Twiddler's Syndrome Research Articles

Issue Highlights

HomeCirculationVol. 114, No. 15Issue Highlights Free AccessIn BriefPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessIn BriefPDF/EPUBIssue Highlights Originally published10 Oct 2006https://doi.org/10.1161/circ.114.15.1555Circulation. 2006;114:1555IMPLICATIONS OF THE FAILURE TO IDENTIFY HIGH-RISK ELECTROCARDIOGRAM FINDINGS FOR THE QUALITY OF CARE OF PATIENTS WITH ACUTE MYOCARDIAL INFARCTION: RESULTS OF THE EMERGENCY DEPARTMENT QUALITY IN MYOCARDIAL INFARCTION (EDQMI) STUDY, by Masoudi et al.The appropriate diagnosis and treatment of patients with an acute myocardial infarction depends largely on the correct interpretation of the electrocardiogram in the emergency department. Despite the importance of the skill of correctly interpreting the electrocardiogram, relatively little is known about the prevalence of misinterpretations and their consequences. This retrospective study by Masoudi et al examined the failure to identify high-risk electrocardiographic findings in patients presenting to 1 of 5 emergency departments in California and Colorado. The authors report the failure to identify important ST-segment depressions, elevations or T-wave inversions on the first electrocardiogram of patients with a confirmed acute myocardial infarction. They also investigated the association of the misinterpretations with the quality of patient care and in-hospital mortality. The present study identifies an important opportunity for us to improve care. See p 1565.DRAWBACKS AND PROGNOSTIC VALUE OF FORMULAS ESTIMATING RENAL FUNCTION IN PATIENTS WITH CHRONIC HEART FAILURE AND SYSTOLIC DYSFUNCTION, by Smilde et al.The adverse prognostic impact of impaired renal function has been well demonstrated across the entire spectrum of cardiovascular diseases. In this issue of Circulation, Smilde and colleagues performed a detailed comparison of the 2 widely-used creatinine-based estimates of renal function (Cockcroft-Gault and Modification of Diet in Renal Disease equations) with the much more difficult to perform “gold standard” iothalamate clearance, in patients with chronic heart failure and reduced left ventricular ejection fraction. Although subtle differences were observed between these assessments of renal function, each provided important prognostic information regarding subsequent cardiovascular outcomes. See p 1572.HIGH SERUM C-REACTIVE PROTEIN LEVEL IS NOT AN INDEPENDENT PREDICTOR FOR STROKE: THE ROTTERDAM STUDY, by Bos et al.Increasingly, C-reactive protein (CRP) is recommended for cardiovascular risk prediction. Major adverse events in subjects at risk or in patients with cardiovascular disease not only include acute coronary syndromes and sudden death, but also stroke. The 2 clinical entities differ considerably, however, and stroke itself is heterogenous and includes ischemic as well as hemorrhagic stroke. Thus, predictive markers for stroke or acute coronary syndromes may differ. Bos et al used the Rotterdam cohort of over 6000 subjects without previous stroke to address this issue. During a follow-up period of more than 8 years, almost 500 strokes occurred. High CRP levels were associated with the risk of any stroke and of ischemic strokes. However, CRP did not improve stroke risk prediction as assessed by the Framingham score, age, or sex. Thus, it appears that the clinical utility of CRP for stroke prediction above and beyond simple clinical parameters is quite limited. It is possible that CRP might reflect the degree of inflammation that is caused by the risk factors of a given patient, but CRP, by itself, does not add predictive value. Nevertheless, vascular inflammation may still be involved in the development of cerebrovascular disease. See p 1591.Visit http://circ.ahajournals.org:Cardiology Patient PageThe Metabolic Syndrome. See p e528.Images in Cardiovascular MedicineCough Cardiopulmonary Resuscitation Revisited. See p e530.Case of Anomalous Right Superior Vena Cava. See p e532.Hiccups and Dysphonic Metallic Voice: A Unique Presentation of Twiddler Syndrome. See p e534. Download figureDownload PowerPointCorrespondenceSee p e536. Previous Back to top Next FiguresReferencesRelatedDetails October 10, 2006Vol 114, Issue 15 Advertisement Article InformationMetrics https://doi.org/10.1161/circ.114.15.1555 Originally publishedOctober 10, 2006 PDF download Advertisement

