Cardiac implantable electronic device malfunction due to twiddler’s syndrome in a patient with bipolar affective disorder

Abstract

© BMJ Publishing Group Limited 2021. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION A 49yearold man presented to our emergency department with dyspnoea and fluid overload. He denied any chest pain. Comorbidities included nondilated ischaemic cardiomyopathy and bipolar affective disorder. An admission ECG demonstrated sinus rhythm with left bundle branch block (QRS duration=126 ms). A chest Xray confirmed pulmonary congestion. Blood tests were unremarkable, with no evidence of infection. Highly sensitive troponin T was mildly elevated at 34 ng/L. He was successfully treated for decompensated heart failure with furosemide and dobutamine, achieving an 11 kg diuresis. Transthoracic echocardiography confirmed severe biventricular dilatation with global left ventricular (LV) systolic impairment and an LV ejection fraction of 16%. He was referred for assessment at our regional transplant centre and listed for a biventricular pacemaker following discussion at our multidisciplinary team (MDT) meeting. Two weeks later, he returned for an uneventful elective implant of a cardiac resynchronisation therapy with defibrillator (CRTD) device. Nine days on, he again presented to our emergency department with dyspnoea, testing positive for COVID19 pneumonitis. On day 4 of this admission, he required transfer to the intensive care department for inotropic support. Unfortunately, this experience caused a significant deterioration in his mental health with paranoid thoughts and concerning behaviours. Following a thorough psychiatric assessment, he was discharged to a psychiatric hospital on day 10. Six weeks later, he was repatriated to our centre with swelling at the site of CRTD implant, with concerns raised regarding a possible wound infection. However, C reactive protein was just 4 mg/L with a white cell count of 6.2×10/L. A chest Xray demonstrated retraction of the leads back to the generator pocket, with the defibrillator coil clearly visible in the left subclavian vein (figure 1). He held a delusional belief that this was caused following an assault by hospital security staff, although there was no evidence to support this claim. He vehemently denied manipulating the device. The patient was taken to the cardiac catheterisation laboratory whereupon the reopening of the pocket, all three leads of the CRTD appeared heavily entangled with no evidence of infection (figure 2). The device was extracted without complication and the MDT consensus was to avoid reimplantation. Twiddler’s syndrome was first described 1968, it is a rare but serious cause of cardiac implantable electronic device malfunction typically occurring within a year of implant widely reported in elderly patients with cognitive impairment but only recently observed in a patient with schizophrenia. Figure 1 Chest Xray showing entanglement of all three CRTD leads in the pulse generator pocket with retraction of the defibrillator coil visible in the left subclavian vein. CRTD, cardiac resynchronisation therapy with defibrillator.