Portopulmonary hypertension: Still an appropriate consideration for liver transplantation?

Liver Transplantation(LT) in patients with portopulmonary hypertension(PoPH) has historically resulted in unpredictable and often poor outcomes. The United Kingdom experience for the period 1992-2012 is reported in this paper. Methods: A retrospective analysis of patients, pre-operatively fulfilling the PoPH European Respiratory Society Task Force on Pulmonary-Hepatic Vascular Disorders diagnostic criteria was conducted across all UK liver transplant centres. Data collection included co-morbidities, use of pre- and post- operative pharmacotherapy, patient survival, and cause of death. To enable survival stratification, PoPH was classified as mild, moderate or severe based on mean pulmonary pressure(MPAP) of <35mmHg, 35-49mmHg and ≥50 mmHg respectively. Results: Of 127 patients reported to have PoPH, just 28 fulfilled the diagnostic criteria (14 mild, 9 moderate, 5 severe). Twenty patients (71.4%) were male with median age and MELD of 50 years (range 23-62) and 18(range 6-43), respectively. Twelve (42.8%) patients died within five years of LT. The majority of deaths (10 of 12; 83%) occurred within the first 6 months post-LT, due to right heart failure (n= 3), progressive PoPH(n= 2) and sepsis(n= 2). Of those receiving preoperative pharmacotherapy(n=8), 5 are currently alive and were classified as mild-moderate PoPH. Both severe PoPH patients optimised pre-operatively with pharmacotherapy died within a year of LT. Conclusion: Development of effective vasodilatory therapies in the setting of pulmonary arterial hypertension has led to a dramatic improvement in patient survival. The available data indicate that in this era of pharmacotherapy, PoPH in isolation no longer represents a valid consideration to transplant. This article is protected by copyright. All rights reserved.