Molmenti, Ernesto P.1; Roodhouse, Thomas W.1; Huang, Howard1; Coke, Christine1; Levy, Marlon F.1; Jennings, Linda W.1; Brooks, Barbara K.1; Conkey, Amy1; Johnson, Catherine1; Sanchez, Edmund Q.1; Marubashi, Shigeru1; Jung, Ghap1; Goldstein, Robert M.1; Fasola, Carlos G.1; Gonwa, Thomas A.1; Klintmalm, Goran B.1 Author Information

Sanchez, Edmund; Marubashi, Shigeru; Jung, Ghap; Goldstein, Robert M.; Molmenti, Ernesto P.; Fasola, Carlos G.; Gonwa, Thomas A.; Jennings, Linda W.; Conkey, Amy C.; Brooks, Barbara K.; Klintmalm, Goran B.; Levy, Marlon F. Author Information

955 Introduction: Severe pulmonary hypertension (PHT) in conjunction with end-stage liver disease (ESLD) is a significant perioperative risk to patients undergoing orthotopic liver transplantation (OLT) (1). In a previous study, only 2 of 7 patients with PHT had a long-term survival after OLT. The remaining 5 patients died as a result of right heart failure (2). In a prospective study, patients with severe portopulmonary hypertension requiring OLT were administered epoprostenol and nitric oxide (NO) prior to OLT to determine if the PHT could be lowered to a mean pressure of less than 35mmHg. If the patient responded to either therapy and had good RV contraction, OLT was undertaken. Post-transplantation, an epoprostenol infusion continued until PAP normalized. Method: Five patients were diagnosed with severe portopulmonary hypertension, either during evaluation for OLT or at the time of transplantation. A trial of inhaled NO up to 40ppm was performed and then an infusion of epoprostenol was started at 5 ng/kg/hr and increased to the maximum tolerated by the patient. Transplantation was only performed if mean PAP ≤ 35mmHg was achieved and RV function was assessed normal on echocardiogram. Post-operatively, the epoprostenol infusion was maintained until PAP was in the normal range and RV function was assessed by echocardiogram as normal. Results:(Table)TableConclusion: Prior to instituting a protocol requiring the reversal of MPAP to ≤ 35 mmHg before transplantation post-operative mortality was 71% (5 of 7 patients). After instituting this protocol, using chronic epoprostenol infusion perioperatively and NO therapy, 100% of patients (5 of 5) have retained good RV function and quality of life, and MPAP have not increased.