Current Approach to the Diagnosis and Management of Portopulmonary Hypertension.
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.
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Lynn A
LA
Fussner
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA. Krowka.Michael@mayo.edu.|Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA. Krowka.Michael@mayo.edu.
Michael J
MJ
Krowka
D000959:Antihypertensive Agents / Q000627:therapeutic use; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D006162:Guanylate Cyclase / Q000502:physiology; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D016031:Liver Transplantation; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011453:Prostaglandins / Q000627:therapeutic use
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D011453:Prostaglandins; D006162:Guanylate Cyclase
Echocardiography; Liver transplant; Portopulmonary hypertension; Pulmonary arterial hypertension; Right heart catheterization; Vasodilator