Arrhythmogenic Right Ventricular Cardiomyopathy: Prognostic Value of Electroanatomic Voltage Mapping

Background: Endocardial voltage mapping (EVM) identifies low-voltage right ventricular (RV) areas, which may represent the electroanatomic scar substrate of life-threatening tachyarrhythmias. We prospectively assessed the prognostic value of EVM in a consecutive series of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods: We studied 69 consecutive ARVC patients [47 males; median age 35 years(28-45)] who underwent electrophysiological study and both bipolar and unipolar EVM. The extent of confluent bipolar (<1.5mV) and unipolar (<6.0mV) low-voltage electrograms was estimated using the CARTO-incorporated area calculation software. Results: Fifty-three patients (77%) showed ≥1 RV electroanatomic scars with an estimated burden of bipolar vs unipolar low-voltage areas of 24.8% (7.2-31.5) and 64.8% (39.8-95.3), respectively (P=0.009). In the remaining patients with normal bipolar-EVM (n=16;23%), the use of unipolar EVM unmasked ≥1 region of low-voltage electrogram affecting 26.2% (11.6-38.2) of RV wall. During a median follow-up of 41 (28-56) months, 19(27.5%) patients experienced arrhythmic events, such as sudden death (n=1), appropriate ICD interventions (n=7), or sustained ventricular tachycardia (n=11). Univariate predictors of arrhythmic outcome included previous cardiac arrest or syncope (HR=3.4; 95%CI:1.4-8.8; P=0.03) and extent of bipolar low-voltage areas (HR=1.7 per 5%; 95%CI=1.5-2; P<0.001), while the only independent predictor was the bipolar low-voltage electrogram burden (HR=1.6 per 5%; 95% CI:1.2-1.9; P<0.001). Patients with normal bipolar-EVM had an uneventful clinical course. Conclusions: The extent of bipolar RV endocardial low-voltage area was a powerful predictor of arrhythmic outcome in ARVC, independently of history and RV dilatation/dysfunction. A normal bipolar-EVM characterized a low-risk subgroup of ARVC patients

The effects of COVID-19 pandemic countermeasures on patients receiving botulinum toxin therapy and on their caregivers: a study from an Italian cohort

COVID-19 outbreak had a huge worldwide impact, and several countermeasures to contain its spread have been adopted, such as the interruption of nonurgent outpatient clinics. We wanted to describe the effects of the national lockdown on the well-being of a cohort of Italian patients with cerebral palsy (CP) receiving botulinum toxin (BT) therapy and of their caregivers. Twenty-five patients receiving BT therapy were surveyed using the structuralized questionnaire by Dressler and Adib Saberi, while the caregivers were assessed using the Caregiver Burden Scale. The lockdown delayed BT therapy by 9 ± 2.8 months. Around 44% of the selected patients noticed increased muscle cramps, 24% increased muscle pain, and 32% both of them. Due to the lockdown, the patient's quality of life was reduced by 68.4 ± 21.1%. After the lockdown, 100% of patients perceived BT therapy as more important than before. Around 76% of the patients perceived the lockdown as inadequate and felt that their rights were not respected. The overall score of the Caregiver Burden Scale, as regarded before the lockdown, was 29.12 ± 11.63, while the overall score as regarded after the lockdown was 37.44 ± 14.85. The overall score increased, from before the lockdown to after the lockdown, for 92% of caregivers. The BT outpatient clinic's interruption was seen to significantly worsen the psychophysical condition of subjects with CP and the care burden of their caregivers, exposing them to greater stress than before. Therefore, any kind of BT treatment suspension or delay should be avoided

Cardiology in a Digital Age: Opportunities and Challenges for e-Health: A Literature Review

To date, mortality rates associated with heart diseases are dangerously increasing, making them the leading cause of death globally. From this point of view, digital technologies can provide health systems with the necessary support to increase prevention and monitoring, and improve care delivery. The present study proposes a review of the literature to understand the state of the art and the outcomes of international experiences. A reference framework is defined to develop reflections to optimize the use of resources and technologies, favoring the development of new organizational models and intervention strategies. Findings highlight the potential significance of e-health and telemedicine in supporting novel solutions and organizational models for cardiac illnesses as a response to the requirements and restrictions of patients and health systems. While privacy concerns and technology-acceptance-related issues arise, new avenues for research and clinical practice emerge, with the need to study ad hoc managerial models according to the type of patient and disease