Hiccups and Dysphonic Metallic Voice

Twiddler syndrome refers to a rare complication observed in patients with implanted pacemakers or defibrillators that causes device malfunction. The condition arises as a result of the patient’s conscious or unconscious manipulation of the device, causing its rotation in the pocket, resulting in torsion and dislodgment of the implanted lead(s). A 65-year-old obese woman with idiopathic dilated cardiomyopathy and diabetes mellitus had undergone implantation of a biventricular implantable cardioverter-defibrillator (CRT-D, Medtronic Insync Maximo 7304) with the positioning of 3 leads: a passive bipolar lead in the auricula of the right atrium (Medtronic Capsure Z Novus 5554), a bipolar, single-coil, screw-in lead (Medtronic Sprint 6931) in the apex of the right ventricle (RV), and a unipolar left-ventricular (LV) pacing lead (Medtronic Attain 4193) in an anterolateral tributary of the coronary sinus. Approximately 1 month after implantation, the patient presented to the emergency room with effort …

Biventricular Pacing Using Dual‐Site Right Ventricular Stimulation: Is It Placebo Effect?

Alternate site lead placement in cardiac resynchronization therapy has been used successfully but remains to be validated. A 62-year-old heart failure patient in whom coronary sinus lead placement was not possible underwent implantation of the lead in the right ventricular outflow tract (RVOT) and demonstrated clinical improvement as measured by New York Heart Association class and noninvasive parameters. When heart failure recurred, it was determined that his RVOT electrode had been pulled back (Twiddler's syndrome). Repositioning again improved his clinical status and noninvasive hemodynamic measurements. With dual-site right ventricular (RV) pacing there was no echocardiographic measurable intraventricular dyssynchrony. Tissue Doppler imaging correlated with clinical improvement using dual-site RV pacing, providing evidence that this technique may represent a viable alternative in cardiac resynchronization therapy.

Multiple inappropriate defibrillator discharges due to Twiddler's syndrome

A 56-year-old man with a history of prior myocardial infarction, ischemic cardiomyopathy and nonsustained episodes of ventricular tachycardia had received a subpectorally placed implantable cardioverter-defibrillator (ICD) (Medtronic Micro-Jewel II® 7223 Cx with a single-coil electrode Medtronic Sprint® 6932) for primary prevention of sudden cardiac death three years ago. He was referred to our department after having received twenty-five inappropriate shocks. Interrogation and testing of the device revealed exit-block; incorrect sensing could easily be reproduced and visualized in the ICD’s marker channel by shaking the ICD can. The patient denied tampering with the device, had no history of recent weight loss, psychiatric illness or any other obvious condition with which rotation of the device could be associated. Chest x-ray revealed knotting of the lead just distally of the ICD header (Fig. 1) and multiple twisting of the lead on its way through the anonymous vein. Twiddler’s syndrome with multiple kinks could be confirmed intraoperatively (Fig. 2). The lead was extracted and the whole system replaced. Twiddler’s syndrome, originally described in a pacemaker patient by Bayliss in 1968 [1], denotes a complication in pacemaker and defibrillator patients, in which manipulation with the device leads to twisting of can and electrodes with the consequence of lead displacement. While most of the patients persistently deny manipulation with the device, this is the accepted mechanism leading to lead dislodgement. Previously reported factors that may predispose to device rotation include advanced age, female gender, obesity, small size of the device relative to its pocket and psychiatric disease. Fortunately, Twiddler’s syndrome is a rare complication in ICD patients, however, it may lead to severe complications such as inappropriate shock delivery, which itself may cause ventricular fibrillation, or unrescued sudden cardiac death. Despite the fact that only a small number of Twiddler’s syndromes in subpectorally implanted devices have been published so far [e.g. 2, 3], it cannot be reasoned that ICDs in this position are less predisposed to twiddle.

