SPC365

A perfect match for an arrhythmic storm

Session: Casos Clínicos: Arritmias e Dispositivos Cardíacos
Speaker: João Fernandes Pedro

Congress: CPC 2024
Topic: C. Arrhythmias and Device Therapy
Theme: 04. Arrhythmias, General
Subtheme: 04.4 Arrhythmias, General – Treatment
Session Type: Sessão de Casos Clínicos
FP Number: ---
Authors: João Fernandes Pedro; Doroteia Silva; Ana Marques; Ana Gaspar; Laura Carvalho; Ana Rita Silva; João Ananias; Maria Serrano; Renato Costa Reis; Ana Rita Rodrigues; Carlos Candeias; João Miguel Ribeiro

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="background-color:white"><span style="font-family:Calibri,sans-serif"><span style="color:#242424">Long QT Syndrome (LQTS) is a disorder affecting myocardial repolarization, potentially leading to life-threatening cardiac arrhythmias, namely Torsades de Pointes. In recent years, the role of immunity and inflammation, especially cytokine-mediated, has been recognised as a trigger of</span></span>&nbsp;<span style="font-family:Calibri,sans-serif"><span style="color:#242424">electrical instability in patients genetically predisposed to arrhythmias.</span></span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="background-color:white"><span style="font-family:Calibri,sans-serif"><span style="color:#242424">A 34-year-old male with known congenital LQTS, previous episodes of syncope and a family history of sudden cardiac death in a first degree relative, was brought to the Emergency Department in</span></span>&nbsp;<span style="font-family:Calibri,sans-serif"><span style="color:#242424">cardiac arrest, on advanced life support. First evaluated rhythm was ventricular fibrillation and the estimated time of cardiac arrest was 69 minutes (No flow 8&rsquo;, Low flow 61&rsquo;). He was previously treated with propranolol and refused genetic testing and ICD implantation on primary prevention. At hospital admission, eCPR was performed and VenoArterial (VA) -ECMO was implanted. The patient was then admitted in the ICU. Immediately post cannulation, electrocardiogram documented sinus rhythm and long corrected QT and echocardiogram showed severe bi-ventricular dysfunction (estimated ejection fraction of 10%, TAPSE 10 mm), diffuse hypocontractility and a slightly increased myocardial thickness. Myocardial stunning after prolonged cardiorespiratory arrest due to LQTS was the first diagnostic hypothesis.</span></span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="background-color:white"><span style="font-family:Calibri,sans-serif"><span style="color:#242424">The patient evolved in&nbsp;cardiogenic shock with multi-organ failure. After 72 hours, however, severe left ventricular&nbsp;dysfunction and myocardial thickening persisted. Also, a&nbsp;marked elevation of troponin levels was noted. According to these findings, the hypothesis of acute myocarditis was suspected and an endomyocardial biopsy was performed, enabling the diagnosis of acute lymphocytic myocarditis, with myocardial PCR analysis (cardiotropic virus panel) being negative. Autoimmunity serological analysis was also negative. There was&nbsp;no recurrence of arrhythmias throughout the entire ICU stay.</span></span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="background-color:white"><span style="font-family:Calibri,sans-serif"><span style="color:#242424">Gradual improvement of biventricular function was noted, allowing decannulation from VA-ECMO at day 8, without any other therapeutical interventions. Cardiac magnetic resonance was later performed and findings were also consistent with the diagnosis of acute myocarditis.</span></span>&nbsp;<span style="font-family:Calibri,sans-serif"><span style="color:#242424">A comatose state persisted for several days (Glasgow come scale score 3) but the multimodal neuroprognostication protocol did&nbsp;not allow us to conclude the prognosis. Several complications occurred, namely ventilator associated pneumonia with severe primary ARDS, leading to new cannulation on Veno-venous ECMO&nbsp;with further clinical improvement. Slowly and over time, the patient recovered complete consciousness, allowing for an intensive rehabilitation program with achievement of&nbsp;complete functional autonomy. The patient was then&nbsp;discharged home at day 53. Prior to discharge, genetic testing was performed and an ICD was implanted (with his consent).</span></span></span></span></span></span></p>