. However, in portopulmonary hypertension(PoP H) patients, advanced liver dysfunction and/or thrombocytopenia often makes the use of intravenous epoprostenol challenging. Here we report the cases of two WHO-FC Ⅳ PoP H patients who were successfully treated with a combination of two oral vasodilators used to treat PAH: ambrisentan and tadalafil. Oral vasodilator therapy using a combination of ambrisentan and tadalafil may be a safe and effective therapeutic option for WHO-FC Ⅳ PoP H patients and should be considered for selected patients with severe and rapidly progressing PoP H.展开更多
Portopulmonary hypertension (PPH) is a form of pulmonary arterial hypertension, characterized by elevation of pulmonary arterial pressure due to increased resistance to pulmonary blood flow. It is a consequence of por...Portopulmonary hypertension (PPH) is a form of pulmonary arterial hypertension, characterized by elevation of pulmonary arterial pressure due to increased resistance to pulmonary blood flow. It is a consequence of portal hypertension, associated or not with liver disease. A retrospective study of patients undergoing liver transplantation was carried out in the Liver Transplantation Unit of the Clinics Hospital of the State University of Campinas (UNICAMP) from 1991 to 2012, covering 389 transplants. Although 50 (12.9%) patients presented with mean pulmonary arterial pressure (MPAP) > 25 mmHg, only one case (0.25%) met the diagnostic criteria for PPH: a 43-year-old female patient with advanced liver disease, caused by primary biliary cirrhosis with portal hypertension, diagnosed as mild portopulmonary hypertension and diagnosed in the operating room. PPH is a serious complication of portal hypertension. It is related to the great difficulty in diagnosis and the high mortality rate due to right heart failure. PPH is an entity with a low frequency of occurrence in individuals undergoing liver transplantation, although there is a wide variation in the prevalence rate presented in the literature and the definitive diagnosis should be confirmed with right heart catheterization.展开更多
文摘Portopulmonary hypertension(PPHTN)is a known complication of cirrhosis.Moderate-to-severe PPHTN implies an extremely poor prognosis.It occurs in 5%-10%of patients referred for liver transplantation(LT),and probably with an higher incidence in patients with large portosystemic shunts.Patients with moderate-tosevere pulmonary hypertension have been previously excluded from LT because of the extremely high surgical risk and since the post-transplant outcome reported was poor.Recently,new perspectives in the management of patients with portopulmonary hypertension are emerging.In fact,some pulmonary vasoactive drugs have become routine in the treatment of patients with idiopathic pulmonary hypertension.These drugs,particularly epoprostenol,have been recently introduced in the treatment of patients with PPHTN,and have been shown to be effective in reducing pulmonary artery pressure as well as pulmonary vascular resistances.Furthermore,recent studies seem to demonstrate that treatment with pulmonary vasoactive drugs could allow liver transplantation with acceptable surgical risks and excellent survival.Although there are not large series nor prospective studies addressing this topic,the clinical scenario of patients with PPHTN seems to be positively changing.
基金supported by a grant from the Special Account for Research Funds(ELKE)of the National and Kapodistrian University of Athens,Greece(7493)
文摘BACKGROUND:Cirrhosis is associated with several extrahepatic manifestations including portopulmonary hypertension (PPHT).Recent data suggest that endothelins (ETs) are related to the pathophysiology of PPHT.The study aimed to measure serum ET levels in hospitalized cirrhotic patients and to determine their association with PPHT and patient outcome.METHODS:Fifty-seven cirrhotic patients [43 males;median age 58 (28-87) years] underwent Doppler echocardiography.Patients with systolic pulmonary arterial pressure ≥40 mmHg and pulmonary acceleration time <100 ms were deemed to have PPHT.ET-1,2,and 3 serum levels were measured with an ELISA assay.All-cause mortality was recorded over a median period of 24 months.RESULTS:Nine out of 57 patients (15.8%) had PPHT.Among various clinical variables,only autoimmune hepatitis was associated with PPHT (OR=11.5;95% CI,1.58-83.4;P=0.01).ET-1 levels [9.1 (1.6-20.7) vs 2.5 (1.4-9.2) pg/mL,P=0.02] and the ET-1/ET-3 ratio [4.73 (0.9-22.4) vs 1.6 (0.3-10.7),P=0.02] were significantly higher in patients with PPHT than in those without.ET-2 and ET-3 levels did not differ between the two groups.There was no difference in survival between the two groups,although ET-1 levels were associated with an adverse outcome in Cox regression analysis (HR=1.11;95% CI,1.02-1.22;P=0.02 per unit increase in ET-1).CONCLUSION:Our data suggest that ET-1 and the ET-1/ET-3 ratio are elevated in patients with PPHT and that ET-1 is associated with a poor outcome irrespective of PPHT.
