The Experts below are selected from a list of 1572 Experts worldwide ranked by ideXlab platform
Michael J. Krowka - One of the best experts on this subject based on the ideXlab platform.
-
Portopulmonary Hypertension.
Clinics in chest medicine, 2020Co-Authors: Jason M Golbin, Michael J. KrowkaAbstract:As a result of the success of orthotopic liver transplantation, there has been increasing interest in the diagnosis and therapeutic options for the pulmonary vascular complications of hepatic disease. These pulmonary vascular complications range from the hepatopulmonary syndrome, which is characterized by intrapulmonary vascular dilatations, to Portopulmonary Hypertension (POPH), which is characterized by an elevated pulmonary vascular resistance as a consequence of obstruction to pulmonary arterial blood flow. This review concentrates on POPH.
-
the myths and realities of Portopulmonary Hypertension
Hepatology, 2020Co-Authors: Hilary M Dubrock, Michael J. KrowkaAbstract:Portopulmonary Hypertension (POPH), defined as an increased mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) in the setting of portal Hypertension, is a commonly misunderstood pulmonary vascular complication of liver disease. Recent literature has expanded our knowledge regarding disease pathogenesis, the role of pulmonary arterial Hypertension (PAH) targeted therapy and liver transplantation (LT) in management, and long-term outcomes. However, many myths and misconceptions regarding POPH prognosis and treatment remain. The aim of this review is to examine the prevailing myths and current realities regarding POPH.
-
treatment barriers in Portopulmonary Hypertension
Hepatology, 2019Co-Authors: Batool Abuhalimeh, Michael J. Krowka, Adriano R TonelliAbstract:: Portopulmonary Hypertension (PoPH) is a form of pulmonary arterial Hypertension (PAH) that can develop as a complication of portal Hypertension. Treatment of PoPH includes PAH-specific therapies, and in certain cases, such therapies are necessary to facilitate a successful liver transplantation. A significant number of barriers may limit the adequate treatment of patients with PoPH and explain the poorer survival of these patients when compared to patients with other types of PAH. Until recently, only one randomized controlled trial has included PoPH patients, and the majority of treatment data have been derived from relatively small observational studies. In the present article, we review some of the barriers in the treatment of patients with PoPH and implications for liver transplantation.
-
riociguat treatment for Portopulmonary Hypertension a subgroup analysis from the patent 1 2 studies
Pulmonary circulation, 2018Co-Authors: Rodrigo Cartinceba, Michael Halank, Hossein Ardeschir Ghofrani, Marc Humbert, John Mattson, Arno Fritsch, Michael J. KrowkaAbstract:In patients with Portopulmonary Hypertension (n = 13) included in the 12-week randomized placebo-controlled PATENT-1 trial, riociguat was well tolerated and improved 6-min walking distance (6MWD), ...
-
relationship between Portopulmonary Hypertension and splenectomy mayo clinic experience and review of published works
Hepatology Research, 2018Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael J. KrowkaAbstract:AIM: Portopulmonary Hypertension is a serious complication of portal Hypertension that can lead to right heart failure and death. To our knowledge, an association between Portopulmonary Hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in Portopulmonary Hypertension and compare selected parameters between Portopulmonary Hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with Portopulmonary Hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of Portopulmonary Hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of Portopulmonary Hypertension. Twenty-seven (19%) Portopulmonary Hypertension patients had autoimmune liver disease, and 5 of 8 (62.5%) splenectomized Portopulmonary Hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized Portopulmonary Hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in Portopulmonary Hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in Portopulmonary Hypertension was not uncommon. History of splenectomy in patients with portal Hypertension and/or autoimmune liver disease may have clinical implications.
S Raevens - One of the best experts on this subject based on the ideXlab platform.
-
portico first randomized controlled trial of vasomodulator therapy in Portopulmonary Hypertension
Hepatology, 2020Co-Authors: S Raevens, Michael B FallonAbstract:: Portopulmonary Hypertension (POPH) is a severe complication of portal Hypertension, which can manifest with or without cirrhosis. The diagnosis is made based on elevated mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) with normal capillary wedge pressure measured during right heart catheterization. Without intervention, POPH carries a very poor prognosis and is characterized by obstruction of pulmonary arterial flow that progresses to right heart failure.
-
PORTICO: first RCT of vasomodulator therapy in Portopulmonary Hypertension
Hepatology, 2020Co-Authors: S Raevens, Michael B FallonAbstract:: Portopulmonary Hypertension (POPH) is a severe complication of portal Hypertension, which can manifest with or without cirrhosis. The diagnosis is made based on elevated mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) with normal capillary wedge pressure measured during right heart catheterization. Without intervention, POPH carries a very poor prognosis and is characterized by obstruction of pulmonary arterial flow that progresses to right heart failure.
