Successful Liver Transplantation Following Medical Management of Portopulmonary Hypertension: A Single-Center Series

Abstract

Severe portopulmonary hypertension (POPH) is an absolute contraindication to orthotopic liver transplantation (OLT). Vasodilators have been used, but the safety of subsequent transplantation and the reversibility of pulmonary hypertension after transplantation are uncertain. This study examined the feasibility and post-transplant effects of liver transplantation following medical control of POPH. Eight consecutive patients (three females and five males, ages 39-51) with POPH as their only contraindication to transplantation were treated with continuous intravenous epoprostenol. Liver transplantation was considered if the mean pulmonary artery pressure (PAM) was lowered to <35 mmHg. Epoprostenol 2-8 ng/kg/min successfully improved hemodynamics in seven of eight patients, usually within 6.5 months of initiating therapy. PAM declined from an average of 43-33 mmHg (p = 0.03); mean pulmonary vascular resistance declined from 410 to 192 dyn s cm −5 (p = 0.01) and cardiac output increased from 6.6 to 10 L/min (p = 0.02). Six of the seven responders were actively listed for liver transplantation. Two died on the waiting list; the remaining four were transplanted and remain alive and well 9-18 months post-OLTtwo without vasodilators, and two on oral medication. We conclude that pulmonary vasodilators permit safe liver transplantation in some cases, and that POPH may be reversible after transplantation.

Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview

“…In one of the largest series, epoprostenol was shown to improve pulmonary haemodynamics and cardiac output in both the acute and long-term setting [39]. These findings have been reproduced by other authors [40,41]. Other PAs have also been evaluated in the management of POPH.…”

“…Our understanding of the impact of PAH-specific therapies that produce improvements in cardiopulmonary haemodynamics to a mean Ppa ,35 mmHg and a PVR ,250 dyn?s?cm -5 REVIEW: POPH AND HPSon post-transplant outcomes is still evolving. Patients who achieve improvements in mean Ppa and PVR to these levels on therapy should be considered potential candidates for LTx [29,30,40,41].…”

“…Initial experience indicated that post-transplant improvement in ''POPH'' was limited to patients with PH secondary to an increased cardiac output (elevated mean Ppa but PVR ,240 dyn?s?cm -5 ). Accumulating evidence from different transplant centres is now manifesting post-transplant improvement in selected POPH patients, some of them being able to wean-off their PAH-specific therapy a few months after LTx (probably ,50% of those surviving the early months after LTx) [33,40,54,56]. Although these are encouraging results, evidence derives from retrospective analyses of transplant databases or case series.…”

Clinical implications of the hyperdynamic syndrome in cirrhosis

“…OLT is a potentially curative therapeutic option for POPH patients. The mortality rate after OLT is 50% if the MPAP is N35 mm Hg, and 100% if the MPAP is N 50 mm Hg [70]. In fact, efficient pharmacologic control of POPH before transplantation is associated with post-transplant survival that is similar to that of patients transplanted for other indications [70,71].…”

Caring for the orphan's orphan: treatment of patients with portopulmonary hypertension

“…First, this is one of the larger studies of the impact of therapy in PPHTN in the recent era [4][5][6][7][8]. While this distinction is more a testament to the relatively small number of patients with PPHTN included in epidemiological studies rather than the absolute magnitude of the current study sample, clinical researchers need to follow the lead of HOEPER et al [3] in forming multicentre national and international consortia in order to study meaningful numbers of these patients over time.…”