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

Insertable cardiac monitor with a long sensing vector: Impact of obesity on sensing quality and safety

BackgroundFat layers in obese patients can impair R-wave detection and diagnostic performance of a subcutaneous insertable cardiac monitor (ICM). We compared safety and ICM sensing quality between obese patients [body mass index (BMI) ≥ 30 kg/m2] and normal-weight controls (BMI <30 kg/m2) in terms of R-wave amplitude and time in noise mode (noise burden) detected by a long-sensing-vector ICM.Materials and methodsPatients from two multicentre, non-randomized clinical registries are included in the present analysis on January 31, 2022 (data freeze), if the follow-up period was at least 90 days after ICM insertion, including daily remote monitoring. The R-wave amplitudes and daily noise burden averaged intraindividually for days 61–90 and days 1–90, respectively, were compared between obese patients (n = 104) and unmatched (n = 268) and a nearest-neighbour propensity score (PS) matched (n = 69) normal-weight controls.ResultsThe average R-wave amplitude was significantly lower in obese (median 0.46 mV) than in normal-weight unmatched (0.70 mV, P < 0.0001) or PS-matched (0.60 mV, P = 0.003) patients. The median noise burden was 1.0% in obese patients, which was not significantly higher than in unmatched (0.7%; P = 0.056) or PS-matched (0.8%; P = 0.133) controls. The rate of adverse device effects during the first 90 days did not differ significantly between groups.ConclusionAlthough increased BMI was associated with reduced signal amplitude, also in obese patients the median R-wave amplitude was >0.3 mV, a value which is generally accepted as the minimum level for adequate R-wave detection. The noise burden and adverse event rates did not differ significantly between obese and normal-weight patients.Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT04075084 and NCT04198220

Isolated multiple pericardial hydatid cysts in an asymptomatic patient: Role of the CMR

Hydatid cysts primarily affect the liver and secondary involvement may be seen in almost any anatomic location. We describe the unusual pericardial location of the Echinococcus cysts in an asymptomatic patient with suggestive cardiac magnetic resonance imaging

Leber's hereditary optic neuropathy, intellectual disability and epilepsy presenting with variable penetrance associated to the m.3460G>A mutation and a heteroplasmic expansion of the microsatellite in MTRNR1 gene - case report

Background: Leber's hereditary optic neuropathy (LHON) associated with mutations in mitochondrial DNA (mtDNA) typically manifests only optic nerve involvement but in some patients may develop additional neurological complications. The cause of this association is not clear. Case presentation: We present a case of a 24-year-old male with a history of subacute, painless, and rapidly progressive bilateral vision loss. We performed ophthalmological, neurological and neuropsychological investigations in the proband and his LHON family. The proband showed optic neuropathy, epilepsy, migraine, and intellectual disability; all the maternal relatives did not manifest optic neuropathy but a moderate to severe intellectual disability. Genetic screening revealed a novel association of the LHON m.3460G>A primary mutation with the m.T961delT+C(n)ins within the mitochondrial encoded 12S RNA (MTRNR1) gene which segregates with the intellectual disability through the maternal branch of the family. We also found a significant increase of mtDNA content in all the unaffected homo/heteroplasmic mutation carriers with respect to either affected or control subjects. Conclusion: This is the first case reporting the co-segregation of a mutation in MTRNR1 gene with a LHON primary mutation, which may be a risk factor of the extraocular signs complicating LHON phenotype. In addition, the data herein reported, confirmed that the key factor modulating the penetrance of optic atrophy in the family is the amount of mtDNA

The Telecardiology Revolution: From Emergency Management to Daily Clinical Practice

Aims: Telecardiology is one of the most widespread applications of telemedicine. We aimed to report the design and development of a telecardiology system in the sanitary district of Cosenza, one of the largest in Italy, with a complex orography, and healthcare reorganization needs, for the management of the emergency network and daily clinical practice. Methods: Our telecardiology network connects 8 hospitals, 9 first aid centers, 20 local 118-EMS stations, 1 helicopter station, 8 hospital emergency departments, 59 hospital departments, and 3 catheterization laboratories. All data are centralized on a dedicated server, accessible from any location for real-time assessment. The quality, source, and timing of the electrocardiograms transmitted were evaluated. Results: From October 2015 to December 2019, a total of 389,970 ECGs were transmitted. The quality of ECGs was optimal in 52%, acceptable in 42%, and poor in 6% of the cases. The number of poor-quality ECGs was only 3% in the last 2 years. Out of the total, 145,097 (37.2%) were transmitted from the emergency departments and 5318 (1.4%) from the 118-EMS. Of interest, a sizable part of the ECG was related to routine clinical practice, comprising 110,556 (28.3%) from the cardiology department and 79,256 (20.3%) from other noncardiovascular departments. Finally, the average reporting time was significantly decreased compared to reporting times without a telecardiology system (5–10 vs. 45–90 min). Conclusion: Our telecardiology system provides efficient cardiology assistance for all types, settings, and phases of cardiovascular diseases

The Telecardiology Revolution: From Emergency Management to Daily Clinical Practice