Antithrombotic treatment management in low stroke risk patients undergoing cardioversion of atrial fibrillation <48 h duration: results of an EHRA survey

Data supporting the safety of cardioversion (CV) of atrial fibrillation (AF) without anticoagulation in patients with AF duration <48 h are scarce. Observational studies suggest that the risk of stroke in these patients is very low when the definite duration of the AF episode is of <48 h and the clinical risk profile as estimated through the CHA2DS2VASc score is low (a score of 0 for men and 1 for women). As the recent 2020 European Society of Cardiology (ESC) guidelines indication for this clinical scenario is based mainly on consensus, we sent out a survey to assess the current clinical practice on anticoagulation prior to and post-CV in patients with AF <24-48 h duration and low stroke risk across centres in Europe. Of the 136 respondents, half were affiliated to university hospitals (68/136; 50%). Non-university hospitals (50/136; 36%) and private hospitals (2/136; 1.4%) accounted over a third of respondents. The main findings of our survey were (i) heterogeneity in the anticoagulation management both before and post-CV in low stroke-risk patients with AF <48 h, (ii) higher utilization of periprocedural low-molecular-weight heparin than of non-vitamin K antagonist oral anticoagulant, (iii) higher utilization of pre-CV transoesophageal echocardiography for electrical CV than for pharmacological CV regardless of the duration of AF, (iv) high adherence to a 4-week post-CV oral anticoagulant (OAC) therapy, mainly for electrical CV, and finally, (v) perceived higher acceptance of lack of post-CV OAC therapy in patients with <24 h than 24-48 h episode duration. The results obtained in this survey highlight the need for more research providing definitive clarification on the safety of CV without anticoagulation in patients with short duration AF

INTERMUSCULAR TWO-INCISION TECHNIQUE FOR S-ICD IMPLANTATION

Background The traditional technique for subcutaneous implantable cardioverter defibrillator (S-ICD) implantation, which involves three incisions and a subcutaneous pocket, is associated with possible complications, including inappropriate interventions. The aim of this prospective multicenter study was to evaluate the efficacy and safety of an alternative intermuscular two-incision technique for S-ICD implantation. Methods The study population included 36 consecutive patients (75% male, mean age 44 ± 12 years [range 20–69]) who underwent S-ICD implantation using the intermuscular two-incision technique. This technique avoids the superior parasternal incision for the lead placement and consists of creating an intermuscular pocket between the anterior surface of the serratus anterior and the posterior surface of the latissimus dorsi muscles instead of a subcutaneous pocket. Results All patients were successfully implanted in the absence of any procedure-related complications with a successful 65-J standard polarity defibrillation threshold testing, except in one, who received a second successful shock after pocket revision. During a mean follow-up of 10 months (range 3–30), no complications requiring surgical revision were observed. At device interrogation, stable sensing without interferences was observed in all patients. Two patients (5.5%) experienced appropriate and successful shock on ventricular fibrillation and in four patients (11%), a total of seven nonsustained self-terminated ventricular tachycardias were correctly detected. No inappropriate interventions were observed. Conclusions Our experience suggests that the two-incision intermuscular technique is a safe and efficacious alternative to the current technique for S-ICD implantation that may help reducing complications including inappropriate interventions and offer a better cosmetic outcome, especially in thin individuals

The "Defibrillation Testing, Why Not?" survey. Testing of subcutaneous and transvenous defibrillators in the Italian clinical practice

