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  • According to most published reports patients with Twiddler s

    2019-04-15

    According to most published reports, patients with Twiddler\'s syndrome are usually diagnosed due to some type of sudden device malfunction [1–4,6–15]. Early detection of this condition can also be achieved with a home monitoring system [8]. The case presented is a rare one in which Twiddler\'s syndrome was detected before device malfunction occurred. According to published reports, the patient\'s unconscious twiddling of the generator is typically the cause of this syndrome. However, the patient in the case presented did not have any mental disorders and denied having rotated the generator herself. The patient recognized and reported that the generator sometimes rotated in the subcutaneous pocket, which was helpful for the early diagnosis. In addition, lead dislodgment may have been avoided by using active fixation leads. Twiddler\'s syndrome has been described as presenting complications in cases where the subcutaneous tissues are lax, the generator is untethered in its pocket, or the size of the pocket exceeds that of the generator. Patients who are most at risk of this condition include middle-aged, obese women, and patients with mental disorders such as dementia [3]. Lax subcutaneous tissue was the most plausible risk factor in the case presented. If several risk factors for Twiddler\'s syndrome, such as obesity, excessive movements of the upper limbs, active manipulation of the generator, large size pocket, or dementia, are observed, preventive maneuvers like anchoring the generator to the bottom of the pocket or sub-pectoral implants should be considered during implantation. Furthermore, Twiddler\'s syndrome usually occurs one year or less after lysophosphatidic acid of the device [1–15]. In the case presented, the condition was diagnosed 5 months after the implantation. Therefore, we should be aware that Twiddler\'s syndrome may occur within one year, especially in high-risk cases such as those described above.
    Introduction Amyloidosis refers to conditions in which amyloid proteins are abnormally deposited in organs. Symptoms vary widely and depend on the kinds of overproduced amyloid proteins and the sites of amyloid deposition. The 2 major types of amyloidosis are immunoglobulin light chain (AL) amyloidosis and amyloid A protein (AA) amyloidosis. AL amyloidosis is a systemic disease that is characterized by extracellular tissue deposition of immunoglobulin heavy or light chain fragments. This extracellular accumulation leads to characteristic histologic changes and organ dysfunction. Amyloid deposits can occur in a variety of organs, including the heart, kidney, liver, gastrointestinal tract, and autonomic nervous system. Cardiac involvement occurs in up to 30–50% of patients with AL amyloidosis and less than 5% with AA amyloidosis [1,2]. AL amyloidosis has a poor long-term prognosis [3,4], the median survival may be as short as 4–6 months, and the major causes of death are arrhythmia and heart failure, although several chemotherapeutic regimens have been attempted in some selected patients with AL amyloidosis. It has been reported that heart failure accounted for 51% of the deaths [5]. In the early stage, patients often present diastolic dysfunction followed by systolic dysfunction, which is refractory to standard therapies for heart failure at late disease stages [6]. In addition, many patients suffer from several kinds of arrhythmia. Arrhythmic features of amyloid cardiomyopathy are conduction abnormalities such as sick node dysfunction, second- and third-degree atrioventricular block, atrial fibrillation, and ventricular arrhythmia. The conduction system is often affected in patients with amyloid cardiomyopathy. Although the sinus node may be most involved, electrophysiologic dysfunction appears to be most common in the His-Purkinje system [7]. A permanent pacemaker is indispensable and commonly used for bradycardiac arrhythmia, but several previous reports suggest a limited effect in reducing the risk of sudden death [1,8].