Twiddler's syndrome

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Twiddler's syndrome
Twiddler's syndrome.jpg
Chest radiograph showing twiddler's syndrome. The leads of the automated external defibrillator are wrapped around the device and can't be seen in the ventricle.
Specialty Cardiology

Twiddler's syndrome is a malfunction of a pacemaker due to manipulation of the device and the consequent dislodging of the leads from their intended location. As the leads move, they stop pacing the heart and can cause strange symptoms such as phrenic nerve stimulation resulting in abdominal pulsing or brachial plexus stimulation resulting in rhythmic arm twitching. [1] Twiddler's syndrome in patients with an implanted defibrilator may lead to inadequate, painful defibrillation-shocks. [2]

Contents

Signs and symptoms

Twiddler's syndrome presents with a range of symptoms, contingent on the extent of entanglement, the electrode's subsequent retraction, and the final location of the dislodged lead. [3] Leads that become more dislodged up can activate the ipsilateral phrenic nerves, which can result in hiccups, involuntary breathing spasms, or diaphragmatic contractions. [1] [4] The brachial plexus is stimulated by additional coiling and withdrawal of the lead, which causes regular arm twitches. [5]

Causes

Patient "twiddling," or manipulation, of the pulse generator within its skin pocket results in the device's painless dislodgment; lead dislodgement follows, eventually leading to pacemaker malfunction. [1] There have also been reports of variations of this phenomenon resulting in the failure of implanted cardioverter-defibrillators and cardiac resynchronization therapy, which can be fatal. [6]

Risk factors

The condition's risk factors include being a woman, being obese, belonging to an older age group, having cognitive impairments, and having an implanted device that is smaller than its pocket. [7] [4] [8] A related increase in subcutaneous tissue laxity, especially in older patients, makes it easier for the device to become dislodged. [6] [7] Furthermore, the newer devices' smaller sizes make it easy for them to rotate inside the skin pocket. [3]

Diagnosis

Given its speed and ability to provide a clear image of the lead coiling and device rotation, the chest X-ray is the most straightforward and important diagnostic tool for Twiddler's syndrome. [3]

Treatment

Repositioning the pulse generator, implanting a new lead, and uncoiling the lead are among the treatments for diagnosed cases. [4] [8] [5]

Related Research Articles

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An artificial cardiac pacemaker is a medical device, nowadays always implanted, that generates electrical pulses delivered by electrodes to one or more of the chambers of the heart, the upper atria or lower ventricles. Each pulse causes the targeted chamber(s) to contract and pump blood, thus regulating the function of the electrical conduction system of the heart.

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Jervell and Lange-Nielsen syndrome (JLNS) is a rare type of long QT syndrome associated with severe, bilateral sensorineural hearing loss. Those with JLNS are at risk of abnormal heart rhythms called arrhythmias, which can lead to fainting, seizures, or sudden death. JLNS, like other forms of long QT syndrome, causes the cardiac muscle to take longer than usual to recharge between beats. It is caused by genetic variants responsible for producing ion channels that carry transport potassium out of cells. The condition is usually diagnosed using an electrocardiogram, but genetic testing can also be used. Treatment includes lifestyle measures, beta blockers, and implantation of a defibrillator in some cases. It was first described by Anton Jervell and Fred Lange-Nielsen in 1957.

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References

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  3. 1 2 3 Salahuddin, Mohammad; Cader, Fathima Aaysha; Nasrin, Sahela; Chowdhury, Mashhud Zia (January 20, 2016). "The pacemaker-twiddler's syndrome: an infrequent cause of pacemaker failure". BMC Research Notes. Springer Science and Business Media LLC. 9 (1). doi: 10.1186/s13104-015-1818-0 . ISSN   1756-0500.
  4. 1 2 3 Pande, Arindam; Kahali, Dhiman; Mandal, Saroj (2012). "A rare case of very early pacemaker Twiddler′s syndrome". Heart Views. Medknow. 13 (3): 114. doi: 10.4103/1995-705x.102157 . ISSN   1995-705X. PMID   23181182.
  5. 1 2 Nicholson, William J.; Tuohy, Kathryn A.; Tilkemeier, Peter (April 24, 2003). "Twiddler's Syndrome". New England Journal of Medicine. Massachusetts Medical Society. 348 (17): 1726–1727. doi:10.1056/nejm200304243481722. ISSN   0028-4793. PMID   12711756.
  6. 1 2 Sharifi, Mohsen; Inbar, Shmuel; Neckels, Brenda; Shook, Heather (March 2005). "Twiddling to the extreme: development of twiddler syndrome in an implanted cardioverter-defibrilllator". The Journal of Invasive Cardiology. 17 (3): 195–196. PMID   15867459.
  7. 1 2 Fahraeus, T (2003). "Early pacemaker twiddler syndrome". Europace. Oxford University Press (OUP). 5 (3): 279–281. doi:10.1016/s1099-5129(03)00032-1. ISSN   1099-5129. PMID   12842644.
  8. 1 2 Castillo, Ricardo; Cavusoglu, Erdal (2006). "Twiddler's Syndrome: An Interesting Cause of Pacemaker Failure". Cardiology. S. Karger AG. 105 (2): 119–121. doi:10.1159/000090213. ISSN   0008-6312. PMID   16357482. S2CID   30025246.

Further reading