Aims: Telecardiology is one of the most widespread applications of telemedicine. We aimed to report the design and development of a telecardiology system in the sanitary district of Cosenza, one of the largest in Italy, with a complex orography, and healthcare reorganization needs, for the management of the emergency network and daily clinical practice. Methods: Our telecardiology network connects 8 hospitals, 9 first aid centers, 20 local 118-EMS stations, 1 helicopter station, 8 hospital emergency departments, 59 hospital departments, and 3 catheterization laboratories. All data are centralized on a dedicated server, accessible from any location for real-time assessment. The quality, source, and timing of the electrocardiograms transmitted were evaluated. Results: From October 2015 to December 2019, a total of 389,970 ECGs were transmitted. The quality of ECGs was optimal in 52%, acceptable in 42%, and poor in 6% of the cases. The number of poor-quality ECGs was only 3% in the last 2 years. Out of the total, 145,097 (37.2%) were transmitted from the emergency departments and 5318 (1.4%) from the 118-EMS. Of interest, a sizable part of the ECG was related to routine clinical practice, comprising 110,556 (28.3%) from the cardiology department and 79,256 (20.3%) from other noncardiovascular departments. Finally, the average reporting time was significantly decreased compared to reporting times without a telecardiology system (5–10 vs. 45–90 min). Conclusion: Our telecardiology system provides efficient cardiology assistance for all types, settings, and phases of cardiovascular diseases

Impact of COVID-19 Pandemic on Remote Monitoring of Cardiac Implantable Electronic Devices in Italy: Results of a Survey Promoted by AIAC (Italian Association of Arrhythmology and Cardiac Pacing)

The COVID-19 pandemic has had a profound impact on the organisation of health care in Italy, with an acceleration in the development of telemedicine. To assess the impact of the COVID-19 pandemic on the spread of remote monitoring (RM) of cardiac implantable electronic devices (CIEDs) in Italy, a survey addressed to cardiologists operating in all Italian CIED-implanting centres was launched. A total of 127 cardiologists from 116 Italian arrhythmia centres took part in the survey, 41.0% of all 283 CIED-implanting centres operating in Italy in 2019. All participating centres declared to use RM of CIEDs. COVID-19 pandemic resulted in an increase in the use of RM in 83 (71.6%) participating centres. In a temporal perspective, an increase in the median number of patients per centre followed up by RM was found from 2012 to 2017, followed by an exponential increase from 2017 to 2020. In 36 participating centres (31.0%) a telehealth visits service was activated as a replacement for in-person outpatient visits (in patients with or without CIED) during the COVID-19 pandemic. COVID-19 pandemic has caused an acceleration in the use of RM of CIEDs and in the use of telemedicine in the clinical practice of cardiology

Additional file 1: of Leber’s hereditary optic neuropathy, intellectual disability and epilepsy presenting with variable penetrance associated to the m.3460G >A mutation and a heteroplasmic expansion of the microsatellite in MTRNR1 gene – case report

Prioritization of mtDNA variants by MToolBox in LHON proband. All potentially deleterious mutations not contributing to the macro-haplogroup definition and, if non-synonymous, predicted as disease-associated by at least one of the pathogenicity prediction methods are reported as prioritized. Nt, nucleotide; AA, amino acid; dbSNP, single nucleotide polymorphism database. (DOC 110 kb

Implantation technique and optimal subcutaneous defibrillator chest position: a PRAETORIAN score-based study

Aims The traditional technique for subcutaneous implantable cardioverter-defibrillator (S-ICD) implantation involves three incisions and a subcutaneous pocket. Recently, a two-incision and intermuscular (IM) technique has been adopted. The PRAETORIAN score is a chest radiograph-based tool that predicts S-ICD conversion testing. We assessed whether the S-ICD implantation technique affects optimal position of the defibrillation system according to the PRAETORIAN score.Methods and results We analysed consecutive patients undergoing S-ICD implantation. The chi(2) test and regression analysis were used to :ults determine the association between the PRAETORIAN score and implantation technique. Two hundred and thir- teen patients were enrolled. The S-ICD generator was positioned in an IM pocket in 174 patients (81.7%) and the two-incision approach was adopted in 199 (93.4%). According to the PRAETORIAN score, the risk of conversion failure was classified as tow in 198 patients (93.0%), intermediate in 13 (6.1%), and high in 2 (0.9%). Patients undergoing the two-incision and IM technique were more likely to have a tow (<90) PRAETORIAN score than those undergoing the three-incision and subcutaneous technique (two-incision: 94.0% vs. three-incision: 78.6%; P = 0.004 and IM: 96.0% vs. subcutaneous: 79.5%; P = 0.001). Intermuscular plus two-incision technique was associated with a low-risk PRAETORIAN score (hazard ratio 3.76; 95% confidence interval 1.01-14.02; P= 0.04). Shock impedance was tower in PRAETORIAN low-risk patients than in intermediate-/high-risk categories (66 vs. 96 Ohm; P = 0.001). The PRAETORIAN score did not predict shock failure at 65 J.Conclusion In this cohort of S-ICD recipients, combining the two-incision technique and IM generator implantation yielded the lowest PRAETORIAN score values, indicating optimal defibrillation system position