What is Twiddler syndrome?

   Displacement of pacemaker leads due to twisting  of the box on part of the patient is called

   Twiddler’s syndrome, first described in 1968  (Nicholson et al., 2003).Twiddler syndrome

    that causes device malfunction is a rare complication in patients with an implantable      cardioverter    defibrillators (ICD) (Fahraeus & Höijer, 2003). Twisting of the pulse generator

    within the device pocket may cause the dislocation of the lead, diaphragmatic stimulation,

    and loss of capture (Figure 7). The prevelance  of this syndrome is 0.07% (Gungor et al.,

    2009). Classically, Twiddler syndrome occurs in obese women with loose, fatty

    subcutaneous tissue and is characterized by rotate on of pulse generator on its long axis with       subsequent coiling of pacemaker leads (Bhatia et al., 2007). Other risk factors are mental  disorders,     female sex, and the small size of the implanted generator with a large pocket  (Cardall et   al., 1999). This disorder may induce lead dislodgement or lead fracture and cause  life-threatening  symptoms in case of pacemaker dependency. When the pulse generator is  rotated    along the transverse axis it is referred by us as the Reel syndrome, a variant of  Twiddler syndrome (Camero-Varo et al., 1990). 

In Twiddler syndrome, electrocardiography shows failure of capture and the chest

radiography reveals the dislodged and twisted  leads (Pereira et al., 1999). Hypoperfusion  symptoms such as fatigue, tiredness, confusion, presyncope, and syncope may be obse

rved  (Cardall et al., 1999). If the problem has occured because of pacemaker migration or poorly  fashioned pacemaker pocket, the pocket should be revised. As an inappropriate ICD

therapy may be proarrhythmic and may lead to sudden cardiac death, Twiddler syndrome

should be considered in patients with ICD  who had resistant ventricular arrhythmias and

abdominal pulsation. To avoid this life-threatening complication of ICD implantation, we

should take care to limit the pocket size, suture the device to the fascia, and instruct the

patients not to manipulate their device pockets. 

Pacemaker Twiddler syndrome. Postero-anterior and lateral chest X-ray showing

displacement of both leads, especially the ventricle one, retracted and floating in the right

atrium (arrows).