Diagnostische Wertigkeit von Standard-EKG-Methoden, Kälte-Druck-Test und Valsalva-Manöver beim idiopathischen QT-Syndrom

: The idiopathic Long QT Syndrome is an infrequently occurring disorder. Affected patients usually show ECG alterations and are prone to syncope and sudden arrhythmic cardiac death. Adequate therapy with beta-blocking drugs may significantly improve the prognosis of affected patients. The early and precise diagnosis of the disorder therefore has major prognostic impact. This paper reports the diagnostic significance of standard ECG, exercise stress testing, ambulatory ECG monitoring, cold pressor test and Valsalva maneuver in 14 patients with Long QT Syndrome. The results were compared with those obtained in 14 healthy age matched volunteers. QTc duration was significantly longer in patients with Long QT Syndrome during standard 12-lead ECG at rest (489 +/- 55 ms versus 412 +/- 25 ms, p < 0.005), exercise stress test (490 +/- 39 ms vs. 409 +/- 18 ms, p < 0.001), cold pressor test (512 +/- 45 ms vs. 407 +/- 19 ms, p < 0.001), Valsalva maneuver (497 +/- 49 vs. 407 +/- 18 ms, p < 0.001), minimal heart rate during 24-h of ambulatory ECG monitoring (482 +/- 69 ms vs. 402 +/- 22 ms, p < 0.01) and maximal heart rate during Holter monitoring (460 +/- 47 ms vs. 411 +/- 27 ms, p < 0.005). Four of 14 patients with Long QT Syndrome had pathological findings during ambulatory ECG monitoring (two patients showed short episodes of asymptomatic torsade de pointes tachycardia, one patient had an intermittent SA-Block, and one patient showed T-wave alternans). All healthy volunteers had normal ambulatory ECG recordings (p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)