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Title : Perioperative Management of Severe Pulmonary Hypertension with Inhàled Nitric Oxide and Oral Sildenafil (Viagra Pagå 1 Title : Perioperative Management of Severe Pulmonary Hypertension with Inhàled Nitric Oxide and Oral Sildenafil (Viagra TM ) During Orthotopic Liver Transplantation. Case Presentation. Authîrs : Angel Jose deLeon Vaca, M.D.â, Earl M. Strum, M.D.â, Janos Szenohradszki, M.D. Ph.D.â, Lindà Sher, M.D.â, Robert Selby, M.D.â Affiliation : Departments of Anesthesiology â and Surgery â, Keck Schoîl of Medicine, University of Southern California, Los Angelås, CA 90033 Introduction: Pulmonary hypertension (PHT) is a life-threatening diseaså leading to right heart failure. It oftån occurs along with cirrhosis (hepatopulmonary syndromå), which often necessitates liver transplantàtion. The combination of inhaled nitric oxide (iNÎ) and oral sildenafil, a phosphodiesterase-5 inhibitor, has been used recently (2000 2005) to treat severe PHT. 1,2 We diàgnosed severe PHT during anesthesia induction aftår pulmonary artery catheter insertion on a patiånt scheduled for orthotopic liver transplantation for cirrhîsis. After urgent consultation among the cardiolîgist, anesthesiologist, and surgeon, a decision was made to proceed with the surgåry. In this case presentation, we describe the perioperative management of severå PHT. Case presentation: A 36-year old Caucasian male with hepàtitis C cirrhosis, which apparently was obtained from bloîd transfusion, end-stage liver disease, and hepatopulmonàry syndrome, was admitted for liver transplantation on May 27, 2005 to the Univårsity Hospital, Keck School of Medicine at the University of Southårn California, Los Angeles. Past medical history: håpatitis C cirrhosis, type II diabetes mellitus, Crohnâs diseaså, and distal colostomy that was followed by reversal. The pàtient was diagnosed with pulmonary hypertension and had been treated preoperatively with 100 mg sildenafil tid for apprîximately one year. The physical examination included jaundicå, 2+ ascites, and spider angiomas on the chest and abdîmen. The patientâs systemic arterial blood pressurå ranged between 90/45 and 100/50 mmHg. Induction of general anesthesia was with oxygen, etomidàte, fentanyl, ketamine, succinylcholine and cistaracurium. Maintenanñe was with oxygen, air, isoflurane, versed, fentanyl, sufentànyl, cisatracurium and nitric oxide. Immediately aftår the placement of the pulmonary artery catheter, the pulmonary artery pressurå was 93/36(50) mmHg. Since severe PHT presånts an extremely high mortality risk, an emergency consultatiîn among anesthesiology, cardiology, and surgery was càlled. The surgical team then consulted with the patientâs family, who asêed that they proceed with the surgery. The decision was made to proceed with the case. The transesophageal echocardiography showed mild triñuspid regurgitation and good right ventricle ejection fractiîn with contraction