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Das Fieber unklarer Genese (FUO) bzw. Entzündungskonstellationen mit unklarer Ursache stellen hinsichtlich weiterer Diagnostik und Therapie eine große Herausforderung dar. Zur Klärung der Genese wurde bei Patienten, bei denen trotz intensiver laborchemischer und bildgebender Diagnostik keine sichere Diagnose gestellt werden konnte, die FDG-PET eingesetzt. Da trotz ausgiebiger Diagnostik keine eindeutige Diagnose zu stellen war, wurde bei 9 Patienten eine FDG-PET durchgeführt. Zwei Patientinnen mit Takayasu-Arteriitis (TA) erhielten bei unklarer Krankheitsaktivität, und sieben Patienten mit initial unklarer Entzündungskonstellation bzw. FUO und dem klinischen Verdacht auf Vorliegen einer Vaskulitis der großen Gefäße eine PET. Die zwei Patientinnen mit TA wiesen in der PET eine deutlich vermehrte FDG-Aufnahme in derWand großer Gefäße auf, so dass auf eine floride Vaskulitis geschlossen wurde. Bei sechs der übrigen sieben Patienten wurde mit Hilfe des PET-Befundes die Diagnose einer Vaskulitis gestellt. Bei einer Patientin wurde eine floride Vaskulitis der großen Gefäße durch die FDG-PET weitgehend ausgeschlossen. Die gestellten Diagnosen bestätigten sich anhand der Verlaufsbeobachtung. Schlussfolgerung Die FDG-PET kann insbesondere bei Vaskulitiden der großen Gefäße zur Diagnosestellung, Ausbreitungsdiagnostik und Beurteilung der Krankheitsaktivität von Nutzen sein. Diagnosis and treatment of FUO or systemic inflammation with unknown reason are still a great challenge for the treating physician. We used 18F-FDG-PET for further diagnostic work in patients in whom a diagnosis could not be established despite intensive diagnostic efforts. We studied nine patients with 18F-FDG-PET. Two female patients with known Takayasu’s arteritis but undefineable disease activity, and seven patients with the clinical suspicion of an underlying large vessel vasculitis. The diagnosis of active vasculitis could be confirmed by the PET-results in eight patients. Active vasculitis could be nearly ruled out in one. The diagnoses could be confirmed by follow-up visits. 18F-FDG-PET is a useful diagnostic tool in patients with unclear systemic inflammation and FUO. Especially when large vessel vasculitis is suspected, further diagnostic work by PET seems to be of benefit. Furthermore, it offers the opportunity to evaluate disease activity and to check which vessels are involved.

