Antiarrhythmic therapy for narrow QRS supraventricular tachyarrhythmias in newborns and infants in the first year of life: Potent tools to be handled with care.

Supraventricular tachyarrhythmias pose a significant challenge in neonates and infants, particularly within the first year of life, where prompt and effective management is crucial. By synthesizing available evidence and clinical experience, this review aims to provide a comprehensive overview of antiarrhythmic therapy in this vulnerable population, with a focus on narrow QRS supraventricular tachyarrhythmias. This review examines the current understanding of supraventricular tachyarrhythmia management and discusses the challenges associated with antiarrhythmic therapy in newborns and infants during the critical first year of life, evaluating the efficacy and safety of various antiarrhythmic agents commonly utilized in this population, including dosing considerations, adverse effects, and strategies for acute management and prophylactic long-term antiarrhythmic treatment.

Clinical presentation and genetic characterization of early-onset atrial fibrillation in patients affected by long QT syndrome: A single-center experience.

INTRODUCTION: Early-onset atrial fibrillation (AF) has already been observed in approximately 2% of patients with genetically proven long QT syndrome (LQTS). This frequency is higher than population-based estimates of early-onset AF. However, the concomitant expression of AF in LQTS is likely underestimated. The purpose of this study was to examine the clinical presentation, genetic background, and outcomes of a cohort of patients with LQTS and early-onset AF referred to a single tertiary center. METHODS: Twenty-seven patients diagnosed with congenital LQTS were included in the study based on the documentation of early-onset (age ≤50 years) clinical or subclinical AF episodes in all available medical records, including standard electrocardiograms, wearable monitor or cardiac implantable electronic devices. RESULTS: Seventeen patients experienced clinical AF during the follow-up period. Subclinical AF was detected in 10 patients through insertable or wearable cardiac monitors. In our series, the mean heart rate during AF episodes was found to be relatively low despite the patients' young age and the low or minimal effective doses of beta-blockers used for QTc interval control. All patients exhibiting LQTS and early-onset AF were genotype positive, carrying mutations in the KCNQ1 (66%), KCNH2, KCNE1, and SCN5A genes. Notably, most of these patients carried the same p.(R231C) mutation in the KCNQ1 gene (59%) and were from the same families, suggesting concurrent expression of familial AF and LQTS. CONCLUSION: LQTS patients are prone to developing clinical and subclinical AF, even at a younger age. The occurrence of early-onset AF in the LQTS population could be more frequent than previously assumed. AF should be considered as a potential dysrhythmia related to LQTS. Our study emphasizes the importance of carefully researching clinical and/or subclinical episodes of AF through strict heart rhythm monitoring in the LQTS population.

Exercise-induced type 1 Brugada pattern in a child.

The exercise stress testing may unmask the type 1 Brugada pattern on the surface electrocardiogram in a portion of patients with Brugada syndrome. The occurrence of the type 1 Brugada pattern during an exercise test in pediatric patients is not common. Consequently, the diagnostic yield of the exercise test in this population is still to be explored. We present a case of exercise-induced type 1 Brugada pattern in a 12-year-old child with episodes of palpitations and discuss the available evidence on the role of the exercise stress test in the diagnosis and risk stratification of patients with Brugada syndrome.

Right ventricular phenotyping in incident patients with idiopathic pulmonary arterial hypertension.

BACKGROUND: Right ventricular (RV) imaging has not a definite role in risk stratification of pulmonary arterial hypertension (PAH) patients. We tested the hypothesis that echocardiography-derived phenotypes, depicting different degrees of RV remodeling and dysfunction, may provide additional prognostic information to current risk stratification tools. METHODS: Consecutive incident PAH patients aged ≥18 years, diagnosed between January 2005 and December 2021, underwent clinical assessment, right heart catheterization, standard echocardiography. Simple echocardiographic variables were combined in order to define a priori four phenotypes representing different degrees of RV dilatation and RV-pulmonary arterial (PA) coupling: Phenotype 1 with mildy dilated right ventricle and preserved RV-PA coupling (n = 152 patients); phenotype 2 with mildly dilated right ventricle and poor RV-PA coupling (n = 143 patients); phenotype 3 with severely dilated right ventricle and preserved RV-PA coupling (n = 201 patients); phenotype 4 with severely dilated right ventricle and poor RV-PA coupling, with or without severe tricuspid regurgitation (n = 519 patients). Risk stratification was based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) 3-strata model and Registry to Evaluate Early and Long-Term PAH disease Management (REVEAL) 2.0 score. RESULTS: These phenotypes were present in all risk groups. Notably, regardless of the ESC/ERS risk stratum assigned to the patient, phenotype 4 was associated with a 2-fold increase of the odds of death (HR 2.1, 95% CI 1.6-2.8, p < 0.001), while phenotype 1 was associated with a 71% reduction in the odds of dying (HR 0.29, 95% CI 0.18-0.47, p < 0.001). CONCLUSIONS: Echocardiography-derived phenotypes describing RV remodeling and dysfunction may provide prognostic information which is independent of and additional to the clinically defined risk in incident PAH patients.

