PURPOSE: The hepatopulmonary syndrome (HPS) is defined by the presence of portal hypertension, intrapulmonary vascular dilatations (IPVDs) and hypoxemia (alveolar-arterial gradient (AaDO2) > 20 mm Hg). HPS is common among cirrhotic subjects, with a prevalence of 16-24%. The natural history of this condition is dismal, with a mean survival of only 2.5 years after diagnosis. Liver transplantation (LT) is the only known treatment for HPS, but previous reports have indicated that LT in severe HPS, with a pre-transplant partial pressure of arterial oxygen (PaO2) of ≤; 50 mm Hg carries a very high peri-operative mortality. We sought to review our own center's experience in LT in HPS.
METHODS: 5 HPS patients (ages 31-62 yrs, 3 male) were referred to a specialized HPS Clinic at the University of Toronto between 06/2004 –02/2006, and received LTs between 07/2005 –10/2006. Patients 1 and 3 had living-related LTs, and patients 2, 4 and 5 had deceased-donor LTs. A retrospective chart review was performed at both institutions.
RESULTS: All 5 patients survived LT and had subsequent improvement in PaO2 or saturation (Figure 1). Exhaled nitric oxide (eNO) levels decreased in all patients (4) and DLCO increased in 3/4 patients (Table 1). Duration of mechanical ventilation ranged from 1-60 days, and duration of hospitalization from 12-93 days. Unique post-operative complications were as follows: patient 1, anastamotic bile leak with Candida albicans peritonitis and sepsis; patient 2, acute oliguric renal failure requiring dialysis, and severe delirium; patient 3, refractory post-operative hypoxemia requiring high-frequency oscillatory ventilation, Trendelenburg positioning, and inhaled nitric oxide (NO); patient 5, urosepsis. Currently, all 5 patients are alive, with follow-up ranging between 6-21 months.
CONCLUSION: LT in HPS is associated with a high risk of severe post-operative complications, but we have shown an excellent survival rate in this small series. Improvement in gas-exchange occurred in all patients, but timing to improvement was variable.
CLINICAL IMPLICATIONS: Patients with HPS, even when severe, should be considered for living-related or deceased-donor LT.
DISCLOSURE: Samir Gupta, No Financial Disclosure Information; No Product/Research Disclosure Information