In the present study, we investigated whether the intravenous injection of air-filled albumin microspheres (Infoson) as a contrast medium improves the echocardiographic quantification of left ventricular enddiastolic and endsystolic volumes, stroke volume, ejection fraction, and regional wall motion in patients with suboptimal endocardial border definition on echocardiography. In 30 adult patients, apical two and four chamber views were performed. In comparison to biplane cineventriculography enddiastolic and endsystolic volumes, stroke volume, ejection fraction, and regional wall function were assessed for heart cycles with and without left ventricular contrast. In comparison to biplane cineventriculography echocardiography underestimates enddiastolic (167±64ml, 111±43; p<0.0001) and endsystolic volumes (77±63 ml, 54±40 ml; p<0.0002), stroke volume (90±25 ml, 57±17 ml; p<0.0001), and ejection fraction (58±16%, 55±14%; p<0.03). By contrast echocardiography ejection fraction (58±16%) agreed with the angiocardiographically measured ejection fraction. Furthermore, after contrast injection correlations improved between cineventriculography and echocardiography for the assessment of left ventricular enddiastolic volumes (without contrast r=0.90, SEE=19 ml; with contrast r=0.93, SEE=19 ml), endsystolic volumes (without contrast r=0.94, SEE=14 ml; with contrast r=0.95, SEE=15ml), stroke volume (without contrast r=0.63, SEE=14 ml; with contrast r=0.67, SEE=14 ml), ejection fraction (without contrast r=0.84, SEE=8%; with contrast r=0.88, SEE=7%), regional wall motion (p<0.01) and its reproducibility (p<0.02). In adult patients with suboptimal endocardial border delineation intravenous contrast echocardiography improves the assessment of left ventricular ejection fraction, regional wall motion, and its reproducibility without severe side effects. In der vorliegenden Studie wurde im Vergleich zur biplanen linksventrikulären Kineventrikulographie geprüft, ob die intravenöse Injektion von luftgefüllten Albumin-Partikeln (Infoson) als Kontrastmittel die echokardiographische Bestimmung der linksventrikulären enddiastolischen und endsystolischen Volumina, des Schlagvolumens, der Ejektionsfraktion und der regionalen Wandfunktion bei Patienten mit einer reduzierten echokardiographischen Bildqualität verbessert. Bei 30 erwachsenen Patienten mit einer unvollständigen Endokarddarstellung wurden der apikale Vier- und Zweikammerblick aufgezeichnet. Im Vergleich zur biplanen Kineventrikulographie wurden echokardiographisch das enddiastolische, das endsystolische Volumen, das Schlagvolumen, die Ejektionsfraktion sowie die regionale Wandfunktion für Herzzyklen mit und ohne linksventrikuläre Kontrastmitteldarstellung bestimmt. Im Vergleich zur biplanen Kineventrikulographie wurden echokardiographisch das enddiastolische Volumen (167±64 ml, 111±43 ml; p<0,0001), das endsystolische Volumen (77±63 ml, 54±40 ml, p<0,0002), das Schlagvolumen (90±25 ml, 57±17 ml; p<0,0001) und die Ejektionsfraktion (58±16%, 55±14%; p<0,03) zu gering bestimmt. Nach der Gabe des Echokardiographiekontrastmittels stimmte die echokardiographisch gemessene Ejektionsfraktion (58±16%) mit der angiokardiographisch gemessenen Ejektionsfraktion überein. Weiterhin wurden die Korrelationen zwischen der echokardiographischen und angiokardiographischen Bestimmung des linksventrikulären enddiastolischen Volumens (ohne Kontrastmittel r=0,90, SEE=19 ml; mit Kontrastmittel r=0,93, SEE=19 ml), des endsystolischen Volumens (ohne Kontrastmittel r=0,94, SEE=14 ml; mit Kontrastmittel r=0,95, SEE=15 ml), des Schlagvolumens (ohne Kontrastmittel r=0,63, SEE=14 ml; mit Kontrastmittel r=0,67, SEE=14 ml) und der Ejektionsfraktion (ohne Kontrastmittel r=0,84, SEE=8%; mit Kontrastmittel r=0,88, SEE=7%) sowie der regionalen Wandfunktion (p<0,01) und deren Reproduzierbarkeit (p<0,02) durch die Kontrastmittelgabe verbessert. Zusammenfassend bietet die intravenöse Kontrastmittelechokardiographie die Möglichkeit, bei Patienten mit einer unvollständigen linksventrikulären Endokarddarstellung nebenwirkungsarm eine exaktere Beurteilung der linksventrikulären Ejektionsfraktion, der Regionalfunktion einschließlich deren Reproduzierbarkeit und eine geringere Unterschätzung der linksventrikulären Volumina im Vergleich zur biplanen Kineventrikulographie zu erreichen.

Summary In 46 patients with a normal functioning mitral valve prosthesis (15 St. Jude, 19 Medtronic Hall, 12 Hancock) cardiac output was measured by pulsed Doppler echocardiography across the valve prosthesis. Simultaneously cardiac output was determined by thermodilution or pulsed Doppler echocardiography in the left ventricular outflow tract (2.8 l/min–9.5 l/min). The prosthetic valve area was calculated using the pressure half-time method. Cardiac output was calculated by multiplying time-velocity integrals with the mitral valve area. Cardiac output measurements across the mitral prosthesis correlated significantly with thermodilution (r=0.96, SEE=0.400 l/min) and pulsed Doppler echocardiography flow measurements in the left ventricular outflow tract (r=0.82, SEE=0.679 l/min). The mean percent error of the Doppler transmitral flow measurement was 10.8%. Doppler transmitral flow underestimated cardiac output values of more than 6.5 l/min in 6 of 7 patients. Cardiac output measurements across Hancock (SEE=0.473 l/min) and St. Jude prostheses (SEE=0.538 l/min) were more accurate than across Medtronic Hall prostheses (SEE=0.847 l/ min).