Lead system causing twiddler's syndrome in patients with an implantable cardioverter-defibrillator

Three male patients with an implantable cardioverterdefibrillator (ICD) were referred for lead extraction because of twiddler’s syndrome associated with lead malfunction detected during routine follow-up (Table 1). The ICDs were implanted in the left subpectoral region in a submuscular pocket. On chest radiograph, the position of the generator was the same as at implant in all 3 patients. All patients were satisfied with the device and denied manipulation of the generator or discomfort at the implant site. All had a history of recent strenuous physical exercise. None of the patients was obese or reported important weight loss since the time of implant. We could not rotate the generator within the generator pocket in any of the patients, even during surgery, because the pocket fitted snugly in all. The leads were twisted tightly outside the generator pocket, and atrial and defibrillation leads were twirled around each other in the patients with a dual-chamber ICD (Figure 1). The torque of the leads had deformed the insulation, and in 1 patient there were insulation defects at the site of the connector and the yoke. The leads were extracted in all 3 patients, in 2 with a laser sheath. New leads were inserted in 2 patients through an exchange procedure and by renewed catheterization of the subclavian vein in the first patient. Care was taken not to leave any redundant lead outside the generator pocket, which was closed separately. The original ICD was reimplanted in all patients. One patient had recurrence of the syndrome. Seven months after the revision, the chest radiograph showed an unchanged position of the leads and generator. He then took up swimming but presented 3 months later with recurrence of twiddler’s syndrome. The other patients showed no recurrence during a follow-up of 10 and 16 months. Discussion Twiddler’s syndrome of ICDs is typically attributed to either conscious or unconscious rotation of the generator. The patients are often obese women with large defibrillator pockets, sometimes attributed to weight loss facilitating manipulation and rotation of

Secure pacemaker fixation critical for prevention of Twiddler's syndrome

A two year old, female spayed border collie presented three weeks after permanent pacemaker implantation for weakness, lethargy and collapse. Electrocardiogram documented complete (3 rd degree) atrioventricular (AV) block, the absence of pacing and a ventricular escape rhythm. Thoracic radiographs revealed retraction and dislodgement of the passive fixation lead from the right ventricular apex. During a procedure to reposition the lead, it was noted that lead retraction had resulted from rotation of the generator with twisting and entanglement of the lead on itself and around the generator, a complication recognized in human patients as “Twiddlers syndrome”. The pacemaker lead was removed, a new lead securely placed at the right ventricular apex, and the generator was secured into a revised subcutaneous pocket. Pacing was re-established and the Twiddler's syndrome has not reoccurred.

Reel Syndrome and Pulsatile Liver in a Patient With a Two-chamber Pacemaker

Twiddler's syndrome is characterized by coiling of the pacemaker lead due to the rotation of the pacemaker generator on its long axis. Reel syndrome is another form of Twiddler's syndrome. It occurs due to the rotation of the pacemaker generator on its transverse axis with subsequent coiling of the pacemaker leads around the pulse generator. In this article we describe a patient with a two-chamber pacemaker who presented with sudden onset of abdominal pulsation and was subsequently diagnosed as Reel syndrome. To the best of our knowledge, this case is the first case of Reel syndrome that developed in a patient with a two-chamber pacemaker.

Twiddler syndrome

Twiddler syndrome

108th ENMC International Workshop, 3rd Workshop of the MYO-CLUSTER project: EUROMEN, 7th International Emery-Dreifuss Muscular Dystrophy (EDMD) Workshop, 13–15 September 2002, Naarden, The Netherlands

Inserm UR582 (ex 523), Institut de Myologie, Bâtiment Babinski, G.H. Pitie-Salpetriere, 47, boulevard de l’Hopital, 75 651 Paris Cedex 13, France Laboratoire de Genetique Moleculaire et Chromosomique, IURC, Montpellier, France Institute of Cardiology, University of Bologna, Bologna, Italy Department of Paediatrics and Neonatal Medicine, Imperial College School of Medicine, Hammersmith Campus, London, UK Department of Neurology, Academic Medical Centre, Amsterdam, The Netherlands Service de Cardiologie, GH Cochin, Paris, France Randall Center, Kings College, London, UK Neuromuscular Unit, M.R.C. Polish Academy of Sciences, Warsaw, Poland ITOI, Unit of Bologna, c/o IOR, Bologna, Italy Istituto Ortopedico Rizzoli, Neuromuscular Unit, Bologna, Italy MRIC, North East Wales Institute, Wrexham, UK Department of Internal Medicine and Cardiology Medical University of Warsaw, Warsaw, Poland Department of Cardiology, University Hospital Maastricht, Maastricht, The Netherlands Dipartimento di Biopatologia e Diagnostica per Immagini, Rome, Italy Istituto di Genetica Biochimica ed Evoluzionistica, CNR (IGBE-CNR), Pavia, Italy Division of Medical Genetics, University of Leicester, Leicester, UK Institute of Human Genetics, Greifswald, Germany

