Portopulmonary hypertension (PPHTN) represents a constrictive pulmonary vasculopathy in patients with portal hypertension. Liver transplantation (LT) may be curative and is usually restricted to patients with mild-to-moderate disease severity characterized by a mean pulmonary artery pressure (mPAP Ͻ 35 mm Hg). Patients with severe disease (mPAP Ͼ 50 mm Hg) are usually excluded from transplantation. We describe a patient with severe PPHTN, initiated on sequential and ultimately combination therapy of prostacyclin, sildenafil, and bosentan (PSB) pretransplantation and continued for 2 years posttransplantation. Peak mPAP on PSB therapy was dramatically reduced from 70 mm Hg to 32 mm Hg pretransplantation, and continued therapy facilitated a further fall in mPAP to 28 mm Hg posttransplantation. The pulmonary vascular resistance index fell from 604 to 291 dyne second Ϫ1 cm Ϫ5 . The perioperative mPAP rose to 100 mm Hg following an episode of sepsis and fell with optimization of PSB therapy. In conclusion, this is the first reported patient with severe PPHTN using this combination of vasodilator therapy as a bridge to LT and then as maintenance in the posttransplantation phase. This regimen may enable LT in similar patients in the future, without long-term consequences. Liver Transpl 14: [287][288][289][290][291] 2008.
See Editorial on Page 270Portopulmonary hypertension (PPHTN) is defined as the development of pulmonary arterial hypertension associated with liver disease or portal hypertension. 1 The diagnostic criteria for PPHTN are a mean pulmonary artery pressure (mPAP) Ͼ 25 mm Hg at rest, mPAP Ͼ 30 mm Hg during exercise, a normal capillary wedge pressure (Ͻ15 mm Hg), and an elevated pulmonary vascular resistance index (Ͼ240 dyne/second/cm Ϫ5 ) alongside portal hypertension (with or without cirrhosis). [2][3][4][5] The recognized noncirrhotic causes of portal hypertension associated with PPHTN include extrahepatic portal vein obstruction, congenital hepatic fibrosis, nodular regenerative hyperplasia, and hepatoportal sclerosis. [5][6][7] Patients with PPHTN have nonspecific symptoms such as worsening fatigue, dyspnea, and peripheral edema. Men and women are equally affected. 7 No clear relationship exists between severity of liver disease, degree of portal hypertension, and severity of PPHTN. 8,9 Treatment of PPHTN is currently limited by lack of randomized controlled trials, concerns related to drug delivery, safety profiles, and failure of reversibility of the pulmonary disease. 10,11 Vasodilators, in particular, Abbreviations: LT, liver transplantation; mPAP, mean pulmonary artery pressure; NR, normal range; PPHTN, portopulmonary hypertension; PSB, prostacyclin, sildenafil, and bosentan.