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An unusual cause of electrical storm in a young woman
Session:
Sessão de Casos Clínicos - I
Speaker:
Carlos Xavier Resende
Congress:
CPC 2019
Topic:
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Theme:
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Subtheme:
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Session Type:
Sessão de Casos Clínicos
FP Number:
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Authors:
Carlos Xavier Resende; Gonçalo Pestana; Pedro Grilo Diogo; Sandra Amorim; Luís Adão; Maria Júlia Maciel Barbosa
Abstract
<p>A 35-year-old woman with a history of neurocardiogenic syncope, without cardiovascular risk factors or family history of sudden death, was admitted to our emergency department (ED) for recurrent syncope. Episodes were preceded by sudden onset palpitations, some at rest, without apparent situational cause. She had a recently electrocardiogram (EKG), transthoracic echocardiogram (TTE) and exercise EKC performed which were normal, at admission to the ED she was undergoing 24h-Holter monitoring.</p> <p>Multiples episodes of non-sustained polymorphic ventricular tachycardia(VT) were recorded during monitoring in the ED, all of them preceded by premature ventricular contractions(PVC) with a short coupling interval. Iatrogenic causes were excluded, TTE and EKG were normal (namely normal QT interval and no Brugada pattern) and emergent coronary angiography showed no coronary disease.</p> <p>Initial treatment with amiodarone and magnesium sulfate were ineffective and the patient had three episodes of promptly shocked ventricular fibrillation (FV). In the setting of electrical storm the patient was sedated and ventilated; given the suspected diagnosis of short-coupled variant of torsades de pointes, intravenous verapamil was started with successful rhythm stabilization.</p> <p>She was extubated at 24h and intravenous verapamil switched for oral administration, prompting three additional FV episodes and, again, stabilization after restarting intravenous dose. A later attempt was successful in substituting oral for intravenous verapamil, with dose titration up to 120mg tid, without arrhythmia recurrence during hospital stay. A cardiac magnetic resonance was also performed, showing a structurally normal heart.</p> <p>Review of the 24h-Holter monitor of the admission day confirmed multiple episodes of polymorphic VT (the longest episode with 2.5 minutes), all starting with a short-coupled PVC.</p> <p>A subcutaneous cardioverter-defibrillator (ICD) was implanted without complications. During hospital stay the patient had one situational syncope, without any arrhythmias on telemetry, with no other relevant occurrences.</p> <p>Four months after hospital discharge the patient remained asymptomatic, free of ICD shocks under verapamil 120mg tid.</p> <p>Idiopathic ventricular arrhythmias occur in structurally normal hearts and include a heterogeneous group of patients with mean age less than patients with ventricular arrhythmias secondary to underlying heart disease. Short-coupled variant of torsade the point is a rare condition triggered by PVC not followed by a compensatory pause and a short coupling interval (<300ms), similar to an R-on-T phenomenon. Verapamil is the drug of choice for rhythm stabilization, but it is not effective in reducing the risk of sudden death. A high clinical suspicion is essential for this diagnosis as it can be lifesaving in such young patients presenting with electric storm.</p>
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