Twiddler's Syndrome

To the Editor: First described in 1968, pacemaker twiddler's syndrome refers to permanent malfunction of a pacemaker due to the patient's manipulation of the pulse generator.1 The sequence of symptoms begins with the patient's deliberate or subconscious spinning of the pacemaker's pulse generator in a capacious pocket. The leads are dislodged, and ventricular pacing ceases. Subsequently, with continual reeling of the leads around the generator, the ipsilateral phrenic nerve is stimulated, resulting in diaphragmatic pacing and the sensation of abdominal pulsations. As the leads are further wrapped around the generator, rhythmic arm twitching occurs when the brachial plexus is paced. . . .

Permanent Cardiac Pacemaker: an Emergency Perspective

The number of patients with permanent pacemaker are increasing and may present into the emergency department with a variety of complications and malfunctions. The presence of a pacemaker may also affect management of unrelated medical issues. This article review the complications of the implant procedure, the causes, diagnosis and management of pacemaker malfunction, the pacemaker syndrome, Twiddler's syndrome. Interaction of permanent pacemaker with other medical condition and its implication in Emergency Department environment are also discussed.

Síndrome de Twiddler en un paciente portador de desfibrilador automático implantable: ¿una complicación evitable?

The Twiddler's syndrome is an uncommon, but potentially life-threatening complication in patients with an implantable cardioverter-defibrillator. Early identification of repetitive vicious attitudes and a periodic radiological follow-up in predisposed patients, may be useful in the primary prevention of this syndrome.

Síndrome de twiddler y fallo de sensado auricular

144 Mujer de 83 anos, hipertensa y obesa, que consulto por disnea. En urgencias presentaba un cuadro de insuficiencia cardiaca, y el electrocardiograma evidencio un bloqueo auriculoventricular completo. Se comenzo tratamiento medico y se implanto marcapasos VDD. Despues del procedimiento solo se hizo una radiografia de torax en proyeccion anteroposterior (fig. 1). El electrocardiograma objetivaba funcionamiento adecuado del marcapasos. Al dia siguiente, se observo un defecto de sensado auricular en los electrocardiogramas, manteniendose una adecuada estimulacion ventricular. Se intento reprogramar el marcapasos sin exito. Se practico una radiografia de torax posteroanterior, que puso de manifiesto una rotacion del generador (fig. 2). En la radiografia lateral se observaba un desplazamiento del cable que se introducia formando un bucle en la cava inferior, dejando alli el dipolo auricular (fig. 3). La punta del cable seguia fijada correctamente al apex del ventriculo derecho. Esto explicaba el defecto de sensado selectivo auricular. La enferma fue reintervenida colocandose correctamente el marcapasos, siendo finalmente dada de alta. El sindrome de twiddler se caracteriza por la disfuncion de un marcapasos1 o de un desfibrilador implantable tanto de localizacion abdominal2 como subpectoral3, secundaria a la rotacion de la bateria en su bolsa subcutanea, con el consecuente desplazamiento de los electrodos. La mayoria de los casos descritos se dan en mujeres mayores y con cierto grado de obesidad que, presumiblemente, tienen un tejido subcutaneo mas laxo4,5. El sindrome de twiddler, facilmente identificable en una radiografia de torax, debe ser considerado cuando se investiguen las causas de disfuncion de un marcapasos o de un desfibrilador implantable. IM A G E N E S E N CA R D I O L O G I A

A Practical Approach for Repositioning Flipped Venous Access Ports

Almost any implanted device such as a pacemaker or venous access port is susceptible to inverting or turning itself over inside its fibrous capsule. This phenomenon called “Twiddler's syndrome” was first used by Bayliss et al (1) in 1968 who reported on the disruption and failure of a transvenous electrode by rotation of the impulse generator. The definition of the term “Twiddler's syndrome” has evolved over the years to also include abdominal Twiddler's syndrome (2) and chemotherapy port Twiddler's syndrome (3).