150–200 g heavy, Walker-carcinoma bearing, male Sprague-Dawley-rats showed rapid, tumour weight dependent, loss of liver glycogen until complete depletion in tumour groups heavier than 40g/animal. Simultaneously the glycogen mobilization after massive glucagon stimulation, was successivly diminished and finally abolished in different groups with increasing tumor weight. Concomitantly the spontaneous and stimulated activity of liver phosphorylase a was found markedly reduced in advanced tumour cachexia, the extent of stimulation of liver phosphorylase a activity by intracardial injections of epinephrine not being altered. Tumour induced inhibition of glycogen mobilization thus appears to have been excluded.

Summary The purpose of this study was to assess the accuracy and clinical utility of pulsed Doppler echocardiography in determining the regurgitant fraction in patients with pure mitral regurgitation. In 30 unselected consecutive patients with mitral regurgitation and in 20 patients without valvular heart disease pulsed Doppler echocardiography was performed to measure blood flow at the mitral and aortic valve. The regurgitant blood volume was calculated as the difference of the stroke volumes measured at the mitral and aortic valve. The regurgitant fraction was computed as regurgitant blood volume/mitral flow. By cardiac catheterization regurgitant blood volume and regurgitant fraction were obtained from the left ventricular angiographic stroke volume and the stroke volume measured by thermodilution. Five patients were excluded because of technically poor left ventricular angiograms. In 4 patients with mitral regurgitation measurement of the regurgitant blood volume and regurgitant fraction was impossible by Doppler because of poor ultrasound signal quality. In 21 patients with mitral regurgitation the correlations between the invasive and the Doppler measurements were significant (regurgitant blood volume:r=0.89, SEE=20.9 ml; regurgitant fraction:r=0.91, SEE=7.1%). However, the mean percent error of the regurgitant fraction measurement (12.0±11.6%) was smaller than of the regurgitant blood volume measurement (24.9±17.0%). In the control group the regurgitant blood volume ranged between -25.1 ml and 11.6 ml and the regurgitant fraction between -17.7% and 12.4%.

Summary To quantify valve area in mitral stenosis, a modified continuity equation method using continuous wave Doppler and thermodilution measurements was applied. In 14 patients with mitral stenosis and sinus rhythm (age: 49±11 years), transmitral flow velocity was recorded by continuous wave Doppler during right and left heart catheterization. Mitral valve area was calculated by three different methods: 1. According to the continuity equation, stroke volume (thermodilution technique) was divided by the registered time velocity integral of the mitral stenotic jet (continuous wave Doppler). 2. Mitral valve area was calculated by the pressure half-time method. 3. Simultaneous pulmonary capillary wedge and left ventricular pressure measurements were used for determination of mitral valve area according to the Gorlin formula. The mitral valve area determined by application of the continuity equation (y) showed a close correlation to the valve area calculated by the Gorlin equation (x):y=0.73x+0.12, SEE=0.11 cm2,r=0.88,P<0.001. In contrast, the correlation between mitral valve area determined by pressure half-time (y) and the Gorlin formula (x) was not as good:y=0.77x+0.11, SEE=0.26 cm2,r=0.65,P<0.05. Thus, the continuity equation method using combined continuous wave Doppler and thermodilution technique allows a valid determination of mitral valve area. In patients with mitral stenosis and sinus rhythm, this technique is superior to the noninvasive determination of mitral valve area by the conventional pressure half-time method.

Summary To determine the accuracy of echocardiography in assessment of exercise-induced wall motion abnormalities, the results of stress-echocardiography were compared with exercise-cineventriculography.

Article abstract-Lower limb areflexia is generally regarded as an essential criterion for the diagnosis of Friedreich's ataxia (FRDA). We describe a family with a recessive form of early-onset ataxia in which one member had a phenotype typical of FRDA whereas another, with retained tendon reflexes in the lower limbs, did not have electrophysiologic evidence of the usual severe afferent axonal neuropathy of FRDA. In contrast, somatosensory evoked potentials, eyemovement recordings, and MRI of the head and cervical cord provided results highly suggestive of FRDA in both patients. We performed genetic linkage analysis in this family, using markers tightly linked to the FRDA locus on chromosome 9. Inheritance of identical paternal and maternal genotypes by the affected members, but not by their unaffected siblings, provided supporting evidence that this disorder may result from mutation within the FRDA gene or is tightly linked to the investigated loci on chromosome 9.