-
hepatopulmonary syndrome and Portopulmonary Hypertension
Chinese Journal of Asthma, 2017Co-Authors: S Raevens, I ColleAbstract:Abstract This chapter focuses on two cardiopulmonary vascular disorders of liver disease: hepatopulmonary syndrome (HPS) and Portopulmonary Hypertension. HPS is characterized by gas exchange disturbances due to pulmonary vasodilation and right-to-left shunting, whereas Portopulmonary Hypertension is defined by pulmonary Hypertension secondary to increased pulmonary vascular resistance, which may lead to hemodynamic failure. Key aspects of assessment include active screening with echocardiography, right heart catheterization, and arterial blood gas analysis, early diagnosis, and prompt referral to specialized hepatology centers. As no curative medical treatment is available and only liver transplantation may lead to resolution, MELD exceptions can be allocated to provide prioritization for transplantation. This chapter will give an overview on diagnosis and screening, pathophysiology and pathogenesis, therapeutic targets, and treatment of both conditions.
-
hepatopulmonary syndrome and Portopulmonary Hypertension recent knowledge in pathogenesis and overview of clinical assessment
Liver International, 2015Co-Authors: S Raevens, Anja Geerts, Christophe Van Steenkiste, Xavier Verhelst, Hans Van Vlierberghe, Isabelle ColleAbstract:Hepatopulmonary syndrome and Portopulmonary Hypertension are cardiopulmonary complications, which are not infrequently seen in patients with liver disease and/or portal Hypertension. These entities are both clinically and pathophysiologically different: the hepatopulmonary syndrome is characterized by abnormal pulmonary vasodilation and right-to-left shunting resulting in gas exchange abnormalities, whereas Portopulmonary Hypertension is caused by pulmonary artery vasoconstriction leading to hemodynamic failure. As both hepatopulmonary syndrome and Portopulmonary Hypertension are associated with significantly increased morbidity and mortality, and as these patients are commonly asymptomatic, all liver transplantation candidates should be actively screened for the presence of these two complications. The aim of is this review is to provide an overview on the hepatopulmonary syndrome and Portopulmonary Hypertension with primary focus on diagnosis and recent knowledge regarding pathogenesis and therapeutic targets.
Justin M. Segraves - One of the best experts on this subject based on the ideXlab platform.
-
relationship between Portopulmonary Hypertension and splenectomy mayo clinic experience and review of published works
Hepatology Research, 2018Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael J. KrowkaAbstract:AIM: Portopulmonary Hypertension is a serious complication of portal Hypertension that can lead to right heart failure and death. To our knowledge, an association between Portopulmonary Hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in Portopulmonary Hypertension and compare selected parameters between Portopulmonary Hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with Portopulmonary Hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of Portopulmonary Hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of Portopulmonary Hypertension. Twenty-seven (19%) Portopulmonary Hypertension patients had autoimmune liver disease, and 5 of 8 (62.5%) splenectomized Portopulmonary Hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized Portopulmonary Hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in Portopulmonary Hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in Portopulmonary Hypertension was not uncommon. History of splenectomy in patients with portal Hypertension and/or autoimmune liver disease may have clinical implications.
-
Relationship between Portopulmonary Hypertension and splenectomy: Mayo Clinic experience and review of published works: Portopulmonary Hypertension and splenectomy
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Rodrigo Cartin-ceba, Michael D. Leise, Michael J. KrowkaAbstract:AIM: Portopulmonary Hypertension is a serious complication of portal Hypertension that can lead to right heart failure and death. To our knowledge, an association between Portopulmonary Hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in Portopulmonary Hypertension and compare selected parameters between Portopulmonary Hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with Portopulmonary Hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of Portopulmonary Hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of Portopulmonary Hypertension. Twenty-seven (19%) Portopulmonary Hypertension patients had autoimmune liver disease, and 5 of 8 (62.5%) splenectomized Portopulmonary Hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized Portopulmonary Hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in Portopulmonary Hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in Portopulmonary Hypertension was not uncommon. History of splenectomy in patients with portal Hypertension and/or autoimmune liver disease may have clinical implications.
-
the relationship between Portopulmonary Hypertension and splenectomy mayo clinic experience and literature review
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael J. KrowkaAbstract:Background & Aims Portopulmonary Hypertension is a serious complication of portal Hypertension that can lead to right heart failure and death. To our knowledge, an association between Portopulmonary Hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in Portopulmonary Hypertension and compare selected parameters between Portopulmonary Hypertension subgroups. Methods Retrospective analysis of patients diagnosed with Portopulmonary Hypertension between 01/01/1988 – 06/30/2015 at Mayo Clinic, Rochester, MN. Compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of Portopulmonary Hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). Results Cohort of 141 patients. 8 (6%) with history of splenectomy prior to the development of Portopulmonary Hypertension. 27 (19%) Portopulmonary Hypertension patients had autoimmune liver disease; 5/8 (62.5%) of splenectomized Portopulmonary Hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non splenectomized Portopulmonary Hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. Conclusions Prior history of splenectomy in Portopulmonary Hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in Portopulmonary Hypertension was not uncommon. The history of splenectomy in patients with portal Hypertension and/or autoimmune liver may have clinical implications.
Michael B Fallon - One of the best experts on this subject based on the ideXlab platform.