Background: Defibrillation testing (DT) can be omitted in patients undergoing transvenous implantable cardioverter-defibrillator (T-ICD) implantation, but it is still recommended for patients at risk for a high defibrillation threshold and for ICD generator changes. Moreover, DT is still recommended on implantation of subcutaneous ICD (S-ICD). The aim of the present survey was to analyze the current practice of DT during T-ICD and S-ICD implantations.Methods: In March 2021, an ad hoc questionnaire on the current performance of DT and the standard practice adopted during testing was completed at 72 Italian centers implanting S-ICD and T-ICD.Results: 48 (67%) operators reported never performing DT during de-novo T-ICD implantations, while no operators perform it systematically. The remaining respondents perform it for patients at risk for a high defibrillation threshold. DT is never performed at T-ICD generator change. At the time of de-novo S-ICD implantation, DT is never performed by 9 (13%) operators and performed systematically by 48 (66%). The remaining operators frequently omit DT in patients with more severe systolic dysfunction. DT is not performed at S-ICD generator change by 92% of operators. DT is conducted by delivering a first shock energy of 65 J by 60% of operators, while the remaining 40% test lower energy values.Conclusions: In current clinical practice, most operators omit DT at T-ICD implantation, even when still recommended in the guidelines. DT is also frequently omitted at S-ICD implantation, and a wide variability exists among operators in the procedures followed during DT

Febrile rhabdomyolysis of unknown origin in refugees coming from West Africa through the Mediterranean

Abstract Objectives Cases of undiagnosed severe febrile rhabdomyolysis in refugees coming from West Africa, mainly from Nigeria, has been observed since May 2014. The aim of this study was to describe this phenomenon. Methods This was a multicentre retrospective observational study of cases of febrile rhabdomyolysis reported from May 2014 to December 2016 in 12 Italian centres. Results A total of 48 cases were observed, mainly in young males. The mean time interval between the day of departure from Libya and symptom onset was 26.2 days. An average 8.3 further days elapsed before medical care was sought. All patients were hospitalized with fever and very intense muscle aches. Creatine phosphokinase, aspartate aminotransferase, and lactate dehydrogenase values were abnormal in all cases. The rhabdomyolysis was ascribed to an infective agent in 16 (33.3%) cases. In the remaining cases, the aetiology was undefined. Four out of seven patients tested had sickle cell trait. No alcohol abuse or drug intake was reported, apart from a single reported case of khat ingestion. Conclusions The long incubation period does not support a mechanical cause of rhabdomyolysis. Furthermore, viral infections such as those caused by coxsackievirus are rarely associated with such a severe clinical presentation. It is hypothesized that other predisposing conditions like genetic factors, unknown infections, or unreported non-conventional remedies may be involved. Targeted surveillance of rhabdomyolysis cases is warranted

Morphofunctional abnormalities of mitral annulus and arrhythmic mitral valve prolapse

Background\u2014Arrhythmic mitral valve prolapse (MVP) is characterized by myxomatous leaflets and left ventricular (LV) fibrosis of papillary muscles and inferobasal wall. We searched for morphofunctional abnormalities of the mitral valve that could explain a regional mechanical myocardial stretch. Methods and Results\u2014Thirty-six (27 female patients; median age: 44 years) arrhythmic MVP patients with LV late gadolinium enhancement on cardiac magnetic resonance and no or trivial mitral regurgitation, and 16 (6 female patients; median age: 40 years) MVP patients without LV late gadolinium enhancement were investigated by morphofunctional cardiac magnetic resonance. Mitral annulus disjunction (median: 4.8 versus 1.8 mm; P1.5 (22 [61%] versus 4 [25%]; P=0.016) were higher in MVP patients with late gadolinium enhancement than in those without. A linear correlation was found between mitral annulus disjunction and curling (R=0.85). A higher prevalence of auscultatory midsystolic click (26 [72%] versus 6 [38%]; P=0.018) was also noted. Histology of the mitral annulus showed a longer mitral annulus disjunction in 50 sudden death patients with MVP and LV fibrosis than in 20 patients without MVP (median: 3 versus 1.5 mm; P<0.001). Conclusions\u2014Mitral annulus disjunction is a constant feature of arrhythmic MVP with LV fibrosis. The excessive mobility of the leaflets caused by posterior systolic curling accounts for a mechanical stretch of the inferobasal wall and papillary muscles, eventually leading to myocardial hypertrophy and scarring. These mitral annulus abnormalities, together with auscultatory midsystolic click, may identify MVP patients who would need arrhythmic risk stratification