EXPERIMENTAL STUDIES
Case of the month by the EHRA Education committee: exercise-related arrhythmias
1 Department of Cardiology, Thoraxcenter, University Medical Center Groningen, University of Groningen, Hanzeplein 1, PO Box 30.001, 9700 RB Groningen, The Netherlands; 2 Interuniversity Cardiology Institute Netherlands, Utrecht, The Netherlands; 3 Institute of Cardiology, University of Bologna, Bologna, Italy; 4 Department of Cardiology, Royal Brompton Hospital, London, UK; 5 Department of Cardiology, University Hospital Gasthuisberg, University of Leuven, Leuven, Belgium; 6 Department di Biotecnologie e Bioscienze, University of Milano-Bicocca, Milano, Italy; 7 Division of Cardiology, Helsinki University Central Hospital, Helsinki, Finland; 8 Eskisehir Osmangazi University, Eskisehir, Turkey; 9 Department of Cardiology, University Hospital Uppsala, Uppsala, Sweden
* Corresponding author. Tel: + 31 50 361 2355; fax: + 31 50 361 4391. E-mail address: i.c.van.gelder{at}thorax.umcg.nl
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Abstract |
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This case describes two nephews with exercise related arrhythmias.
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Answers to the case presented at page 235: |
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Figure 1 shows an abnormal 12-lead electrocardiogram. A downsloping ST-segment with negative T waves in the inferolateral leads and saddle back-shaped ST-segment elevations in leads V1 and V2 are present. No epsilon waves nor negative T waves in the precordial leads are present. There is no prolonged QT. The 12-lead electrocardiogram in Figure 4 is completely different. It shows a low voltage electrocardiogram with aspecific STT abnormalities. Also, neither epsilon waves nor inverted T waves in the right precordial leads are present. From the electrocardiograms, it seems unlikely that the arrhythmias are related to one disease.
An ajmaline test was performed in the first patient because of the saddle back-shaped ST-segment elevations in leads V1 and V2. The outcome was negative.
During exercise, the first patient developed a tachycardia both during exercise and during 24 h Holter monitoring suggestive for a ventricular tachycardia with a left bundle branch block configuration and an intermediate axis. The second patient also develops a ventricular tachycardia during exercise (left bundle branch block configuration, left axis, atrioventricular dissociation, and negative concordancy).
The therapy should at least consist of beta-blockade as both arrhythmias are clearly exercise-related.
The second patient also received an internal cardioverter defibrillator because he suffered from a serious collapse during exercise suggestive for a ventricular tachycardia. He was also treated with an angiotensin-converting enzyme inhibitor because of his impaired left ventricular function. As the first patient had palpitations accompanied by dizziness that were not completely abolished by beta-blocker therapy, he also received a defibrillator.
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Discussion |
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This case shows the presence of exercise-induced ventricular arrhythmias in two nephews in the presence of abnormal but completely different 12-lead electrocardiograms. Left ventricular function was impaired in only one of them. Although the phenotype is variable, the same genetic defect may well underlie this cardiac disease. Patients were sent to our genetic department and at present no mutations have been detected. Screening of family members of these nephews was advised and has recently been started.
An arrhythmogenic right ventricular cardiomyopathy may be the cause because of the occurrence of ventricular tachycardias with a left bundle branch block configuration. However, the present patients do not fulfil the criteria.1
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Reference |
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[1] Sen-Chowdhry S, Syrris P, Ward D, Asimaki A, Sevdalis E, McKenna WJ. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insight into patterns of disease expression. Circulation (2007) 115:1710–1720.
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