-
Portopulmonary Hypertension.
Clinics in liver disease, 2020Co-Authors: Michael Passarella, Michael B Fallon, Steven M KawutAbstract:Portopulmonary Hypertension is a common condition in patients who have portal Hypertension. This article reviews the definition and clinical presentation of this disorder and outlines our current understanding of its pathophysiology. A diagnostic approach is provided , and novel medical therapies that are being investigated to treat this condition are discussed. Finally, the safety of liver transplantation in patients who have Portopulmonary Hypertension is reviewed.
-
portico first randomized controlled trial of vasomodulator therapy in Portopulmonary Hypertension
Hepatology, 2020Co-Authors: S Raevens, Michael B FallonAbstract:: Portopulmonary Hypertension (POPH) is a severe complication of portal Hypertension, which can manifest with or without cirrhosis. The diagnosis is made based on elevated mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) with normal capillary wedge pressure measured during right heart catheterization. Without intervention, POPH carries a very poor prognosis and is characterized by obstruction of pulmonary arterial flow that progresses to right heart failure.
-
PORTICO: first RCT of vasomodulator therapy in Portopulmonary Hypertension
Hepatology, 2020Co-Authors: S Raevens, Michael B FallonAbstract:: Portopulmonary Hypertension (POPH) is a severe complication of portal Hypertension, which can manifest with or without cirrhosis. The diagnosis is made based on elevated mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) with normal capillary wedge pressure measured during right heart catheterization. Without intervention, POPH carries a very poor prognosis and is characterized by obstruction of pulmonary arterial flow that progresses to right heart failure.
-
international liver transplant society practice guidelines diagnosis and management of hepatopulmonary syndrome and Portopulmonary Hypertension
Transplantation, 2016Co-Authors: Michael J. Krowka, Michael B Fallon, Steven M Kawut, Michael A. E. Ramsay, Olivier Sitbon, Valentin Fuhrmann, Julie K Heimbach, Ronald J SokolAbstract:AbstractTwo distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and Portopulmonary Hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Societ
Michael D. Leise - One of the best experts on this subject based on the ideXlab platform.
-
relationship between Portopulmonary Hypertension and splenectomy mayo clinic experience and review of published works
Hepatology Research, 2018Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael J. KrowkaAbstract:AIM: Portopulmonary Hypertension is a serious complication of portal Hypertension that can lead to right heart failure and death. To our knowledge, an association between Portopulmonary Hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in Portopulmonary Hypertension and compare selected parameters between Portopulmonary Hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with Portopulmonary Hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of Portopulmonary Hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of Portopulmonary Hypertension. Twenty-seven (19%) Portopulmonary Hypertension patients had autoimmune liver disease, and 5 of 8 (62.5%) splenectomized Portopulmonary Hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized Portopulmonary Hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in Portopulmonary Hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in Portopulmonary Hypertension was not uncommon. History of splenectomy in patients with portal Hypertension and/or autoimmune liver disease may have clinical implications.
-
Relationship between Portopulmonary Hypertension and splenectomy: Mayo Clinic experience and review of published works: Portopulmonary Hypertension and splenectomy
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Rodrigo Cartin-ceba, Michael D. Leise, Michael J. KrowkaAbstract:AIM: Portopulmonary Hypertension is a serious complication of portal Hypertension that can lead to right heart failure and death. To our knowledge, an association between Portopulmonary Hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in Portopulmonary Hypertension and compare selected parameters between Portopulmonary Hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with Portopulmonary Hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of Portopulmonary Hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of Portopulmonary Hypertension. Twenty-seven (19%) Portopulmonary Hypertension patients had autoimmune liver disease, and 5 of 8 (62.5%) splenectomized Portopulmonary Hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized Portopulmonary Hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in Portopulmonary Hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in Portopulmonary Hypertension was not uncommon. History of splenectomy in patients with portal Hypertension and/or autoimmune liver disease may have clinical implications.
-
the relationship between Portopulmonary Hypertension and splenectomy mayo clinic experience and literature review
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael J. KrowkaAbstract:Background & Aims Portopulmonary Hypertension is a serious complication of portal Hypertension that can lead to right heart failure and death. To our knowledge, an association between Portopulmonary Hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in Portopulmonary Hypertension and compare selected parameters between Portopulmonary Hypertension subgroups. Methods Retrospective analysis of patients diagnosed with Portopulmonary Hypertension between 01/01/1988 – 06/30/2015 at Mayo Clinic, Rochester, MN. Compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of Portopulmonary Hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). Results Cohort of 141 patients. 8 (6%) with history of splenectomy prior to the development of Portopulmonary Hypertension. 27 (19%) Portopulmonary Hypertension patients had autoimmune liver disease; 5/8 (62.5%) of splenectomized Portopulmonary Hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non splenectomized Portopulmonary Hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. Conclusions Prior history of splenectomy in Portopulmonary Hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in Portopulmonary Hypertension was not uncommon. The history of splenectomy in patients with portal Hypertension and/or autoimmune liver may have clinical implications.