Sudden Arrhythmic Death Syndrome : Diagnostic Yield of Comprehensive Clinical Evaluation of Pediatric First-Degree Relatives
Articolo
Data di Pubblicazione:
2014
Citazione:
Sudden Arrhythmic Death Syndrome : Diagnostic Yield of Comprehensive Clinical Evaluation of Pediatric First-Degree Relatives / V. Giudici, A. Spanaki, J. Hendry, S. Mead Regan, E. Field, G.V. Zuccotti, D. Abrams, M. Lowe, J.P. Kaski. - In: PACING AND CLINICAL ELECTROPHYSIOLOGY. - ISSN 0147-8389. - 37:12(2014 Dec 01), pp. 1681-1685. [10.1111/pace.12479]
Abstract:
Aims: Sudden arrhythmic death syndrome (SADS) is most often caused by heritable cardiac diseases. Studies in adults have identified evidence of inherited cardiovascular diseases in up to 53% of families, but data on the prevalence of familial disease in children are scarce. The aim of this study was to evaluate the yield of clinical screening in pediatric first-degree relatives of victims of SADS using a systematic and comprehensive protocol. Methods: Patients referred for family screening after sudden cardiac death (SCD) of a family member were, retrospectively, enrolled into the study. Systematic evaluation of the children included clinical examination, family history, electrocardiogram (ECG), echocardiogram, 24-hour tape, and signal-averaged ECG. Older patients also underwent exercise testing, cardiac magnetic resonance imaging, and ajmaline provocation testing. Results: A total of 90 children from 52 consecutive families were included in the study. An inherited cardiac disease was identified in seven first-degree children from seven (13.5%) families (five children were diagnosed with Brugada syndrome, one with long QT syndrome, and one with catecholaminergic polymorphic ventricular tachycardia). Two further children had late potentials on signal-averaged ECGs with no other abnormalities. Conclusions: These data show a high prevalence of inherited heart disease in pediatric first-degree relatives of SADS victims. The results highlight the importance of a systematic, comprehensive approach and ongoing screening of pediatric family members.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
arrhythmia ; Brugada syndrome ; long QT syndrome ; pediatric ; screening ; sudden death
Elenco autori:
V. Giudici, A. Spanaki, J. Hendry, S. Mead Regan, E. Field, G.V. Zuccotti, D. Abrams, M. Lowe, J.P. Kaski
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