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The therapeutic dance into the electrical storm of the TANGO2 disease: a case report
Session:
Sessão Speaker’s Corner - Casos clínicos desafiantes… em Cardiologia – 2
Speaker:
Sílvia A. Gomes
Congress:
CPC 2022
Topic:
P. Other
Theme:
37. Miscellanea
Subtheme:
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Session Type:
Sessão de Casos Clínicos
FP Number:
---
Authors:
Sílvia a. Gomes; Sérgio Laranjo; Conceição Trigo; Fátima f. Pinto
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">INTRODUCTION</span> </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">TANGO2-related metabolic encephalopathy and arrhythmia is a rare, newly recognized and likely under-diagnosed condition. First described in 2016, the phenotype is characterized by developmental delay and recurrent metabolic crisis associated with rhabdomyolysis. During this episodes, patients may present QTc prolongation and ventricular arrhythmias. </span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">REPORT</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">We report a 13-year-old, with developmental delay who presented with severe rhabdomyolysis. The initial electrocardiogram (EKG) was normal.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">Due to worsening of rhabdomyolysis, QTc prolongation was identified (QTc 570ms) and oral β-blocker therapy started. A non-sustained ventricular tachycardia developed (Fig.1), inicialy managed with magnesiaum bolus and impregnation dose of lidocaine, followed by perfusion. The echocardiogram did not identify structural or functional alterations. After a short period, frequent polymorphic ventricular extrasystoles (VPB) in short-long-short sequences developed. An arrhythmic storm of VPB induced torsade de Pointes (TdP) was triggered, leading to cardiac arrest. After resuscitation and due to the bradycardia dependent nature of the TdP, a temporary percutaneous pacing lead was placed and esmolol infusion started. Normal plasma levels of potassium, calcium and magnesium were maintained. Despite therapeutic optimization, new episodes of TdP developed, requiring defibrillation. The electrical instability ran in parallel with increasing severity of rhabdomyolysis and systolic ventricular function decline. Genetic testing identified a pathogenic variant in homozygosity in the TANGO2 gene. Stable sinus rhythm was finally achieved with optimization of the caloric intake , stable blood glucose levels and normal serum electrolytes. EKG showed normalization of the QTc interval. </span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">CONCLUSIONS</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">The full TANGO2-related phenotype emerges over time and the prognosis is linked to the appearance of EKG abnormalities. QT interval prolongation can lead to life-threatening ventricular tachycardias.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">The arrhythmia mechanism seems to be secondary to metabolite build up in cardiomyocytes, what can explain the cardiac phenotype during the crises wich subside after their resolution. In this patients, avoiding bradycardia is fundamental, since long QT related TdP seems to be triggered by bradycardia and short-long-short VPB. </span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">During acute metabolic crises, the management of arrhythmias relies on metabolic control. </span></span></span></p>
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