Conference: 2010 International PHA Conference and Scientific Sessions
Release Date: 06.24.2010
Presentation Type: Abstracts
Hollatz T, Musat A, Runo J.
University of Wisconsin-Madison, Madison, WI, USA
BACKGROUND: Portopulmonary hypertension (PoPH) portends a poor prognosis and is an absolute contraindication to liver transplantation due to increased perioperative mortality. Epoprostenol has been used to successfully treat PoPH allowing liver transplantation; however, the data on subcutaneous, oral and inhalational agents is limited.
METHODS: Eight patients (4 men, 4 women) with end stage liver disease and PoPH confirmed on right heart catheterization, ages 46-61, were treated for PoPH and then received orthotopic liver transplant(OLT) at our center. Four patients were treated with subcutaneous treprostinil alone, two patients with sildenafil alone, one patient with both sildenafil and iloprost and one patient with a combination of sildenafil and epoprostenol. The use of epoprostenol or iloprost was initiated after documentation of intolerance to treprostinil. Patients were considered eligible for liver transplantation once the hemodynamics improved to a mean pulmonary artery pressure (mPAP) less than 35 mmHg and/or pulmonary vascular resistance (PVR) less than 240 dyn s cm-5.
RESULTS: The mPAP decreased from a mean of 44 to 30 mmHg and PVR decreased from a mean of 444 to 159 dyn s cm-5 after mean treatment duration of approximately 9 months (range 1-17). Patients were then eligible for liver transplant and were subsequently transplanted with average time on list being five months (range 2-9). The mean dose of treprostinil at time of transplant was 32.5 ng/kg/min (range 19-53) and for sildenafil was 35 mg tid (range 20-40 tid). The doses for the patients on epoprostenol and iloprost were 6 ng/kg/min and 5 mcg inhaled six times daily, respectively. Intraoperatively all patients were treated with either subcutaneous/IV treprostinil or epoprostenol, two patients were additionally treated with inhaled nitric oxide in the perioperative period due to elevated pulmonary artery pressures. Postoperatively one of the eight patients did require increased doses of both epoprostenol and sildenafil due to worsening right heart failure symptoms but subsequently improved and these medications are currently being weaned. The remainder of the patients had no complications related to PoPH post-operatively. Three have been taken off all pulmonary vasodilators and the remaining four are on stable or decreasing doses of their preoperative medications. Follow-up ranges from 2-34 months post-OLT at which time all patients are alive.
CONCLUSIONS: Treatment of moderate to severe PoPH with subcutaneous treprostinil and sildenafil, alone or in combination with other agents, allows previously ineligible patients for liver transplantation to be successfully transplanted with low perioperative morbidity and mortality. In addition, PoPH may be reversible with liver transplantation.