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The association of pulmonary hypertension with portal hypertension, also called portopulmonary hypertension (PPHTN), is a known complication of chronic liver disease. Previously, the presence of PPHTN was considered to be a contraindication to orthotopic liver transplantation (OLT). Although there are selected case reports of successful OLT in the setting of PPHTN, an excessive mortality rate is associated with OLT and PPHTN. Heretofore, therapy for chronic management of PPHTN was lacking. Recently, continuous intravenous infusion of epoprostenol has been demonstrated to improve symptomatology and survival in the general population of patients with primary pulmonary hypertension. We now report the use of epoprostenol in the more specific instance of PPHTN. Over a period of 6-14 months, epoprostenol (10-28 ng/kg/min) therapy was associated with a 29-46% decrease in mean pulmonary artery pressure, a 22-71% decrease in pulmonary vascular resistance, and a 25-75% increase in cardiac output in a group of four patients. These results suggest that effective chronic therapy for PPHTN is available. In conjunction with inhaled nitric oxide as acute intraoperative therapy, epoprostenol infusion represents an additional therapeutic option for treatment of PPHTN in the liver transplant candidate.

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This study attempts to evaluate the efficacy of dobutamine stress echocardiography for preoperative cardiac risk stratification in patients undergoing orthotopic liver transplantation. Two hundred twenty consecutively submitted patients were evaluated in preparation for orthotopic liver transplantation. Dobutamine stress echocardiography was performed in 80 patients with known or suspected coronary artery disease. Follow-up information was available in 40 patients in the form of cardiac catheterization and/or outcome from liver transplantation to validate the dobutamine stress echo findings. The prevalence of coronary artery disease in this cohort was 5% and was closely associated with the presence of diabetes mellitus. Dobutamine stress echocardiography, when interpreted as abnormal in the presence of wall motion abnormalities only, is associated with a sensitivity, specificity, and positive and negative predictive value of 100%. Dobutamine stress echocardiography is highly efficacious and should be the screening study of choice to detect coronary artery disease in patients undergoing orthotopic liver transplantation. Copyright 1998 by the American Association for the Study of Liver DiseasesO rthotopic liver transplantation (OLT) in patients with coronary artery disease (CAD) is associated with significant morbidity and mortality. 1 Carey et al 2 found that 27% of patients older than 50 years who underwent evaluation for OLT had moderate to severe CAD; the CAD was clinically unsuspected in 13.3% of those with CAD. 3 Furthermore, they identified diabetes mellitus (DM) as an independent risk factor. Despite these data, the ideal preoperative cardiac screening study in patients with end-stage liver disease (ESLD) has yet to be determined. Studies have shown that dobutamine stress echocardiography (DSE) is an accepted technique for the evaluation of CAD in general, 3,4 and more specifically before major vascular surgery 5,6 and kidney transplantation. 7 Donovan et al 8 have shown that the absence of inducible ischemia by DSE confers low risk for myocardial infarction during OLT; however, the inclusion criteria for their study were broad and included many low-risk patients. We therefore sought to evaluate the efficacy of DSE in identifying significant cardiac risk before OLT in the subgroup of patients with a history of and/or significant risk factors for CAD. Materials and MethodsPatient evaluations and decisions regarding cardiac workup were made prospectively; however, the data were analyzed in a retrospective fashion.Two hundred twenty consecutive patients were evaluated for OLT. Careful attention was paid to symptoms of cardiac ischemia and/or risk factors for CAD during the initial evaluation. DSE was performed in 80 patients based on the following criteria: age greater than 50 years but less than 60 with two or more cardiac risk factors (tobacco use, obesity, hypercholesterolemia, hypertension, family history); age greater than than 60 years; presence of active symptoms indicative of CAD; electrocardiographic...

The management of the liver transplant (OLT) candidate with portopulmonary hypertension (PPHTN) has dramatically changed in the past 3 years. Careful preoperative evaluation with functional characterization of right ventricular function plays a critical role. The pulmonary vascular response to epoprostenol infusion serves as a deciding factor for OLT candidacy. Careful perioperative attention to avoid right ventricular failure from acutely elevated pulmonary artery pressures or sudden increases in right ventricular preload is a key physiologic tenet of management. With increased surgical expertise, anesthetic sophistication, and availability of epoprostenol, PPHTN is no longer considered an absolute contraindication for OLT.

Orthotopic liver transplantation can be marked by significant hemodynamic instability requiring the use of a variety of hemodynamic monitors to aide in intraoperative management. Invasive blood pressure monitoring is essential, but the accuracy of peripheral readings in comparison to central measurements has been questioned. When discrepancies exist, central mean arterial pressure, usually measured at the femoral artery, is considered more indicative of adequate perfusion than those measured peripherally. The traditional pulmonary artery catheter is less frequently used due to its invasive nature and known limitations in measuring preload but still plays an important role in measuring cardiac output (CO) when required and in the management of portopulmonary hypertension. Pulse wave analysis is a newer technology that uses computer algorithms to calculate CO, stroke volume variation (SVV) and pulse pressure variation (PPV). Although SVV and PPV have been found to be accurate predicators of fluid responsiveness, CO measurements are not reliable during liver transplantation. Transesophageal echocardiography is finding an increasing role in the real-time monitoring of preload status, cardiac contractility and the diagnosis of a variety of pathologies. It is limited by the expertise required, limited transgastric views during key portions of the operation, the potential for esophageal varix rupture and difficulty in obtaining quantitative measures of CO in the absence of tricuspid regurgitation.

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Chronic epoprostenol, in conjunction with a multidisciplinary, well-planned perioperative evaluation and treatment plan, may be the answer to a heretofore untreatable disease.