Late recurrence and rapid evolution of severe hepatopulmonary syndrome after liver transplantation

Abstract

Recurrence of hepatopulmonary syndrome (HPS) after orthotopic liver transplantation (OLT) in an adult has never been reported. We describe a 23-year-old woman who initially underwent OLT because of debilitating and severe HPS associated with nonalcoholic steatohepatitis (NASH). Although the clinical resolution of HPS was well documented day 117 post-OLT, the reappearance of NASH was documented by liver biopsy. Severe hypoxemia because of recurrent HPS rapidly evolved beginning approximately day 700 post-OLT. Retransplantation was attempted, but the patient died post-OLT of sepsis and/or multiorgan failure. Copyright 1999 by the American Association for the Study of Liver Diseases H epatopulmonary syndrome (HPS) is defined by the triad of (1) arterial hypoxemia (PaO 2 Ͻ 70 mm Hg or alveolar-arterial oxygen gradient Ͼ 20 mm Hg), (2) intrapulmonary vascular dilatations, and (3) chronic liver disease. 1-3 The current criteria used to infer the existence of pulmonary vascular dilatations include either positive results from a contrast-enhanced transthoracic echocardiography (delayed microbubble opacification in the left atrium greater than three cardiac cycles after right atrial opacification), or abnormal extrapulmonary uptake of technetium-labeled macroaggregated albumin ( 99m TcMAA) after a lung perfusion scan (brain uptake Ͼ5%). 3,4 Chronic liver disease has usually implied cirrhotic or noncirrhotic portal hypertension. 2,3 Complete resolution of HPS after orthotopic liver transplantation (OLT) has been well documented, and some investigators consider this syndrome a valid indication for OLT, 5-7 especially in the pediatric age group. 8 We describe the subsequent recurrence and rapid evolution of severe HPS in a woman approximately 700 days post-OLT who initially underwent transplantation for progressive hypoxemia caused by HPS associated with nonalcoholic steatohepatitis (NASH). Case ReportThe pretransplantation course and posttransplantation clinical resolution of HPS in this patient have been previously reported (Mayo Clinic case no. 3). 5 Briefly, this patient underwent OLT at the age of 23 years because of progressive and severe hypoxemia (PaO 2 , 53 mm Hg standing at rest; 42 mm Hg with exercise, breathing room air) associated with biopsy-proven NASH. Portal hypertension was inferred by the existence of esophageal varices and splenomegly. Delayed, positivecontrast echocardiography and abnormal extrapulmonary (brain) 99m TcMAA uptake after lung perfusion scanning (23%; normal Ͻ5%) were compatible with the existence of intrapulmonary vascular dilatations. The triad of chronic liver disease, arterial hypoxemia, and pulmonary vascular dilatations fulfilled the published criteria for the diagnosis of HPS.By day 117 post-OLT, the patient was asymptomatic, no longer required supplemental oxygen, and had normal oxygenation breathing room air, with a PaO 2 of 76 mm Hg supine and 81 mm Hg standing. The PaO 2 when breathing 100% oxygen was 576 mm Hg supine and 560 mm Hg standing. Brain uptake after a 99m TcMAA lung sc...

Recurrent portopulmonary hypertension after liver transplantation: Management with epoprostenol and resolution after retransplantation

“…One patient was reported to experience recurrent hepatopulmonary syndrome after graft failure, but the patient died before retransplantation and it is not known if a functioning graft would have corrected the syndrome. 27 Our patient not only experienced recurrence of portopulmonary hypertension after graft failure, but also resolution of the syndrome each time hepatic function was restored. Given the rarity of severe portopulmonary hypertension and its recurrence after failure of a liver graft, it seems likely that the predilection for the development of portopulmonary hypertension is based on extrahepatic factors, with hepatic failure as the mediating event.…”

“…Orthotopic liver transplantation has been reported to reverse extrahepatic organ dysfunctions associated with end-stage liver disease, including both hepatopulmonary syndrome and hepatorenal syndrome. [26][27][28] Little is known about the recurrence of end-organ dysfunction after recurrence of liver failure in patients who have received a liver transplant. One patient was reported to experience recurrent hepatopulmonary syndrome after graft failure, but the patient died before retransplantation and it is not known if a functioning graft would have corrected the syndrome.…”

Pulmonary-Hepatic vascular Disorders (PHD)

“…Such patients, depending on local surgical and post-OLT expertise facilities, might benefit from referral to highly specialised transplantation centres with significant HPS experience. Spontaneous recurrence of HPS [150,151] and development of PPHTN before [31] or following OLT for HPS [6,29] have been reported, but appear to be rare events. Liver transplantation from living donors for HPS has been shown to be successful in children [152], but no data are available in adults.…”

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

“…A note of caution: recurrence of HPS after OLT has been reported. 6 HPS associated with noncirrhotic portal hypertension is uncommon. [7][8][9][10] In such patients, some degree of accompanying noncirrhotic liver injury may or may not exist.…”