Few data exist concerning survival after the diagnosis of hepatopulmonary syndrome (HPS). Although orthotopic liver transplantation (OLT) frequently results in complete resolution of HPS, the relationship between transplantation and survival has not been described. The study rationale was to describe long-term survival in patients with HPS. Data were derived from patients diagnosed with HPS at Mayo Clinic (n β«Ψβ¬ 61) between 1985 and 2002, including those undergoing OLT (n β«Ψβ¬ 24) and those who did not (n β«Ψβ¬ 37). A case-control, Kaplan-Meier survival analysis between HPS patients and 77 patients without HPS matched for liver disease cause, model for end-stage liver disease (MELD), severity of liver disease by the Child classification, and age was described for OLT and non-OLT groups. Patients with HPS had a mean partial pressure of arterial oxygen (PaO 2 ) decline of 5.2 Ψ 2.3 mm Hg per year awaiting OLT. For HPS patients, despite similar baseline PaO 2 , brain uptake of technetium macroaggregated albumin ( 99m TcMAA), or measures of hepatic dysfunction, 5-year survival associated with OLT was 76% versus 23% who did not undergo transplantation (P < .0001). Comparing those who did not undergo transplantation, HPS patients had worse 5-year survival than matched controls (P β«Ψβ¬ .0003). However, reasons to deny OLT (comorbidity) in the setting of HPS may well have contributed to observed survival differences. Baseline PaO 2 <50 mm Hg was associated with worse survival irrespective of the decision to perform OLT. In conclusion, hypoxemia of HPS is frequently progressive. As OLT outcome relates to pretransplantation PaO 2 , additional MELD points should advance the priority for OLT in HPS. (HEPATOLOGY 2005;41:1122-1129.)
H epatopulmonary syndrome (HPS) is a pulmonary vascular disorder characterized by the clinical triad of chronic liver disease, intrapulmonary vascular dilatations, and arterial hypoxemia. 1,2 Portal hypertension (with or without cirrhosis) is often present. 2,3 The intrapulmonary vascular dilatations are identified by transthoracic contrast echocardiography (qualitative) or radionuclide lung perfusion scanning with brain uptake to measure shunt fraction (quantitative). 2 The degree of arterial hypoxemia associated with HPS has an unpredictable corre-lation with the severity or cause of the underlying liver disease. 1-3 The mechanism by which portal hypertension results in pulmonary vascular dilatation is unknown but appears to involve local effects of increased nitric oxide. 4 Although orthotopic liver transplantation (OLT) has become the mainstay of therapy for HPS in many centers, few data exist that describe long-term survival. 1,5 Despite the well-documented resolution of HPS after OLT, 1,5,6 we were interested in the long-term clinical course of all patients with this syndrome since the inception of the liver transplantation program at Mayo Clinic Rochester in 1985. Our aims were to determine longterm survival in HPS versus case controls, assess the impact of OLT on survival, and document partial pressure of arterial oxygen (PaO 2 ) change pre-and post-OLT.
Patients and Methods
Patient Selection. All patients with HPS evaluated in the Liver Transplantation Clinic at Mayo Clinic in Rochester, Minnesota, were included in this retrospective analysis. The study protocol conformed to the ethical guidelines of the 1975 Declaration of Helsinki as reflected Abbreviations: HPS, hepatopulmonary syndrome; OLT, orthotopic liver transplantation; 99m TcMAA, technetium macroaggregated albumin; PaO 2 , partial pressure of arterial oxygen; CTP, Child-Turcotte-Pugh; MELD, model for end-stage liver disease; GMT, geometric mean count for 99m TcMAA.