The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

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 Patients and Methods Patient

Portopulmonary hypertension (POPH) is the elevation of pulmonary artery pressure due to increased resistance to pulmonary blood flow in the setting of portal hypertension. Increased mortality has occurred with attempted liver transplantation in such patients and thus, screening for POPH is advised. We examined the relationship between screening echocardiography and right heart catheterization determinations of pressure, flow, volume, and resistance. A prospective, echocardiography-catheterization algorithm was followed from P ulmonary hypertension associated with advanced liver disease has variable etiologies and prognostic implication. [1][2][3][4] First, the hyperdynamic, high-flow circulatory state (as a consequence of splanchnic vasodilatation caused by portal hypertension) results in high cardiac output (CO) and an increase in mean pulmonary artery pressure (MPAP), but the pulmonary vascular resistance (PVR) remains normal. Second, elevated MPAP with increased central blood volume estimate as measured by the pulmonary arterial occlusion pressure (PAOP) results in variable effect on PVR. Third, for reasons yet to be characterized, and regardless of liver disease severity, additional hemodynamic change can occur due to development of pulmonary artery endothelial/smooth muscle proliferation, vasoconstriction, in situ thrombosis, and plexogenic arteriopathy. Such pathology results in progressive obstruction to pulmonary arterial flow from the right ventricle to the lungs, very high pulmonary artery

Nigro and Neisser (1983) contrasted two ways of remembering personal experiences: the rememberer may 'see' the event from his or her perspective as in normal perception, or 'see' the self engaged in the event as an observer would. Several factors contribute to the determination of perspective, but Nigro and Neisser also reported that many subjects claimed they could change to another perspective at will. We sampled personal memories from several life periods and assessed ability to change the initially reported perspective. Changing was easier for recent or vividly recalled events, harder for older and less vividly recalled events. Memory perspectives may differ in other aspects than their imagery. A second study was conducted to determine whether affective experience is altered when perspectives are changed. The affect experienced decreased when shifting from a field to an observer perspective, but did not change with the converse shift. These studies provide further evidence that remembering is more than retrieval. The information that enters awareness is determined by the information sources in memory and the organisational scheme adopted for recollection.

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To determine the natural history of portopulmonary hypertension (POPH), a retrospective screening-right heart catheterization-survival analysis of patients was performed. We categorized patients by three treatment subgroups: (1) no therapy for pulmonary hypertension (PH) or liver transplantation (LT), (2) therapy for PH alone and (3) therapy for PH followed by LT. Seventyfour patients were identified between 1994 and 2007. Nineteen patients received no therapy for PH and no LT representing the natural history of POPH. Five-year survival was 14%, and 54% had died within 1 year of diagnosis. Five-year survival in 43 patients receiving therapy for PH but no LT was 45%, and 12% had died within 1 year of diagnosis. Twelve patients underwent LT and 5-year survival for the nine receiving therapy for PH was 67% versus 25% in the three who were not pretreated with prostacyclin therapy. The survival of untreated patients with POPH was poor. Subgroups of patients selected to medical treatment with or without LT had better long-term survival. Mortality did not correlate with baseline hemodynamic variables, type of liver disease or severity of hepatic dysfunction. Medical therapy for POPH should be considered in all patients with POPH, but the treatment effects and impact on those considered for LT still requires well-designed, prospective study before practice guidelines can be suggested.

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