SIGNIFICANCE OF NONINVASIVE DIAGNOSTIC-TECHNIQUES IN PATIENTS WITH LONG QT-SYNDROME

The idiopathic long QT syndrome (LQTS) is an infrequently occurring disorder. Affected patients may have electrocardiographic alterations and are prone to syncope and sudden arrhythmogenic cardiac death. Adequate therapy may improve the prognosis of affected patients significantly. Therefore the early and precise diagnosis of LQTS has major prognostic impact. This study reports the diagnostic significance of standard electrocardiographic techniques and autonomic maneuvers in 14 patients with LQTS. The findings are compared with those of 14 healthy age-matched control persons. QTc duration was significantly longer in patients with LQTS during standard 12-lead electrocardiography (489 +/- 56 vs 412 +/- 30 ms, p <0.005), exercise stress testing (490 +/- 38 vs 409 +/- 18 ms, p <0.001), cold testing (512 +/- 45 vs 407 +/- 19 ms, p <0.001), Valsalva maneuver (497 +/- 49 vs 407 +/- 19 ms, p <0.001), minimal heart rate during 24-hours of ambulatory electrocardiographic recording (482 +/- 69 vs 402 +/- 22 ms, p <0.01) and maximal heart rate during Holter monitoring (460 +/- 47 vs 411 +/- 27 ms, p <0.005). Four of 14 patients with LQTS had pathologic findings during ambulatory electrocardiographic monitoring (2 patients with short episodes of torsades de pointes tachyarrhythmia, 1 patient with intermittent sinoatrial block, and 1 patient with intermittent TU-wave alterations), whereas all control persons had normal ambulatory electrocardiographic recordings (p <0.05). Thus, noninvasive standard electrocardiographic techniques in combination with autonomic maneuvers may contribute significant information for a precise diagnosis in patients with suspected LQTS. The greatest diagnostic impact lies in the significant prolongation of QTc duration at rest and during provocation tests.