基金supported by a grant from the Shanghai Municipal Health Bureau(No.054041)
文摘BACKGROUND: Portopulmonary hypertension (PPH) is difficult to recognize in the early and middle stages because it is frequently asymptomatic. As right ventricular function is impaired in patients with moderate and severe PPH, any dramatic hemodynamic changes in liver transplantation or other procedures may result in death from pulmonary and cardiac events. In this study, we investigated the prevalence of PPH in patients with portal hypertension (PHT) mainly caused by hepatitis B virus, and evaluated the effect of 2-dimensional Doppler echocardiography (2D-ECHO) in screening for PPH. METHODS: One hundred and five PHT patients received transthoracic 2D-ECHO preoperatively, systolic pulmonary arterial pressure (SPAP, normal range <30 mmHg) and pulmonary acceleration time (PAT, normal range >= 120 msec) were measured to screen for PPH (positive result: SPAP >= 30 mmHg and/or PAT <100 msec). Subsequently, pulmonary hemodynamic parameters were measured by right heart catheterization (RHC) for definitive diagnosis of PPH. The results of the two methods were compared to assess the screening effect of 2D-ECHO. RESULTS: The prevalence of PPH in this study was 3.8% (4/105). About 90% (95/105) of patients had a detectable tricuspid regurgitation by 2D-ECHO and the mean SPAP was 27.7 +/- 5.9 mmHg. Twenty-two of these 95 patients had an SPAP >30 mmHg. The mean PAT of all patients was 140 23 msec and 5 were <100 msec. Twenty-two patients were screened out by 2D-ECHO and 4 were diagnosed by RHC. A positive significant correlation (r=0.55, P<0.01) was found between SPAP measured by 2D-ECHO and mean pulmonary artery pressure (MPAP) measured by RHC, and a weak but significant negative correlation (r=-0.27, P=0.005) existed between PAT and pulmonary vascular resistance (PVR). The sensitivity, specificity, agreement rate, positive predictive value and negative predictive value of the screening test were 100%, 82%, 83%, 18% and 100%, respectively. CONCLUSIONS: The prevalence of PPH in this study is lower than in Western countries. As a screening test, 2D-ECHO has very high sensitivity and negative predictive value. A negative test result can directly be used to exclude PPH, while a positive result should be confirmed by RHC.
文摘Pulmonary vascular disorders including portopulmonary hypertension(PoPHT) are among the common complications of liver disease and are prognostically significant. Survival is very low without medical treatment and liver transplantation. With advances in medical therapy for elevated pulmonary artery pressure(PAP) and liver transplant surgery, survival of patients with Po PHT and advanced liver disease is significantly improved. Because of the prognostic significance of Po PHT and the limited donor pool, a comprehensive preoperative cardio-pulmonary assessment is of great importance in cirrhotic patients prior to transplant surgery. Therefore, a detailed transthoracic Doppler echocardiographic examination must be an essential component of this evaluation. Patients with mild Po PHT can safely undergo liver transplant surgery. In cases of moderate to severe Po PHT, right heart catheterization(RHC) should be performed. In patients with moderate to severe Po PHT on RHC(mean PAP 35-45 mm Hg), vasodilator therapy should be attempted. Liver transplantation should be encouraged in cases that demonstrate a positive response. Bridging therapy with specific pulmonary arterial hypertension treatment agents should be considered until the transplant surgery and should be continued during the peri- and post-operative periods as needed.
