A case series of the twiddler syndrome.

Background: Twiddler syndrome (TS) is a complication of cardiac implantable electronic device (CIED) implantation, caused by the deliberate or unconscious manipulation of the device by the patient himself, which results in dislocation of the leads by retraction towards the subcutaneous pocket. Case summary: This report describes two clinical cases that occurred in our centre, for which two different solutions were successfully implemented. In the first case, a complete removal of the stimulation system was performed, and a leadless pacemaker (PM; Medtronic Micra VR) was implanted. In the second case, the patient underwent a revision procedure. The PM was disconnected, and the electrodes were debrided, a submuscular pocket for the PM was created, and at the end of the procedure, the PM was anchored to the pectoralis major. Discussion: Twiddler syndrome is a not so rare and serious complication of CIED implantation, leading to device malfunctioning and higher risk of infection of the pocket due to multiple re-interventions. In these two cases, different surgical solutions were performed, both resulting to be effective to solve the effects of TS.

Syncope and Cannabis: hypervagotonia from chronic abuse? A case report and literature review.

BACKGROUND: Cannabis is the most consumed drug worldwide and number of users is increasing, particularly among youth. Moreover, cannabis potential therapeutic properties have renewed interest to make it available as a treatment for a variety of conditions. Albeit rarely, cannabis consumption has been associated with cardiovascular diseases such as arrhythmias, myocardial infarction (MI) and potentially sudden death. CASE PRESENTATION: A 24-year-old woman presented to the emergency department sent by her cardiologist because of a recent finding of a 16 seconds asystole on the implantable loop recorder (ILR) she implanted 7 months before for recurrent syncopes. She declared that she is a heavy cannabis user (at least 5 cannabis-cigarette per day, not mixed up with tobacco, for no less than 12 years) and all syncopes occurred shortly after cannabis consumption. After a collective discussion with the heart team, syncope unit, electrophysiologists and toxicologist, we decided to implant a dual chamber pacemaker with a rate response algorithm due to the high risk of trauma of the syncopal episodes. 24 months follow-up period was uneventful. CONCLUSIONS: Cannabis cardiovascular effects are not well known and, although rare, among these we find ischemic episodes, tachyarrhythmias, symptomatic sinus bradycardia, sinus arrest, ventricular asystole and possibly death. Because of cannabis growing consumption both for medical and recreational purpose, cardiovascular diseases associated with cannabis use may become more and more frequent. In the light of the poor literature, we believe that cannabis may produce opposite adverse effects depending on the duration of the habit. Acute administration increases sympathetic tone and reduces parasympathetic tone; conversely, with chronic intake an opposite effect is observed: repetitive dosing decreases sympathetic activity and increases parasympathetic activity. Clinicians should be aware of the increased risk of cardiovascular complications associated with cannabis use and should investigate its consumption especially in young patients presenting with cardiac dysrhythmias.

Does Patient Compliance Influence Wearable Cardioverter Defibrillator Effectiveness? A Single-Center Experience.

The study was designed to assess patient adherence to wearable cardioverter defibrillator as an indicator of device effectiveness. The patient training is not widely properly standardized. We enrolled 25 patients with a wearable cardioverter defibrillator to prevent sudden cardiac death between June 2020 and August 2022. Among them, 84% were male with a median age of 63.6 years. The indication was an ischemic (44%) and a non-ischemic (56%) disease. The patients were followed-up until the decision to upgrade to an implantable device was taken. We trained the patients according to our suggested protocol. The median wear time was 90 days, and the median daily wear time was 23.5 h, similar throughout sex, age, and indication groups. In total, 24% of the participants underwent cardioverter defibrillator implantation. Between the device-implanted and non-implanted groups, left ventricular ejection fraction and left ventricular indexed end-diastolic volume were significantly different (EF 35.8 ± 12 vs. 46.4 ± 8.5%, p = 0.028, iEDV 108 ± 52 vs. 70.7 ± 21.1 mL/m2, p = 0.024). We did not find any differences in cardiac magnetic resonance data, even though all patients who underwent device implantation had late gadolinium enhancement spots. Our results support standardized patient training to obtain great patient adherence to the instructions to the wearable device and therefore its effectiveness.

Nationwide survey on the current practice of ventricular tachycardia ablation.

