hepatopulmonary syndrome


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hepatopulmonary syndrome

a syndrome consisting of liver disease (usually cirrhosis), hypoxemia, and the presence of intrapulmonary vascular dilatations; notable for the common presence of orthodeoxia and platypnea.

hepatopulmonary syndrome

arterial hypoxemia caused by pulmonary vasodilation in conjunction with chronic liver disease, usually occurring as a result of portal hypertension in cirrhosis.

hepatopulmonary syndrome

A condition in which hypoxemia due to intrapulmonary shunting and/or a V/Q mismatch develops in a Pt with liver cirrhosis; usually there is no apparent parenchymal lung disease, but Pts may have orthodeoxia, an unusual finding of ↑ hypoxemia with a change from the supine to the erect position; the pathogenesis of HPS is uncertain but may be due to an ↑ production of endogenous nitric oxide; shunting of HPS may respond to IV methylene blue

hepatopulmonary syndrome

(hĕp″ă-tō-pŭl′mō-năr″ē) [″ + L. pulmo, lung]
A combination of liver disease, decreased arterial oxygen concentration, and dilatation of the blood vessels of the lung. Clinically the patient may have signs and symptoms of liver disease, including gastrointestinal bleeding, esophageal varices, ascites, palmar erythema, and splenomegaly. Pulmonary signs include clubbing of the fingers, cyanosis, dyspnea, and decreased arterial oxygen concentration while in an upright position (orthodeoxia).
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Indications for LT in children with BA Complications of PHT Gastro-intestinal bleeding Recurrent cholangitis Hepatopulmonary syndrome Failure of liver Worsening coagulopathy synthetic function Intractable ascites Spontaneous bacterial peritonitis Nutritional difficulties Failure to thrive Fat-soluble vitamin deficiencies Developmental delay Failing to achieve developmental milestones in infancy Motor difficulties secondary to PHT Delayed puberty Chronic encephalopathy Miscellaneous CLD Intractable pruritus complications Development of focal lesion, suspected hepatocellular carcinoma Osteoarthropathy Poor quality of life LT = liver transplantation; BA = biliary atresia; PHT = portal hypertension; CLD = chronic liver disease.
Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression.
2) Serious complications from cirrhosis, which may contribute to hospitalization and even death, include variceal bleeding, spontaneous bacterial peritonitis, hepatocellular carcinoma, hepatorenal syndrome, hepatic encephalopathy, and hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) is present in 8 to 17% of patients with cirrhosis.
Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome.
Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis.
Approximately 80% of patients with the hepatopulmonary syndrome eventually have improved oxygenation after liver transplantation, thereby making worsening hypoxemia the primary indication for transplantation in many instances.
Clinical states associated with the platypneaorthodeoxia syndrome (a) Intracardiac right-to-left shunts (patent foramen ovale or atrial septal defect) After pulmonary resection (eg, pneumonectomy, lobectomy) Associated cardiac abnormality (eg, aortic aneurysm, pericardial effusion) Associated skeletal deformity (kyphoscoliosis) Intrapulmonary right-to-left shunts Hepatopulmonary syndrome Pulmonary diseases Chronic obstructive pulmonary disease Pulmonary embolism Upper airway tumor Acute respiratory distress syndrome Miscellaneous causes Autonomic neuropathy (a) ARDS, acute respiratory distress syndrome.
Transient hepatopulmonary syndrome in a patient with acute hepatitis A.
Novel presentation and approach to management of hepatopulmonary syndrome with use of antimicrobial agents.