Humans ; Hepatopulmonary Syndrome/surgery* ; Liver Transplantation/adverse effects* ; Extracorporeal Membrane Oxygenation/adverse effects* ; Postoperative Complications ; Patients

BACKGROUNDOnly a few reviews of small case series and individual case reports including a relatively small number of adult patients undergoing liver transplantation for hepatopulmonary syndrome (HPS) are available, and there has been no prospective evaluation of the long-term outcome of HPS patients after orthotopic liver transplantation (OLT). The aim of this study was to determine the frequency of HPS in OLT patients with chronic end-stage liver-disease, and the short-term and long-term postoperative outcome of HPS patients after OLT.

METHODSThis prospective study included 31 HPS and 30 control, non-HPS patients. The preoperative conditions were similar between the two groups. Twenty-six of 31 HPS patients and all of the non-HPS patients underwent OLT. Standardized methods, such as arterial blood gas at room air and 99m-technetium macroaggregated albumin ((99m)Tc MAA) lung and brain perfusion scanning were performed for the diagnosis of HPS. Patients were followed after OLT.

RESULTSThe incidence of HPS in OLT patients was 9.3% (26/279). Hypoxemia in HPS was obviously improved with a normalized shunt of (99m)Tc MAA in the lungs after OLT. The immediate postoperative survival rate (within 28 days after OLT) of HPS was 76.9% (20/26). The one year survival was 61.5% (16/26) and four-year survival was 57.7% (15/26); much higher than HPS patients without OLT (0). But high postoperative morbidity and mortality were observed in HPS patients whose death occurred within 3 months of OLT due to complications summarized in this study.

CONCLUSIONSLiver transplantation was an effective treatment for HPS. But the postoperative mortality rate following OLT in HPS patients was still much higher than that of patients without HPS.

Female ; Hepatopulmonary Syndrome ; mortality ; surgery ; Humans ; Liver Transplantation ; adverse effects ; methods ; Male ; Postoperative Period ; Prospective Studies ; Survival Analysis ; Treatment Outcome

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

OBJECTIVETo observe the effect of orthotopic liver transplantation (OLT) on hepatopulmonary syndrome (HPS) and investigate risk factors predicting the prognosis of OLT.

METHODSTwenty-six cases of HPS and 30 cases of non-HPS were analyzed treated from April 2004 to January 2006. Survival rates after OLT were compared and risk factors predicting the prognosis of OLT in HPS were researched by univariant and COX analysis.

RESULTSThe 28 days survival rate in HPS after OLT was 76.9% (20/26), half a year survival rate and one year survival rate were both 61.5% (16/26). Whereas the one year survival rate of patients without HPS was 100%(P < 0.05). By univariant analysis, shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs, PaO2 and PaO2/FiO2 in room air before operation were relative to the prognosis of peri-operative period and half a year outcome after OLT in HPS (P < 0.05). Shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs (OR = 1.182, P = 0.001), and mechanical ventilation time (OR = 1.003, P = 0.053) after OLT were independent risk factors predicting the prognosis of OLT in HPS by COX analysis. Shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs > or = 28.4%, or PaO2 < or = 56 mm Hg (1 mm Hg = 0.133 kPa) before OLT predicted the poor outcome of OLT in HPS. The sensitivity were 83.3% and 85.0% respectively, and the specificity were 95.0% and 83.3% respectively.

CONCLUSIONSOLT is an effective treatment for HPS.Shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs before OLT and mechanical ventilation time after OLT were independent risk factors for the prognosis of OLT in HPS.

Adult ; Aged ; Female ; Follow-Up Studies ; Hepatopulmonary Syndrome ; surgery ; Humans ; Liver Transplantation ; Male ; Middle Aged ; Prognosis ; Risk Factors ; Survival Analysis ; Treatment Outcome

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