Reel Syndrome: An Atypical Cause for Inappropriate Shocks in a Patient with Automated Implantable Cardioverter Defibrillator (AICD)

A 71-year-old woman, with the past medical history of heart failure with reduced ejection fraction (EF) and automated implantable cardioverter defibrillator (AICD) placement (for low EF 5-10%) in 2015, presented in February 2017 with the complaint of AICD shocks following an episode of vomiting. She denied any chest pain, abdominal pain, shortness of breath, palpitation, or dizziness. Electrocardiogram (EKG) on admission showed ectopic atrial rhythm with premature ventricular contractions in bigeminies, anterior fascicular block, and left axis deviation. On examination of the cardiovascular system, there was a normal S1 heart sound with a loud A2. There was no jugular venous distention on the neck or pitting edema on the legs. Laboratory studies showed no elevation of cardiac enzymes. Evaluation with chest x-ray showed the right ventricular lead had migrated to the right atrium and the defibrillator generator was flipped with leads coiled around it in transverse axis. AICD interrogation was performed which revealed inappropriate shocks were due to atrial fibrillation with rapid ventricular rate and loss of capture of the right ventricular lead. The diagnosis of Reel syndrome was made, and an electrophysiologist was consulted for replacement of the AICD. Reel syndrome is a variant of Twiddler’s syndrome, which is a rare complication of pacemaker implantation. It manifests with the rotation of generator on transverse axis with leads coiling around it. Twiddler’s syndrome, on the other hand, is the rotation of the generator on its long axis, which causes damage to the leads by twisting. Reel syndrome is usually observed within months from the placement of the generator compared to Twiddler, which takes years to occur. Twiddler and Reel's syndromes have similar contributing factors, such as female gender, obesity, large pocket, old age, dementia, and deep brain stimulation. Our patient only had two risk factors, namely, the sex and age. We propose that every patient with a pacemaker malfunction and AICD shocks should have a posterior-anterior (PA) chest x-ray and a lateral chest x-ray in addition to AICD interrogation. Twiddler’s syndrome is effortlessly observed because of the twisting of dual leads compared to Reel syndrome, which is not straightforward because of the lack tortuosity of the leads.