Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension.
Portopulmonary hypertension (PoPH) refers to pulmonary arterial hypertension associated with portal hypertension with or without evidence of an underlying liver disease. Despite the potential for curing PoPH with liver transplantation, the presence of moderate or severe PoPH is associated with increased morbidity and mortality and is, therefore, a contraindication to transplantation. Previous studies have predominantly used intravenous epoprostenol for treatment in order to qualify patients for liver transplantation. In this retrospective case series, we describe the clinical course of 11 patients whom we successfully treated (predominantly with oral sildenafil and subcutaneous treprostinil) in order to qualify them for liver transplantation. The mean pulmonary artery pressure significantly improved from 44 to 32.9 mm Hg, and the pulmonary vascular resistance decreased from 431 to 173 dyn second cm(-5) . There were significant improvements in the cardiac output and the transpulmonary gradient with these therapies as well. All 11 patients subsequently received liver transplants with a 0% mortality rate to date; the duration of follow-up ranged from 7 to 60 months. After transplantation, 7 of the 11 patients (64%) were off all pulmonary vasodilators, and only 2 patients required transiently increased doses of prostacyclins. In conclusion, an aggressive approach to the treatment of PoPH with sildenafil and/or treprostinil and subsequent liver transplantation may be curative for PoPH in some patients.
Division of Pulmonary and Critical Care, Department of Medicine, University of Wisconsin School of Medicine and Public Health, 600 Highland Avenue, Madison, WI 53792, USA.
Trina J
TJ
Hollatz
Alexandru
A
Musat
Susanne
S
Westphal
Catherine
C
Decker
Anthony M
AM
D'Alessandro
Jon
J
Keevil
Li
L
Zhanhai
James R
JR
Runo
D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage; D011464:Epoprostenol / Q000008:administration & dosage / Q000031:analogs & derivatives*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000188:drug therapy*; D007902:Length of Stay; D017093:Liver Failure / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000008:administration & dosage; D010879:Piperazines / Q000008:administration & dosage*; D011183:Postoperative Complications / Q000209:etiology / Q000517:prevention & control; D011652:Pulmonary Circulation / Q000187:drug effects*; D011687:Purines / Q000008:administration & dosage; D012189:Retrospective Studies; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage*
D000959:Antihypertensive Agents; D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate; D011464:Epoprostenol; C427248:treprostinil