Hepatopulmonary syndrome (HPS) is a progressive, debilitating complication of end-stage liver disease. In contrast to the well-established reversal of HPS after deceased donor liver transplantation (DDLT), little has been written about the natural course of HPS after the newer procedure of living donor liver transplantation (LDLT). We describe HPS in a small series of 4 liver transplant recipients (2 DDLT; 2 LDLT) at a single center. Before transplantation, these 4 patients had a mean shunt fraction of 23.6 ุ 14.3% and a mean PaO 2 of 58.5 ุ 11.3 mm Hg. All 4 patients used supplemental oxygen before transplantation. Sixteen weeks after transplantation, all 4 patients had normalized or improved shunt fraction and PaO 2 . These patients regained normal pulmonary function within a few months, despite the period of hepatic regeneration after LDLT. In conclusion, both DDLT and LDLT are associated with rapid and dramatic reversal of HPS. (Liver Transpl 2004;10: 529-533.)
H epatopulmonary syndrome (HPS) is a progres- sive, debilitating complication of end-stage liver disease that occurs in 4 to 22% of liver transplant candidates. 1 -3 The diagnosis of HPS rests on the triad of cirrhosis, hypoxemia, and intrapulmonary vascular dilation. 4,5 HPS can greatly impair quality of life. Patients suffer from progressive dyspnea and often require supplemental oxygen. Several medications have been used to treat HPS, but the results have been disappointing. 5 Liver transplantation is the only known cure for HPS. 5 The onset of HPS may be an indicator of poor prognosis. In the absence of transplantation, patients with HPS have a mortality rate as high as 41% within 2.5 years after the onset of symptoms. 6 Although the reversal of HPS after deceased donor liver transplantation (DDLT) is well established, 1,5,7 the course of HPS after living donor liver transplantation (LDLT) is not well described. Japanese reports have documented the reversal of HPS in children after LDLT, 8 but there is little information on the course of HPS after LDLT in the United States. We describe our experience with HPS after DDLT and LDLT in a single-center setting.