Abstract
Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension occurring in the setting of portal hypertension with or without hepatic cirrhosis. The presence of both portal and pulmonary vascular disease contributes to complicated hemodynamics and therapeutic challenges, though the severities do not appear to correlate directly. Diagnosis of POPH, and distinction from the commonly observed hyperdynamic state of end-stage liver disease, is typically accomplished with an initial screening transthoracic echocardiogram, followed by right heart catheterization for confirmation of hemodynamic parameters. Though few studies have directly evaluated use in POPH, pulmonary artery-directed therapy is the cornerstone of management, along with consideration of liver transplantation. Perioperative and long-term outcomes are variable, but uniformly worse in the setting of uncontrolled pulmonary pressures. Risk stratification and optimal patient selection for these interventions are areas of ongoing investigation.
| Original language | English (US) |
|---|---|
| Article number | 29 |
| Journal | Current Gastroenterology Reports |
| Volume | 18 |
| Issue number | 6 |
| DOIs | |
| State | Published - Jun 1 2016 |
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Keywords
- Echocardiography
- Liver transplant
- Portopulmonary hypertension
- Pulmonary arterial hypertension
- Right heart catheterization
- Vasodilator
ASJC Scopus subject areas
- Gastroenterology
Cite this
Current Approach to the Diagnosis and Management of Portopulmonary Hypertension. / Fussner, Lynn A.; Krowka, Michael Joseph.
In: Current Gastroenterology Reports, Vol. 18, No. 6, 29, 01.06.2016.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Current Approach to the Diagnosis and Management of Portopulmonary Hypertension
AU - Fussner, Lynn A.
AU - Krowka, Michael Joseph
PY - 2016/6/1
Y1 - 2016/6/1
N2 - Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension occurring in the setting of portal hypertension with or without hepatic cirrhosis. The presence of both portal and pulmonary vascular disease contributes to complicated hemodynamics and therapeutic challenges, though the severities do not appear to correlate directly. Diagnosis of POPH, and distinction from the commonly observed hyperdynamic state of end-stage liver disease, is typically accomplished with an initial screening transthoracic echocardiogram, followed by right heart catheterization for confirmation of hemodynamic parameters. Though few studies have directly evaluated use in POPH, pulmonary artery-directed therapy is the cornerstone of management, along with consideration of liver transplantation. Perioperative and long-term outcomes are variable, but uniformly worse in the setting of uncontrolled pulmonary pressures. Risk stratification and optimal patient selection for these interventions are areas of ongoing investigation.
AB - Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension occurring in the setting of portal hypertension with or without hepatic cirrhosis. The presence of both portal and pulmonary vascular disease contributes to complicated hemodynamics and therapeutic challenges, though the severities do not appear to correlate directly. Diagnosis of POPH, and distinction from the commonly observed hyperdynamic state of end-stage liver disease, is typically accomplished with an initial screening transthoracic echocardiogram, followed by right heart catheterization for confirmation of hemodynamic parameters. Though few studies have directly evaluated use in POPH, pulmonary artery-directed therapy is the cornerstone of management, along with consideration of liver transplantation. Perioperative and long-term outcomes are variable, but uniformly worse in the setting of uncontrolled pulmonary pressures. Risk stratification and optimal patient selection for these interventions are areas of ongoing investigation.
KW - Echocardiography
KW - Liver transplant
KW - Portopulmonary hypertension
KW - Pulmonary arterial hypertension
KW - Right heart catheterization
KW - Vasodilator
UR - http://www.scopus.com/inward/record.url?scp=84964260996&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84964260996&partnerID=8YFLogxK
U2 - 10.1007/s11894-016-0504-2
DO - 10.1007/s11894-016-0504-2
M3 - Article
C2 - 27098816
AN - SCOPUS:84964260996
VL - 18
JO - Current Gastroenterology Reports
JF - Current Gastroenterology Reports
SN - 1522-8037
IS - 6
M1 - 29
ER -