Right heart failure as a cause of pulmonary congestion in pulmonary arterial hypertension.

AIMS: Recent studies have shown that lung ultrasound-assessed pulmonary congestion is worse in heart failure when pulmonary vascular resistance (PVR) is increased, suggesting a paradoxical relationship between right heart failure and increased lung water content. Accordingly, we wondered if lung ultrasound would reveal otherwise clinically silent pulmonary congestion in patients with pulmonary arterial hypertension (PAH). METHODS AND RESULTS: All patients referred for suspicion of PAH in a tertiary centre from January 2020 to December 2022 underwent a complete diagnostic work-up including echocardiography, lung ultrasound and right heart catheterization. Pulmonary congestion was identified by lung ultrasound B-lines using an 8-site scan. The study enrolled 102 patients with idiopathic PAH (mean age 53 ± 13 years; 71% female). World Health Organization functional classes I, II, and III were found in 2%, 52%, and 46% of them, respectively. N-terminal pro-brain natriuretic peptide (NT-proBNP) was 377 pg/ml (interquartile range [IQR] 218-906). B-lines were identified in 77 out of 102 patients (75%), with a median of 3 [IQR 1-5]. At univariable analysis, B-lines were positively correlated with male sex, age, NT-proBNP, systolic pulmonary artery pressure (sPAP), right atrial pressure (RAP), PVR, left ventricular end-diastolic volume and tricuspid annular plane systolic excursion (TAPSE), and negatively with cardiac output and stroke volume. At multivariable analysis, RAP (p < 0.001), TAPSE/sPAP (p = 0.001), and NT-proBNP (p = 0.04) were independent predictors of B-lines. CONCLUSION: Lung ultrasound commonly discloses pulmonary congestion in PAH. This finding is related to right ventricular to pulmonary artery uncoupling, and may tentatively be explained by increased central venous pressure impeding lymphatic outflow.

Single-Chamber and Dual-Chamber Pacemaker Devices in Adults with Moderate and Complex Congenital Heart Disease: A Single Tertiary Referral Center Experience.

The number of device implantation procedures has increased in adult patients with congenital heart disease (ACHD). Despite significant improvements in materials and implantation techniques, these patients are exposed to higher risk of device related complications than general population. Herein, we describe our single tertiary referral center experience on transvenous pacemaker (PM) implantation and follow-up in adult patients with moderate and complex congenital heart disease (CHD) as limited data are available on long-term outcome. We considered all adults with moderate and complex CHD aged more than 16 years who underwent transvenous single-chamber and dual-chamber PM implant for sinus node dysfunction or atrioventricular block between January 2013 to December 2022 at our Unit. Seventy-one ACHD patients were included in the study (mean age 38.6 ± 15.2 years, 64% with moderate CHD, 36% with complex CHD). Among 32 patients implanted with a dual chamber PM (DDD PM), 4 devices were reprogrammed in VDD mode, 3 in VVI and 2 in AAI mode during follow-up because of lead dysfunction or permanent atrial arrhythmia. In addition, 26 patients had a single chamber PM (AAI or VVI PM) and 13 patients had single-lead pacing system with a free-floating atrial electrode pair (VDD PM). Just one of 13 single-lead VDD PM was reprogrammed in VVI mode due to a low atrial sensing. In DDD PM group, 10 re-interventions were needed due to lead dysfunction (8 cases) and lead-related infective endocarditis (2 cases). Only 3 patients in the single-lead PM group developed lead dysfunction with 2 re-interventions needed, but no infective endocarditis was reported. The rate of long-term complications is high in moderate and complex ACHD with transvenous PM devices, and it is mainly lead-related. In our experience, the less leads implanted, the less complications will occur. Considering the heterogeneity of the ACHD population, transvenous single-chamber or dual-chamber PM device implantation should always be tailored on the single patient, balancing risks and benefits in this complex population.

Long-term clinical outcomes of patients with drug-induced type 1 Brugada electrocardiographic pattern: A nationwide cohort registry study.

