I Think a Niece of Mine was Referred to a Neurologist: Difference between revisions

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I Think a Niece of Mine was Referred to a Neurologist (view source)

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[[Puzzle 2004_10_469 - Answer|Answer]]
[[Puzzle 2004_10_469 - Answer|Answer]]
[[Image:Puzzle_2004_6_302_fig2.jpg|Figure 2. Post-pause T-wave accentuation. The relative pause (RR interval 968 msec) caused by an extrasystole is followed by an AV-nodal escape beat (the P wave and the QRS complex fuse) with a markedly prolonged ST segment (QT interval exceeds 500 msec).|thumb]]
[[Image:Puzzle_2004_6_302_fig3.jpg|Figure 3. ECG recording from a dual-chamber ICD. The upper lead is the local activity in the atrium, the lower lead of the ventricle. The initiation of the arrhythmia is not shown. It is clear that the heart rate is considerably higher in the ventricle. A 21.7 J shock terminates the arrhythmia.|thumb]]
The ECG shows sinus arrhythmia with a normal QRS axis. PQ interval and QRS width are normal. Repolarisation is unstable (see lead V2) and the QT interval is slightly prolonged. In the first beats in lead II the QT interval is 460 msec, corrected for heart rate
469 msec (third ST segment). ST-T morphology is abnormal (very low amplitude in the lateral leads, ‘sloppy’ T waves in the inferior leads and biphasic T waves in V2). Altogether this ECG carries a high suspicion of the patient being a carrier of the mutant
KCNH2 allele. Further cardiological evaluation is certainly warranted and should consist of 24-hour Holter monitoring and exercise testing. Figure 2 demonstrates a nice example of post-pause T-wave deformation during 24-hour monitoring, which is typically seen in LQT patients. DNA analysis revealed the E698X mutation in the patient. Treatment with β-blockade was started and the valproic
acid was discontinued. Two years later she again had a typical syncope with apparently no specific trigger. Because the β-blocker had been used faithfully, an ICD was implanted. A few months later she suffered another syncope. Interrogation of the ICD revealed a rapid ventricular arrhythmia which was terminated by a successful defibrillation shock of 21.7 J (figure 3).
In conclusion, in families with a long-QT syndrome any potentially related symptoms and/or minor ECG abnormalities warrant further investigation. In this patient, at a considerable distance from the index LQT patient, the diagnosis was suspected on the basis of
history and discrete ECG abnormalities (figure 1). DNA analysis proved the familial genetic abnormality; the cause of the recurrent syncope was only proved after ICD implantation.
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