Abstract:Syncope in children is primarily related to vagal hyperreactivity, but ventricular tachycardia (VT) way rarely be seen. Catecholaminergic polymorphic VT is a rare entity that can occur in children without heart disease and with a normal QT interval, which may cause syncope and sudden cardiac death. In this report, we describe the clinical features, treatment, and clinical follow-up of three children with syncope associated with physical effort or emotion and catecholaminergic polymorphic VT. Symptoms were cont… Show more
“…ICD implantation accounts for approximately 1–3.6% of all ICD implants, and this long‐term management strategy has a very dominant role in children presenting with ES and ICD indications for primary electrical disease, such as long QT syndrome and CPVT in various pediatric reports …”
Section: Discussionmentioning
confidence: 99%
“…24 ICD implantation accounts for approximately 1-3.6% of all ICD implants, 25,34 and this longterm management strategy has a very dominant role in children presenting with ES and ICD indications for primary electrical disease, such as long QT syndrome and CPVT in various pediatric reports. 15,20,21,23,[54][55][56][57][58] Mortality rates of 4-5% in large pediatric series are lower than those reported in adults, 20,21,24,59 but in larger series, 20-25% inappropriate shocks were delivered for sinus or supraventricular tachycardia, atrial flutter, T-wave oversensing, and lead fractures. 20,21,24,32,33,59,60 Short, self-limiting arrhythmias may contribute to inappropriate shocks seen in children.…”
Electrical storm (ES) presents a difficult management problem that has predominantly been described in adults and there are limited published data relating to children. We set out to characterize ES in children to assist management based on published literature and own institutional experience. We retrospectively analyzed the records of children presenting with ES to our institution between July 2001 and July 2011 and conducted a systematic literature review. Four children were identified (median age: 5.7 years, range: 3.3-9.6 years, one male). Each ES was of different character and different management strategies were used. All patients were alive at a median follow-up of 5.7 years and all had received implantable cardioverter-defibrillators. Two patients were felt to have catecholaminergic polymorphic ventricular tachycardia, one possible long QT syndrome and one the "short-coupled" variant of torsades de pointes. At least three of our four patients had possible iatrogenic contribution to their ES. Forty-seven cases of ES in children with variable management strategies were identified from the published literature. ES is a rare medical emergency in children with multiple etiologies requiring individualized management.
“…ICD implantation accounts for approximately 1–3.6% of all ICD implants, and this long‐term management strategy has a very dominant role in children presenting with ES and ICD indications for primary electrical disease, such as long QT syndrome and CPVT in various pediatric reports …”
Section: Discussionmentioning
confidence: 99%
“…24 ICD implantation accounts for approximately 1-3.6% of all ICD implants, 25,34 and this longterm management strategy has a very dominant role in children presenting with ES and ICD indications for primary electrical disease, such as long QT syndrome and CPVT in various pediatric reports. 15,20,21,23,[54][55][56][57][58] Mortality rates of 4-5% in large pediatric series are lower than those reported in adults, 20,21,24,59 but in larger series, 20-25% inappropriate shocks were delivered for sinus or supraventricular tachycardia, atrial flutter, T-wave oversensing, and lead fractures. 20,21,24,32,33,59,60 Short, self-limiting arrhythmias may contribute to inappropriate shocks seen in children.…”
Electrical storm (ES) presents a difficult management problem that has predominantly been described in adults and there are limited published data relating to children. We set out to characterize ES in children to assist management based on published literature and own institutional experience. We retrospectively analyzed the records of children presenting with ES to our institution between July 2001 and July 2011 and conducted a systematic literature review. Four children were identified (median age: 5.7 years, range: 3.3-9.6 years, one male). Each ES was of different character and different management strategies were used. All patients were alive at a median follow-up of 5.7 years and all had received implantable cardioverter-defibrillators. Two patients were felt to have catecholaminergic polymorphic ventricular tachycardia, one possible long QT syndrome and one the "short-coupled" variant of torsades de pointes. At least three of our four patients had possible iatrogenic contribution to their ES. Forty-seven cases of ES in children with variable management strategies were identified from the published literature. ES is a rare medical emergency in children with multiple etiologies requiring individualized management.
“…CPVT is a potentially lethal inherited arrhythmogenic disorder, characterized by adrenergically-mediated ventricular arrhythmias, typically bidirectional or pVT, manifested especially in children/teenagers 1 . We present the case of a 45-year-old patient diagnosed with CPVT following its first manifestation.…”
“…Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare entity which may cause syncope and sudden cardiac death (2). Affected patients typically present with life‐threatening ventricular tachycardia (VT) or ventricular fibrillation occurring during emotional or physical stress, with syncope often being the first manifestation of the disease (3).…”
Section: Discussionmentioning
confidence: 99%
“…In the absence of structural heart defects, five major arrhythmogenic disorders manifesting as polymorphic ventricular tachycardia (PVT) or ventricular fibrillation have been described as possible causes of sudden death in childhood: (1) the long QT‐syndrome, (2) right bundlebranch block and persistent ST elevations (Brugada syndrome), (3) the short‐coupled variant of torsade de pointes, (4) idiopathic ventricular fibrillation with normal electrocardiogram (ECG) and (5) polymorphic ventricular tachycardia induced by catecholamines (CPVT) (1).…”
Due to its potential lethal outcome, exclusion or confirmation of catecholaminergic polymorphic ventricular tachycardia in children with physical and emotional syncope is mandatory. We report a case of catecholaminergic polymorphic ventricular tachycardia in a three-year-old child only diagnosed by genetic mapping.
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