Animals ; Hepatopulmonary Syndrome ; diagnosis ; etiology ; therapy ; Humans ; Liver Transplantation

BACKGROUNDS/AIMS: Hepatopulmonary syndrome is a condition of severe hypoxia with intrapulmonary shunt (IPS) in the setting of hepatic dysfunction. Liver transplantation has been suggested as a definite treatment for hepatopulmonary syndrome with reversal of IPS in some patients. Inconsistency of response and inability to predict reversibility, however, are significant problems. We performed this study to evaluate the prevalence of significant IPS in pretransplantation cirrhotic patients and to find any risk factors of IPS. METHODS: Fifty-seven patients (M:F = 38:19, median age 49 years (range 18 - 71)) with liver cirrhosis awaiting liver transplantation were serially included. Their IPS status was evaluated using contrast- enhanced echocardiography. Significant shunt was defined as a shunt of grade >or= 2. RESULTS: Significant IPS was detected in 30 (52.6%) among 57 patients. Significant shunt was found in 24 (63.2%) of 38 Child-Pugh class C patients and in 6 (31.6%) of 19 Child-Pugh class A or B patients (p < 0.05). No significant difference in prevalence of significant shunt was seen according to age, sex, presence or absence of hepatocellular carcinoma, ascites, hepatic encephalopathy, and gastroesophageal varix. CONCLUSION: Significant intrapulmonary shunt is a common finding in cirrhotic patients awaiting liver transplantation. Child-Pugh class C is the risk factor associated with high prevalence of significant shunt.
Adolescent ; Adult ; Aged ; Echocardiography ; English Abstract ; Female ; Hepatopulmonary Syndrome/etiology ; Human ; Liver Cirrhosis/*physiopathology/surgery ; *Liver Transplantation ; Male ; Middle Aged ; *Pulmonary Circulation ; Risk Factors

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.
Adolescent ; Anoxia ; Arteriovenous Fistula/etiology ; Biliary Atresia/*diagnosis/etiology ; Cyanosis/complications ; Dyspnea/complications ; Echocardiography, Transesophageal ; End Stage Liver Disease/complications/*surgery ; Female ; Hepatic Artery/abnormalities ; Hepatopulmonary Syndrome/*diagnosis/ultrasonography ; Humans ; *Liver Transplantation ; Osteoarthropathy, Secondary Hypertrophic/complications

Adult ; Aged ; Aged, 80 and over ; Female ; Hepatopulmonary Syndrome ; diagnostic imaging ; etiology ; Humans ; Liver Cirrhosis ; complications ; diagnostic imaging ; Lung ; diagnostic imaging ; Male ; Middle Aged ; Radionuclide Imaging ; Radiopharmaceuticals ; Technetium Tc 99m Aggregated Albumin

Blood Gas Analysis ; Echocardiography ; Hepatopulmonary Syndrome ; diagnosis ; physiopathology ; therapy ; Humans ; Hypoxia ; diagnosis ; etiology ; therapy ; Liver Cirrhosis ; complications ; Liver Transplantation ; Lung ; pathology ; physiopathology ; Oxygen ; therapeutic use ; Radiography, Thoracic ; Respiratory Function Tests

OBJECTIVE: To investigate the correlation between radiologic vascular dilatation and serum nitrite concentration and eNOS expression in the endothelial cell and pneumocyte in a rabbit model of hepatopulmonary syndrome induced by common bile duct ligation (CBDL). MATERIALS AND METHODS: Thin-section CT scans of the lung and pulmonary angiography were obtained 3 weeks after CBDL (n=6), or a sham operation (n=4), and intrapulmonary vasodilatation was assessed. The diameter and tortuosity of peripheral vessels in the right lower lobe by thin-section CT and angiography at the same level of the right lower lobe in all subjects were correlated to serum nitrite concentration and eNOS (endothelial nitric oxide synthase) expression as determined by immunostaining. RESULTS: The diameters of pulmonary vessels on thin-section CT were well correlated with nitrite concentrations in serum (r = 0.92, p < 0.001). Dilated pulmonary vessels were significantly correlated with an increased eNOS expression (r = 0.94, p < 0.0001), and the severity of pulmonary vessel tortuosity was found to be well correlated with serum nitrite concentration (r = 0.90, p < 0.001). CONCLUSION: The peripheral pulmonary vasculature in hepatopulmonary syndrome induced by CBLD was dilated on thin-section CT and on angiographs. Our findings suggest that peripheral pulmonary vascular dilatations are correlated with serum nitrite concentrations and pulmonary eNOS expression.
Angiography ; Animals ; Common Bile Duct/injuries ; Dilatation, Pathologic/radiography ; Disease Models, Animal ; Endothelium, Vascular/metabolism ; Hepatopulmonary Syndrome/etiology/*metabolism ; Ligation ; Lung/*blood supply/metabolism/*radiography ; Nitric-Oxide Synthase/*metabolism ; Nitrites/*blood ; Pulmonary Artery/radiography ; Rabbits ; Research Support, Non-U.S. Gov't ; Tomography, X-Ray Computed/methods