Hidden under the Surface: A Rare Cause of Repeated Syncope in a Patient with Recent Pacemaker Implantation

A 66-year-old woman received a pacemaker implantation because of syncope with documented sinus arrest and junctional bradycardia. Three weeks later the pacemaker analysis revealed episodes of nonsustained ventricular tachycardia. Coronary angiography and invasive coronary assessment showed diffuse moderate stenosis but no significant ischemia. Three months later she experienced a new syncope and the pacemaker analysis showed runs of nonsustained ventricular tachycardia at the time of syncope. The combination of brady- and tachyarrhythmias raised concern for cardiac sarcoidosis. 18F-fluorodeoxyglucose positron emission tomography (PET) scan showed increased FDG uptake in the basal segments compatible with inflammatory disease. Cardiac magnetic resonance imaging showed late gadolinium enhancement in the same region of the PET-avid lesions. Diagnostic electrophysiologic study could induce VT. The diagnosis of cardiac sarcoidosis was made, for which high dose corticosteroids were prescribed and an upgrade to a dual chamber implantable cardioverter defibrillator was performed. Because of the localization of the lesions, an endomyocardial biopsy was not performed. All the lesions regressed completely on PET-scan after treatment with high dose corticosteroids.

Mode of death in Shapiro syndrome: a case report

Background Shapiro syndrome is extremely rare and is characterized by the triad of spontaneous periodic hypothermia, hyperhidrosis and agenesis of the corpus callosum, resulting in neurological and psychological disorders. The exact mechanism of this syndrome is unknown and treatment consists of controlling the periodic attacks. This case report describes a case of Shapiro syndrome presenting with ventricular fibrillation (VF) who was treated with dual chamber implantable cardioverter defibrillator (ICD) therapy. Case summary A 45-year-old man, suffering from Shapiro syndrome with frequent hypothermic attacks, was admitted to the emergency department with an out of hospital cardiac arrest caused by VF due to hypothermia. To prevent cardiac death during future hypothermic attacks with VF, the patient was treated with a dual chamber ICD. Within 1 month after ICD implantation the patient had two events of ventricular tachycardia/VF during hypothermia, which were both successfully terminated by an ICD shock. One year after ICD implantation the patient suffered from an uncontrolled urinary tract infection and the patient passed away. Post-mortem interrogation of the ICD did not reveal further episodes of VF and showed a higher supraventricular heartrate in the last days before his death, probably due to a sinus tachycardia driven by the infection. It was concluded that the most likely cause of death was an uncontrolled sepsis. Discussion The current case showed that ICD therapy can be successful in treating VF episodes in patients with unexpected periods of hypothermia.