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 correlation with the severity or cause of the underlying liver disease. [1][2][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. TcMAA. From the
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