Pacemaker Lead Fracture Due to Twiddler's Syndrome

The authors report a case of pacemaker twiddler's syndrome that led to lead fracture and loss of consciousness. The role of chest X rays in the diagnosis of pacemaker twiddler's syndrome as well as the management of the syndrome is discussed.

Lead failure caused by twiddler's syndrome in subpectorally implanted unipolar single-lead cardioverter-defibrillators

Twiddler's syndrome, first described in 1968 by Bayless, Beanlands, and Baird 1 as a rare complication in patients with pacemakers, is characterized by dislodgment of transvenous leads by the twisting of pulse generators in the subcutaneous pocket, resulting in lead malfunction. As might be expected, similar complications could arise in the case of subpectorally placed implantable cardioverterdefibrillators (ICDs). Especially in unipolar defibrillation systems, which use the housing of the ICD itself as the second active defibrillation electrode, device migration could have a fatal influence on the defibrillation field. We report two cases of this rare complication. PATIENT 1. A 66-year-old male patient (body index 24.5 kg/m 2) with severe coronary artery disease and ventricular tachycardia was referred to our clinic. Bypass surgery was

A teenager with pacemaker twiddler syndrome

A teenager with pacemaker twiddler syndrome

Value of radiography in diagnosing complications of cardioverter defibrillators implanted without thoracotomy in 437 patients.

This study evaluated the usefulness of radiography in assessing the frequency and cause of complications of nonthoracotomy-implanted cardioverter defibrillators. Between May 1992 and December 1995, 437 consecutive patients at our institution underwent cardioverter defibrillator placement. Routine follow-up included external device testing at 6 weeks after placement and every 3 months thereafter. Chest radiographs were obtained immediately after placement, annually, and at the time of any suspected complication. Retrospective review of radiographs and medical charts was done for all patients with clinical complications. Forty-five complications (10%) were clinically diagnosed: lead or patch fracture in fifteen (33%) patients, electric lead dysfunction in eight (18%), infection in eight (18%), lead retraction in six (13%), patch fold in two (5%), hematoma in two (5%), and other complications in four (9%) patients. Eighteen complications (40%) were radiographically evident. Lead retraction, hematoma, patch fold, patch migration, and the twiddler syndrome were radiographically confirmed in 100% of cases. The average time for these complications to be detected was 68 days; 92% were detected within 23 days. Conversely, only four (27%) lead fractures, one (13%) electric lead dysfunction, and one (13%) infection were radiographically confirmed. These latter complications were discovered an average of 579 days after cardioverter defibrillator placement. Radiography plays a secondary role in the diagnosis of cardioverter defibrillator complications and is particularly limited beyond 1 month after placement. Radiographs may be helpful in the first month after placement because early complications are the most radiographically apparent.

Twiddler's Syndrome Complicating a Transvenous Defibrillator Lead System

Twiddler's syndrome is a rare complication seen in patients with implanted pacemakers or defibrillators. The condition typically presents with device malfunction and occurs when the patient either consciously or unconsciously twists and rotates the implanted device in its pocket, resulting in torsion and dislodgement of the implanted lead. A case of twiddler's syndrome involving a transvenous defibrillation lead and an abdominally implanted defibrillator is described. This is the first report of this complication with this particular lead. The patient in this report was a middle-aged obese diabetic woman who presented 7 months after defibrillator implantation with device noncapture and intermittent nonsensing. Review of the literature reveals that the majority of patients with this complication are middle-aged obese women with a defibrillator pocket that exceeds the size of the defibrillator. Treatment measures are discussed both for the patient with this complication and for the patient at increased risk for its occurrence.

Defibrillator Twiddler's Syndrome Causing Device Failure in a Subpectoral Transvenous System

Twiddler's syndrome is well described as a complication of cardiac pacing. Defibrillator twiddler's syndrome has been recently reported with abdominal implantations of epicardial and transvenous defibrillator systems. We report a case of a patient with a transvenous defibrillator system implanted with the pulse generator placed in the subpectoral plane. The patient developed twiddler's syndrome, which resulted in retraction of both leads. This caused inappropriate shocks due to sensing both the atrial and ventricular electrograms. While the subpectoral position leaves the generator deeper and more difficult for the patient to access, it may not lessen the chance of twiddler's syndrome. It is possible that the subpectoral position may actually predispose the patient to this malady.