Catecholaminergic polymorphic ventricular tachycardia. An important diagnosis in children with syncope and normal heart

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 controlled with beta-blockers, but one patient died suddenly in the fourth year of follow-up. Despite the rare occurrence, catecholaminergic polymorphic VT is an important cause of syncope and sudden death in children with no identified heart disease and normal QT interval.

Electrical Storm in Children

“…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 …”

“…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.…”

Infecção recorrente em apresentação tardia de taquicardia ventricular polimórfica catecolaminérgica

“…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 in a child: A case report

“…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).…”

“…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).…”