Associazione Nazionale Medici Cardiologi Ospedalieri



Ventricular arrhythmias in a unexpected cardiac magnetic resonance

Tansella Donatella Padova(Padova) РUniversità degli Studi di Padova | Pittorru Raimondo Padova(Padova) РUniversità degli Studi di Padova | Perazzolo Marra Martina Padova(Padova) РUniversità degli Studi di Padova

Background: Electrical storm in a widespread inflammation of the myocardium represent a very challenge condition and recognizes a broad spectrum of etiology.

Case presentation: A 59-year-old man, without significant prior medical history, came to our attention because, following an emergent hernioplasty procedure, he displayed anterior ST-segment elevation and elevated troponin levels (150.000 ng/L). An echocardiogram revealed an estimated left ventricular ejection fraction (EF) of 39% with anterior hypokinesia. Coronary angiography showed no abnormalities. Cardiac magnetic resonance imaging demonstrated ring-like edema and ring-like late gadolinium enhancement in the left ventricle on T2-weighted and PSIR sequences, with no involvement of the right ventricle. Additionally, there was an increase in extracellular volume. Despite several laboratory tests to investigate infectious or autoimmune causes, the etiology remained inconclusive. To further explore the cause, an endomyocardial biopsy was performed. However, the patient experienced an unstable electrical storm three days later.

Management: Recurrent ventricular tachycardia (VT) was initially treated with esmolol, amiodarone, and procainamide, but with limited success. Due to VT-induced hemodynamic instability, the patient was sedated with propofol and intubated. Endomyocardial biopsy (EMB) confirmed lymphocytic myocarditis. In light of the hemodynamic instability and refractory electrical storm, the patient was promptly cannulated for veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and started on methylprednisolone. Although there was an apparent resolution of VT, the patient experienced new episodes, necessitating percutaneous stellate ganglion block and an upgraded treatment plan that included mycophenolate mofetil. Given the high burden of arrhythmias, severe ventricular dysfunction, and the lack of response to medications, the patient is now awaiting cardiac transplantation.

Conclusion: Transmural edema and ring-like late gadolinium enhancement (LGE) in the early phase are predictors of a poor prognosis. In cases of hemodynamic deterioration and refractory electrical storms, it is crucial to promptly initiate mechanical support. Cardiac transplantation remains the sole treatment option for individuals who do not respond to antiarrhythmic and immunosuppressive drugs.