A Case of Occult Myocarditis Causing Fatal Arrhythmia in a Cystic Fibrosis Patient Subjected to Retransplantation

Abstract

Introduction Myocarditis is an inflammatory process that is caused by several factors including infection, systemic disease and/or exposure to drugs and toxins. Its clinical course is extremely variable, ranging from lack of symptoms to sudden cardiac death. Here we report a case of viral myocarditis in a cystic fibrosis (CF) patient subjected to retransplantation for chronic lung allograft dysfunction (CLAD). Case Report The patient was a 42-year-old male who had undergone his first lung transplant 18 years earlier for cystic fibrosis. He was referred for retransplantation for CLAD. The cardiac tests performed at the time of re-listing, as well as those repeated during waiting list time were unremarkable. Intraoperatively, during the first pneumonectomy, the patient developed an episode of ventricular fibrillation (VF), without recurrence after a single intrathoracic defibrillation. Due to the risk of hemodynamic instability, he was put on veno-arterial extracorporeal membrane oxygenation (ECMO). The remainder of the procedure was uneventful, and, after careful monitoring, ECMO cannulae were removed. Soon after skin closure, however, the patient first experienced multiple episodes of VF that required cardioversion, after which he quickly became asystolic. After one hour of advanced cardiac arrest treatments - including norepinephrine, cardiac massage, reopening of the chest and epicardial pacing - the patient was pronounced dead. At autopsy, coronary artery disease was excluded; the only pathological finding was the presence of multifocal acute myocarditis with fibrosis. Tissue molecular analysis detected high viral copies of Epstein Barr virus and Parvovirus B19. Summary In the present case, most probably, a surgery-induced systemic inflammatory response syndrome, with massive cytokine release, was responsible for precipitating an unsuspected preexisting condition of myocarditis. Recipients who undergo retransplantation for CLAD are severely immunocompromised and at risk of getting different forms of infections. Based on this experience, we suggest that cardiac magnetic resonance imaging be added to the preoperative workup of patient candidates for retransplantation for CLAD, so as to be able to detect potentially life-threatening pathologies of the myocardium with higher sensitivity.