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Abstract. Kjeldsen AD, Oxhùj H, Andersen PE, Green A, Vase P (Odense University and Odense University Hospital, Odense; University of Aarhus, Aarhus; and the Department of Otorhinolaryngology, Svendborg, Denmark). Prevalence of pulmonary arteriovenous malformations (PAVMs) and occurrence of neurological symptoms in patients with hereditary haemorrhagic telangiectasia (HHT). J Intern Med 2000; 248: 255±262.Background. Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease. HHT is characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs) and neurological symptoms. Objective. To estimate (i) the prevalence of PAVM, and (ii) the occurrence of neurological symptoms in a geographical well-defined population of HHT patients. Methods. HHT family members were invited to a clinical examination including registration of HHT manifestations, screening for pulmonary arteriovenous malformations and neurological evaluation. Two groups served as controls: (i) first-degree relatives without any signs of HHT; and (ii) age-and gendermatched controls.

Echocardiographic estimates of left ventricular ejection fraction (ECHO-LVEF) in acute myocardial infarction (AMI) were obtained by a new approach, using visual analysis of left ventricular wall motion in a nine-segment model. The method was validated in 41 patients using radionuclide ventriculography (RNV) and contrast ventriculography measurements of LVEF for comparison. ECHO-LVEF from the 41 patients correlated well with the reference methods (y = 1.5x – 14.7, r = 0.93; linear regression analysis; 95 % confidence limit for a single determination of ECHO-LVEF was 17.2). Interobserver variability by linear regression was r = 0.89, SEE = 7.1 with a mean difference between paired observations of-1.5 ± 6.9 (SD). In a random sample of 18 patients (45 observations), ECHO-LVEF allowed separation between RNV-LVEF values ≧ 40 and < 40, representing low and high risk groups following AMI. Thus, the results showed that simple, readily available wall motion-derived estimates of LVEF were as closely associated with LVEF measured by standard reference methods as were previously published, more cumbersome, plani-metric echocardiographic methods. Reporting on global LVEF function in LVEF units rather than in nonstandardized wall motion scores of index values may facilitate intra- and interhospital communication and the use of optimized echocardiographic risk stratification after AMI.