Long-term Outcomes and Survival in Moderate-severe Portopulmonary Hypertension After Liver Transplant

Abstract

Background. Portopulmonary hypertension is present in an estimated 5.3% to 8.5% of liver transplant candidates. Untreated, 5-year survival is estimated between 14% and 28%. Moderate-severe disease is a contraindication to liver transplant due to the high perioperative mortality, but patients optimized with pulmonary vasodilator therapy can become eligible for transplant. There is minimal data regarding posttransplant outcomes and ability to discontinue pulmonary vasodilator therapy posttransplant. Methods. We performed a single-center retrospective analysis to evaluate long-term outcomes of patients with moderate-severe portopulmonary hypertension who were optimized with pulmonary vasodilator therapy, became eligible for liver transplant, and subsequently underwent transplant. We identified 24 patients optimized with pulmonary vasodilator therapy who underwent subsequent liver transplantation and 25 patients who were treated with pulmonary vasodilator therapy alone. Results. In the transplanted cohort, 1-year survival from portopulmonary hypertension diagnosis date: 95.8%, 3-year survival: 90.9%, and 5-year survival: 90.9%. Posttransplant; 1-, 3-, and 5-year survival was 86.9%. Among transplanted patients, 41.6% (10/24) were optimized with nonparenteral therapy. Following transplantation, 100% (14/14) of the surviving patients were able to discontinue parenteral therapy; median time: 7.2 months (interquartile range: 5.1–8.9 mo), while 61.9% (13/21) were able to discontinue pulmonary vasodilator therapy altogether; median time: 13.9 months (interquartile range: 5.1–17.6 mo). Conclusions. Patients who are optimized with pulmonary vasodilator therapy before liver transplant can have excellent long-term outcomes posttransplant. Oral pulmonary vasodilator therapy can be effective treatment to qualify a patient for transplant, and the majority are able to wean from pulmonary vasodilator therapy entirely posttransplant.

The Myths and Realities of Portopulmonary Hypertension

“…Included studies that reported survival data from a minimum of 20 patients. (14,(27)(28)(29)(30)32) *Unpublished data. Abbreviations: PH, pulmonary hypertension; N/A, not applicable.…”

Progressive dyspnoea in a patient with idiopathic non-cirrhotic portal hypertension

“…Similarly, transjugular intrahepatic portosystemic shunt placement has no role in the management of PoPH and may increase right ventricular preload and precipitate right heart failure [10]. Careful patient selection and preoperative optimisation of PoPH are associated with a 5-year post liver transplant survival of 78- [6,15].…”

Topic-Based, Recent Literature Review on Pulmonary Hypertension

“…51,52 A single-center experience of 24 POPH patients with excellent outcomes was published by the University of Wisconsin group. 53 All patients were optimized on medical therapy with 1-, 3-, and 5-year survival rates of 96%, 91%, and 91% in the cohort that underwent transplant. Of those receiving parenteral PAH therapy, all were weaned during a median time of 7 months.…”