0233: Mental stress test for the diagnosis of congenital long QT syndrom: a pilot study

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• 作者：Franç ; ois Huchet ; ⁽¹⁾ ; ^{obl113@hotmail.com" class="auth_mail" title="E-mail the corresponding author} ; Jean-Baptiste Gourraud⁽¹⁾ ; Florence Kyndt⁽²⁾ ; Aurelie Thollet⁽²⁾ ; Jean-Jacques Schott⁽²⁾ ; Richard Redon⁽³⁾ ; Julien Barc⁽³⁾ ; Hervé ; Le Marec⁽³⁾ ; Vincent Probst⁽³⁾
• 刊名：Archives of Cardiovascular Diseases Supplements
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：8
• 期：1
• 页码：66
• 全文大小：48 K

文摘

Many patients and families are referred to our centre for the screening after sudden cardiac death (SCD). Usual tests failed to unmask a specific pathology in 60% of the families. Because of the potential of mental stress to unmask ECG abnormalities, mental stress test (MST) was realized in all patients since summer 2013.

Methods

The protocol was defined before the beginning of the study and usually consisted in mental arithmetic. Control group was defined by 30 healthy volunteers among hospital’s employees who accepted to undergo MST. 12-lead ECG was realized at rest and at stress peak.

Results

ECG from 57 patients (30 affected and 27 non affected family members) coming from 4 different families were analyzed.

Results were compared to 30 healthy volunteers. At least one SCD occurred in each of the four families for a total of 6 SCD. Seven additional patients experienced syncope probably related to arrhythmias (31.4% of symptomatic patients. Transmission of the disease seems followed an autosomal dominant pathway. QTc at rest was longer in affected family members 430±32ms vs non affected family members 402±23ms and healthy volunteers 396±23ms (p<0.001). During MST Δ QTc (msec) was 69±45ms vs 30±27ms vs 32±20ms (<0.001). QTc prolongation was associated with T waves flattening or double-hump morphology (13 vs 1 vs 3; p<0.001). No ventricular arrhythmia was observed during MST. During exercise test there was no prolongation of the QTc interval in the three groups. Genetic analysis have been performed in the probands in each family but remained negative whereas all the genes involved in the LQTS have been tested.

Conclusion

This is the first description of a new type of congenital LQTS characterized by normal QTc at rest but prolongation of the QTc interval and abnormal T waves during mental stress. This new form of LQTS seems related to a severe arrhythmic prognosis and is not related to the gene defects described in this pathology.