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Perioperative Management of Severe Pulmonary Hypertension with Inhaled Nitric Oxide and Oral Sildenafil (ViagraTM) During Orthotopic Liver Transplantation. Case Presentation.
Angel Jose deLeon Vaca, M.D.†, Earl M. Strum, M.D.†, Janos Szenohradszki, M.D. Ph.D.†, Linda Sher, M.D.‡, Robert Selby, M.D.‡
Departments of Anesthesiologyand Surgery‡, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033
Introduction: Pulmonary hypertension (PHT) is a life-threatening disease leading to
right heart failure. It often occurs along with cirrhosis (hepatopulmonary syndrome),
which often necessitates liver transplantation. The combination of inhaled nitric oxide
(iNO) and oral sildenafil, a phosphodiesterase-5 inhibitor, has been used recently (2000-
2005) to treat severe PHT.1,2 We diagnosed severe PHT during anesthesia induction after
pulmonary artery catheter insertion on a patient scheduled for orthotopic liver
transplantation for cirrhosis. After urgent consultation among the cardiologist,
anesthesiologist, and surgeon, a decision was made to proceed with the surgery.
In this case presentation, we describe the perioperative management of severe PHT.
Case presentation: A 36-year old Caucasian male with hepatitis C cirrhosis, which
apparently was obtained from blood transfusion, end-stage liver disease, and
hepatopulmonary syndrome, was admitted for liver transplantation on May 27, 2005 to
the University Hospital, Keck School of Medicine at the University of Southern
California, Los Angeles. Past medical history: hepatitis C cirrhosis, type II diabetes
mellitus, Crohn’s disease, and distal colostomy that was followed by reversal. The patient
was diagnosed with pulmonary hypertension and had been treated preoperatively with
100 mg sildenafil tid for approximately one year. The physical examination included
jaundice, 2+ ascites, and spider angiomas on the chest and abdomen. The patient’s
systemic arterial blood pressure ranged between 90/45 and 100/50 mmHg. Induction of
general anesthesia was with oxygen, etomidate, fentanyl, ketamine, succinylcholine and
cistaracurium. Maintenance was with oxygen, air, isoflurane, versed, fentanyl, sufentanyl,
cisatracurium and nitric oxide. Immediately after the placement of the pulmonary artery
catheter, the pulmonary artery pressure was 93/36(50) mmHg. Since severe PHT presents
an extremely high mortality risk, an emergency consultation among anesthesiology,
cardiology, and surgery was called. The surgical team then consulted with the patient’s
family, who asked that they proceed with the surgery. The decision was made to proceed
with the case. The transesophageal echocardiography showed mild tricuspid regurgitation
and good right ventricle ejection fraction with contraction. His right ventricle wall motion
was good. To lower the high pulmonary artery pressure, inhaled nitric oxide (iNO) was
added to the inhalation agent 90 min after the induction of anesthesia and was continued
until the end of surgery. 150 min after the induction of anesthesia 100 mg sildenafil was
administered sublingually. Pulmonary artery pressure decreased and was stable
throughout the remainder of surgery. The average value was 86/34(46) mmHg. The
patient had supraventricular tachycardia intermittently throughout the surgery, which
improved after IV lidocain, amiodarone and magnesium administration. Postoperatively,
the patient was digitalized and received lasix. The pulmonary and systemic blood
pressure remained stable. In the early postoperative period the patient continued to
receive oral sildenafil 100 mg tid.
Conclusion: According to recent publications, the combined intraoperative
administration of inhaled nitric oxide and oral sildenafil may make it possible to proceed
with liver transplantation that is complicated by severe pulmonary hypertension. We have
shown in this case report that the continuous inhalation of nitric oxide significantly
decreased the very high pulmonary artery systolic pressure during the orthotopic liver
transplantation. The hemodynamic variables were stable throughout the surgery. We
cannot demonstrate, however, that there was an additional beneficial effect of the
sildenafil given one hour after the start of inhaled nitric oxide administration. It is not
known how the chronic preoperative sildenafil treatment influences the effect of
intraoperatively administered sildenafil on pulmonary hypertension.
1. HA Ghofrani, R Wiedemann, F Rose, H Olschewski, RT Schermuly, N Weismann,
W Seeger, and F Grimminger: Combination Therapy with Oral Sildenafil and Inhaled
Iloprost for Severe Pulmonary Hypertension. Annals of Internal Medicine 136: 515-522,
2. E Michelakis, W Tymchak, D Lien, L Webster, K Hashimoto, S Archer: Oral
Sildenafil Is an Effective and Specific Pulmonary Vasodilator in Patients With
Pulmonary Arterial Hypertension. Comparison with Inhaled Nitric Oxide. Circulation.
105:2395-2400, 2002.


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