METHODS: We performed a nationwide survey on the current practice of ventricular tachycardia catheter ablation in Italy during the year 2016. RESULTS: Among 145 operators participating in the survey, 58 (40.0%) did not perform any ventricular tachycardia ablation in 2016. Among those performing ventricular tachycardia ablation, 9 operators (6.2%) performed only right ventricular endocardial catheter ablation, 52 (35.9%) performed endocardial catheter ablation both in the right and left ventricle (LV) and 26 (17.9%) performed both endocardial and epicardial LV catheter ablations. Seventy operators (89.7%) among the 78 performing LV and epicardial ablations treated patients with ischemic cardiomyopathy; ablations in the setting of other causes were less frequently performed. The following were considered as minimum requirements for ventricular tachycardia ablation: presence of a three-dimensional mapping system (120 operators, 82.8%), ICU in the hospital (118 operators, 81.4%), operator's training in high volume centers (93 operators, 64.1%). Twenty-eight operators (19.3%) performed catheter ablation in patients with electrical storm only after hemodynamic stabilization, 41 operators (28.3%) also during the acute phase and 9 operators (6.2%) never performed catheter ablation in electrical storm patients; the remaining 67 operators did not perform ventricular tachycardia ablation at all, or performed ablations only in the right ventricle. CONCLUSION: The present survey provides a snapshot of the current invasive treatment of ventricular tachycardia by catheter ablation. The procedure, especially in the setting of ischemic cardiomyopathy, is performed nationwide. Complex cases, including those with electrical storm, should be managed within a preestablished integrated network of regional referral centers able to transfer patients as soon as possible.

Cardiac involvement in patients with lamin A/C gene mutations: a cohort observation.

INTRODUCTION: LMNA gene mutations are associated with cardiac and skeletal muscle alterations. METHODS: A cohort of 21 mutated individuals was assessed with clinical and instrumental investigations over the years. RESULTS: The median observation period was 6 years. Cardiac compromise was detected in 16 patients. Bradyarrhythmias were the most frequent manifestations, followed by supraventricular arrhythmias. Two individuals suffered from nonsustained and 1 from sustained ventricular tachyarrhythmias. Dilated cardiomyopathy was detected in 3 patients. Evaluation of the frequencies of the clinical expressions showed a high probability of suffering from analogue heart compromise in study subjects bearing the same LMNA gene mutation. CONCLUSIONS: Cardiac involvement represents a very common phenotypic expression of LMNA gene mutation. Subjects sharing common genetic background seem to suffer from analogue pattern of cardiac manifestation.

Cardiac and muscle imaging findings in a family with X-linked Emery-Dreifuss muscular dystrophy.

The following is a report on a large family with 5 males affected by the X-linked recessive form of Emery-Dreifuss muscular dystrophy with mutation in the STA gene. A detailed longitudinal cardiological evaluation and muscle imaging studies allowed for the assessment of intrafamilial variability of cardiac and muscle involvement. Long term cardiological follow up in the 5 affected males and in 7 female carriers revealed different degrees of severity, ranging from tachycardia-bradycardia syndrome and variable biatrial and left ventricle dilatation, to an episode of isolated symptomatic sustained ventricular tachycardia requiring a device implantation. Muscle imaging in the affected males showed involvement of the soleus and medial head of gastrocnemius on leg muscles and variable involvement on thigh muscles that have not been previously reported. In some cases, imaging showed clear signs of muscle involvement even when no overt signs of weakness could be detected during clinical examination.

Muscle MRI findings in patients with an apparently exclusive cardiac phenotype due to a novel LMNA gene mutation.

The case of a family in which several members displayed conduction defects inherited as a dominant trait is reported. The proband was a young woman with a 1st degree atrio-ventricular block and high serum creatine kinase. Several members of the family featured cardiologic symptoms. All adult family members were clinically evaluated and blood tests including serum creatine-kinase levels, standard and Holter ECG, echocardiogram and muscle MRI were performed. LMNA gene analysis was carried out and a novel missense mutation consisting in substitution of exon 4 c.799 T/C, p.Tyr267His was revealed. The mutation was present in seven family members, five of whom displayed cardiac defects alone with no involvement of the skeletal muscle. In all mutated individuals muscle MRI featured a pattern of skeletal muscle involvement similar to that observed in autosomal dominant Emery Dreifuss muscular dystrophy, suggesting that even patients bearing a LMNA gene mutation associated to an apparently selective cardiac phenotype may present subclinical skeletal muscle involvement.