基金Supported by Beijing Science and Technology Commission Capital Clinical Application Research,No.Z181100001718220
文摘BACKGROUND Portopulmonary hypertension(PoPH) is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for LT.Many patients with liver disease require but cannot receive LT due to severe PoPH and eventually died.We report a patient with severe PoPH who underwent successful LT and had near normal pulmonary pressure without drug treatment.CASE SUMMARY A 39-year-old woman was hospitalized with the chief complaint of jaundice and exertional dyspnea and fatigue.Caroli disease and liver cirrhosis was diagnosed 6 years previously.Her liver disease met the criteria for LT.However, right heart catheterization showed that her mean pulmonary artery pressure was increased at 50 mmHg, pulmonary vascular resistance was 460 dyn?s/cm^5 and pulmonary artery wedge pressure was 20 mmHg, which may have been the reasons for her chief complaint.The patient was diagnosed with severe PoPH and was not listed for LT immediately.After 5 mo of pharmacotherapy, her severe PoPH was moderate, and she underwent successful LT.Pulmonary artery pressure steadily decreased according to post-operative echocardiographic monitoring and drugs have been discontinued for a month.CONCLUSION The safety of LT can be greatly improved by reducing mean pulmonary artery pressure to a low level, and LT may cure PoPH.
文摘Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are two frequent complications of liver disease, with prevalence among liver transplant candidates of 6% and 10%, respectively. Both conditions result from a lack of hepatic clearance of vasoactive substances produced in the splanchnic territory. Subsequently, these substances cause mainly pulmonary vascular remodeling and some degree of vasoconstriction in POPH with resulting elevated pulmonary pressure and right ventricular dysfunction. In HPS the vasoactive mediators cause intrapulmonary shunts with hypoxemia. Medical treatment is disappointing overall. Whereas liver transplantation (LT) results in the disappearance of HPS within six to twelve months, its effect on POPH is highly unpredictable. Modern strategies in managing HPS and POPH rely on a thorough screening and grading of the disease’s severity, in order to tailor the appropriate therapy and select only the patients who will benefit from LT. The anesthesiologist plays a central role in managing these high-risk patients. Indeed, the important hemodynamic and respiratory modifications of the perioperative period must be avoided through continuation of the preoperatively initiated drugs, appropriate intraoperative monitoring and proper hemodynamic and respiratory therapies.
文摘BACKGROUND: Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH. DATA SOURCES: An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension. RESULTS: Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH. CONCLUSIONS: After an accurate diagnosis of PPH treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.
文摘Intravenous epoprostenol is recommended for World Health Organization functional class(WHO-FC) Ⅳ patients with pulmonary arterial hypertension(PAH) in the latest guidelines. However, in portopulmonary hypertension(PoP H) patients, advanced liver dysfunction and/or thrombocytopenia often makes the use of intravenous epoprostenol challenging. Here we report the cases of two WHO-FC Ⅳ PoP H patients who were successfully treated with a combination of two oral vasodilators used to treat PAH: ambrisentan and tadalafil. Oral vasodilator therapy using a combination of ambrisentan and tadalafil may be a safe and effective therapeutic option for WHO-FC Ⅳ PoP H patients and should be considered for selected patients with severe and rapidly progressing PoP H.
文摘Portopulmonary hypertension (PPH) is a form of pulmonary arterial hypertension, characterized by elevation of pulmonary arterial pressure due to increased resistance to pulmonary blood flow. It is a consequence of portal hypertension, associated or not with liver disease. A retrospective study of patients undergoing liver transplantation was carried out in the Liver Transplantation Unit of the Clinics Hospital of the State University of Campinas (UNICAMP) from 1991 to 2012, covering 389 transplants. Although 50 (12.9%) patients presented with mean pulmonary arterial pressure (MPAP) > 25 mmHg, only one case (0.25%) met the diagnostic criteria for PPH: a 43-year-old female patient with advanced liver disease, caused by primary biliary cirrhosis with portal hypertension, diagnosed as mild portopulmonary hypertension and diagnosed in the operating room. PPH is a serious complication of portal hypertension. It is related to the great difficulty in diagnosis and the high mortality rate due to right heart failure. PPH is an entity with a low frequency of occurrence in individuals undergoing liver transplantation, although there is a wide variation in the prevalence rate presented in the literature and the definitive diagnosis should be confirmed with right heart catheterization.
