Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

Abstract

Hepatopulmonary syndrome (HPS) is a pulmonary vascular disorder occurring as a consequence of advanced liver disease, characterized by hypoxemia due to intrapulmonary vascular dilatations. HPS independently increases mortality, regardless of the cause or severity of liver disease. Liver transplantation (LT) improves survival in HPS. We present the largest consecutive series of HPS patients specifically addressing long-term survival relative to the degree of hypoxemia and the era in which LT was conducted. We evaluated 106 HPS patients at the Mayo Clinic from 1986 through 2010. Survival was assessed using Kaplan-Meier methodology. LT was accomplished in 49 HPS patients. Post-LT survival (1, 3, 5, and 10 years) did not differ between groups based on baseline partial pressure of arterial oxygen (PaO 2 ) obtained at the time of HPS diagnosis. Improvements in overall survival at 1, 3, and 5 years post-LT in those HPS patients transplanted after January 1 2002 (n 5 28) (92%, 88%, and 88%, respectively) as compared with those transplanted prior to that time (n 5 21) (71%, 67%, and 67%, respectively) did not reach statistical significance (5-year P 5 0.09). Model for Endstage Liver Disease (MELD) exception to facilitate LT was granted to 21 patients since January 1 2002 with post-LT survival of 19/21 patients and one wait-list death. Conclusion: Long-term outcome after LT in HPS is favorable, with a trend towards improved survival in the MELD exception era since 2002 as compared to earlier HPS transplants. Survival after LT was not associated with PaO 2 levels at the time of HPS diagnosis. (HEPATOLOGY 2013;57:2427-2435 See Editorial on Page 2097 H epatopulmonary syndrome (HPS) is a pulmonary vascular disorder characterized by a clinical triad of hepatic dysfunction (usually with portal hypertension), arterial hypoxemia, and intrapulmonary vascular dilatations. HPS is not uncommon and affects between 5%-32% of individuals being assessed for liver transplantation (LT), depending on the criteria used to define arterial hypoxemia. 1 LT is the only therapy that has been shown to consistently and significantly improve or resolve HPS.Mortality associated with HPS, however, can be significant and not necessarily related to the severity of liver disease as measured by MELD (Model for Endstage Liver Disease) or CTP (Child-Turcotte-Pugh) scores. For those HPS patients with partial pressure of arterial oxygen (PaO 2 ) <60 mmHg, a MELD exception has been granted in an attempt to improve survival for those with no other contraindications to LT. 2 In the pre-MELD exception era (up to 2002), we had previously shown that HPS has a poor prognosis, with a 5-year survival of 23% without LT as compared to a 76% survival with LT. 3 In this report we present additional and extended 10-year survival data for our cohort of 106 patients with HPS, including 49 patients who underwent LT since the inception of our LT program. We aimed to further define the role of arterial hypoxemia as measured by baseline PaO 2 (at the time of HPS diagnosis) in ...

Inflammatory bowel diseases, chronic liver diseases and the lung

“…Some studies have suggested that very severe arterial hypoxaemia in HPS confers a worse prognosis with or without liver transplant [129,131,132], an observation that has not been replicated in all series [133].…”

Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

“…Pharmacological agents have not proven to be uniformly beneficial in HPS, and liver transplantation (LT) is currently the only effective treatment option available for these patients. [3][4][5] POPH, on the other hand, involves elevated pulmonary artery (PA) pressures (mean, MPAP) and increased PVR, as measured by right heart catheterization (RHC), because of generalized pulmonary arterial vasoconstriction and proliferative vasculopathy, with microthrombi, vascular remodeling, and plexiform changes. Symptoms include dyspnea, along with signs of right heart failure.…”

Use of Orthodeoxia by Pulse Oximetry in the Detection of Hepatopulmonary Syndrome

“…Among other studies there´s the TAC and the single chest chamber, however it´s specificity and sensibility are low, for this reason its utilization would be indicated more to discard any respiratory etiology that may be causing hypoxemia [7][8][9][10][11]. Within the treatment, in some bibliographies, supplementary oxygen and hepatic transplant are mentioned as the only satisfactory answer.…”