Portopulmonary hypertension: Still an appropriate consideration for liver transplantation?

Abstract

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 article. A retrospective analysis of patients, preoperatively fulfilling the PoPH European Respiratory Society Task Force on Pulmonary-Hepatic Vascular Disorders diagnostic criteria was conducted across all UK LT centers. Data collection included comorbidities, use of preoperative and postoperative pharmacotherapy, patient survival, and cause of death. To enable survival stratification, PoPH was classified as mild, moderate, or severe based on mean pulmonary pressure of <35 mm Hg, 35-49 mm Hg, and ≥50 mm Hg, respectively. Of 127 patients reported to have PoPH, just 28 fulfilled the diagnostic criteria (14 mild, 9 moderate, 5 severe). Twenty (71.4%) patients were male with median age and Model for End-Stage Liver Disease of 50 years (range, 23-62 years) and 18 (range, 6-43), respectively. Twelve (42.9%) patients died within 5 years of LT. The majority of deaths (10 of 12; 83%) occurred within the first 6 months after LT, aetiologies of which included 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 to moderate PoPH. Both severe PoPH patients optimized preoperatively with pharmacotherapy died within a year of LT. 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 The available data indicate that in this era of pharmacotherapy, PoPH in isolation no longer represents a valid consideration to transplant. Liver Transplantation 22 1637-1642 2016 AASLD.

Liver transplantation and cardiac illness: Current evidences and future directions

“…There were equal numbers (n = 14) of patients with mild and moderate to severe PoPH in this series. Furthermore, 62.5% of survival was noted in patients who received preoperative vasodilator therapy . Stabilization or reversibility of PoPH appears to be an attainable goal using the combination of pulmonary vasodilators and liver transplantation in patients with PoPH.…”

Acute Respiratory Failure and Pulmonary Complications in End-Stage Liver Disease

“…Verma et al in 2016 showed that PoPH patients who undergo OLT still have high mortality, with 12/28 (43%) patients dying after transplant by 5 years. 9 Risk stratification of these patients is important, as those patients with uncontrolled hemodynamics despite aggressive advanced PH management have unacceptably high mortality. It appears that it is likely safe to transplant candidates with PoPH who have a mPAP 35 mm Hg with a PVR 400 dynes•s•cm À5 ( 5 Wood units), and this is reflected in the MELD exception program in the United States, in which these patients are granted points to gain priority on the waitlist.…”

“…30 Currently, PoPH, by itself, is not uniformly considered an absolute indication for OLT given the unpredictability and poor outcomes even after transplant. 9,30 Patients with PoPH who have other indications for OLT should be treated with advanced PH therapies as needed to achieve hemodynamics required for safe OLT.…”

“…30 One and 3-year survival for PoPH patients who undergo OLT is 85 and 81%, 32 while 5-year survival is approximately 57%. 9 As a comparison, survival for all primary liver transplant patients in the United States at 1, 3, and 5 years is 91.8, 83.8, and 76.1%, respectively. 33 Improvements in survival for PoPH patients will depend on advances in PH treatments and general OLT management, as well as better patient selection.…”

Portopulmonary hypertension and liver transplantation: A duo in question

“…The 7‐center UK portopulmonary hypertension (POPH) liver transplantation (LT) experience from 1992 to 2012 published in this issue of Liver Transplantation raises important issues in terms of diagnostics, application of aggressive medical treatments, and an evolving “era effect.” At the same time, due to a lack of a standardized approach to the preoperative evaluation, preoperative treatment, and detail regarding postoperative outcomes, such a multicenter effort, not unexpectedly, has deficiencies.…”