Chest
Volume 118, Issue 5, November 2000, Pages 1497-1500
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Selected Reports
Progressive Portopulmonary Hypertension After Liver Transplantation Treated With Epoprostenol

https://doi.org/10.1378/chest.118.5.1497Get rights and content

Portopulmonary hypertension (PPHTN) is an uncommon complication ofadvanced liver disease. Epoprostenol has been effective in thetreatment of PPHTN and has been used as a bridge to orthotopic livertransplantation (OLT). The role of OLT in the reversal of PPHTN isunclear. We report a case of severe PPHTN (mean pulmonary arterypressure of 45 mm Hg) that progressed after OLT. Acute dosing withepoprostenol improved the pulmonary vascular resistance by 55% and thecardiac index by 134%. Hemodynamic and symptomatic improvements weremaintained after 18 months of long-term treatment with epoprostenol.This is the first reported case of a successful favorable outcome aftertreatment for progressive PPHTN after OLT. Our case report complementsprevious reports by highlighting the potential effective use ofepoprostenol as a definitive treatment for PPHTN.

Section snippets

Case Report

A 32-year-old white woman was referred to the Advanced LungDisease Service at the Cleveland Clinic Foundation forprogressive dyspnea. When she was 14 years old, she had received adiagnosis of seronegative chronic active hepatitis, based on a liverbiopsy. At the age of 23 years, she had undergone a splenorenal shuntfor symptomatic esophageal varices. At age 27 years, she had undergonean uncomplicated OLT, with shunt removal and intraoperativesplenectomy. The results of a preoperative

Discussion

Portopulmonary hypertension is an uncommon syndrome that occurs in< 2% of patients with portal hypertension and is present in bothcirrhotic and noncirrhotic liver disease.6 The prognosisof PPHTN is poor, with a mean survival of 15 months afterdiagnosis.1 The histologic picture of PPHTN is similar tothat of primary pulmonary hypertension (ie, medialhypertrophy, intimal fibrosis, and plexogenic pulmonaryarteriopathy).7 The pathogenesis of PPHTN is poorlyunderstood, although several mechanisms

Conclusions

PPHTN is a rare disease with a high mortality rate and limitedtreatment options. Epoprostenol has been used to treat PPHTN and hasbeen used as a bridge to OLT. With improvement of hepatocellularfunction and reversal of portosystemic shunts, PPHTN is potentiallyreversible after OLT. However, PPHTN may persist, progress, or developafter OLT. This case report illustrates the use of epoprostenol as adefinitive treatment for PPHTN that progresses after transplantation.With the availability of

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    Literature reviews have suggested that most patients with PPHTN show improved or normalized pulmonary artery pressures, although this may take up to 2 years.15 Nevertheless, persistence, progression, recurrence, and even de novo evolution of pulmonary hypertension after OLT have also been observed.13,15–18 It is noteworthy that the present case represents the most rapid response among those who showed normalized hemodynamics in the postoperative period.

  • Hemodynamic Profile of Portopulmonary Hypertension

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    When evaluating a candidate with PPHTN for OLT it should be considered that resolution of symptoms following OLT may take months or longer. Persistence, or even progression of disease, has also been reported.27 This implies that both the patient and the transplanted graft have to face an increased pressure in the right heart chambers for a long time as well as the delayed or incomplete remodeling of pulmonary vasculature.

  • Portopulmonary hypertension: State of the art

    2008, Annals of Hepatology
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    For LT candidates with documented moderate to severe POPH (mPAP > 35 mmHg) who will have vasodilator therapy initiated, the goal of therapy should be to reduce the mPAP to < 35 mmHg and the PVR < 400 dynes/ s/cm-5 before proceeding to LT.3 The reason for this cutoff is that previous data have demonstrated no increased mortality risk when the mPAP is < 35 mmHg.9,27 A recent report from Sussman and coworkers,71 demonstrated that in 8 patients with POPH as their only contraindication for LT, pretreatment with intravenous epoprostenol caused a significant improvement in their hemodynamics, allowing 75% to be listed for LT. There have been reports of persistence of progression of PAH even after LT.72,73 Recurrence of PAH following failure of the transplanted liver can also occur.74 A multicenter database was organized at 10 LT referral centers to identify the outcome of patients with POPH being evaluated for LT between 1996 and 2001.67

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