BACKGROUND: There are limited real-world data on the extended prognosis of patients with drug-induced type 1 Brugada electrocardiogram (ECG). OBJECTIVE: We assessed the clinical outcomes and predictors of life-threatening arrhythmias in patients with drug-induced type 1 Brugada ECG. METHODS: This multicenter retrospective study, conducted at 21 Italian and Swiss hospitals from July 1997 to May 2021, included consecutive patients with drug-induced type 1 ECG. The primary outcome, a composite of appropriate ICD therapies and sudden cardiac death, was assessed along with the clinical predictors of these events. RESULTS: A total of 606 patients (mean age 49.7 ± 14.7 years; 423 [69.8%] men) were followed for a median of 60.3 months (interquartile range 23.0-122.4 months). Nineteen patients (3.1%) experienced life-threatening arrhythmias, with a median annual event rate of 0.5% over 5 years and 0.25% over 10 years. The SCN5A mutation was the only predictor of the primary outcome (hazard ratio 4.54; P = .002), whereas a trend was observed for unexplained syncope (hazard ratio 3.85; P = .05). In patients who were asymptomatic at presentation, the median annual rate of life-threatening arrhythmias is 0.24% over 5 years and increases to 1.2% if they have inducible ventricular fibrillation during programmed ventricular stimulation. CONCLUSION: In patients with drug-induced type 1 Brugada ECG, the annual risk of life-threatening arrhythmias is low, with the SCN5A mutation as the only independent predictor. Unexplained syncope correlated with worse clinical outcomes. Ventricular fibrillation inducibility at programmed ventricular stimulation significantly increases the median annual rate of life-threatening arrhythmias from 0.24% to 1.2% over 5 years.

Contact allergy to subcutaneous implantable cardioverter defibrillator in a child with Brugada syndrome.

Allergic reactions to components of cardiac implantable electronic devices are rare and often go undiagnosed, which can lead to a misdiagnosis of device infection. Contact allergy to subcutaneous implantable cardioverter defibrillator (S-ICD) is extremely rare. In this report, we present a case of cobalt-related contact allergy in a pediatric patient with Brugada syndrome who underwent S-ICD implantation.

Combined subcutaneous implantable cardioverter defibrillator and pacemaker devices in complex congenital heart disease: a single-center experienced based study.

BACKGROUND: Subcutaneous implantable cardioverter defibrillators (S-ICD) are widely accepted therapy in congenital heart disease (CHD) patients at risk of life-threatening ventricular arrhythmias or sudden cardiac death (SCD) when pacing is not required. Occasionally, pacemaker (PM)-dependent CHD patients will subsequently develop an indication for a cardioverter defibrillator. The use of S-ICD in complex CHD patients who have had already PM devices implanted implies some specific considerations, as the safety for these patients in unknown and recommendations among physicians may vary widely. METHODS: We review the data and studied the indications for S-ICD in complex CHD with previous PM and discuss its usefulness in clinical practice. RESULTS: From a large cohort of 345 patients enrolled in the S-ICD Monaldi care registry, which encompass all the patients implanted in the Monaldi Hospital of Naples, we considered 11 consecutive complex CHD patients (10M/1F aged 40.4 ±18.4 years) who underwent S-ICD implant after a previous PM implant, from February 2015 to October 2022. Mean follow-up was 25.5 ± 22 months. All the patients showed a good compliance to the device system with no complications (infections or skin erosions). CONCLUSIONS: In complex CHD with already implanted PM devices, S-ICD implant appears to be a safe alternative to PM upgrading to transvenous ICD system, avoiding abandoned leads or life-threatening lead extraction. However, there are important issues with regard to testing and programming that need to be addressed at the time of implantation.

Contact force sensing radiofrequency catheter ablation guided by electroanatomic mapping in pediatric patients with permanent junctional reciprocating tachycardia: A case series.

Limited data are available on the use of irrigated contact force (CF) catheters for radiofrequency (RF) ablation of permanent junctional reciprocating tachycardia (PJRT) in children. We considered five consecutive pediatric patients with diagnosis of PJRT who underwent RF ablation with irrigated CF catheter guided by electroanatomic mapping, obtaining a low number of RF pulses and minimal fluoroscopy exposure. High CF values are not necessary to obtain effective RF lesions and successful ablation.

Lung Ultrasound, Echocardiography, and Fluid Challenge for the Differential Diagnosis of Pulmonary Hypertension.

OBJECTIVES: The differential diagnosis between pulmonary arterial hypertension (PAH) and postcapillary pulmonary hypertension (PH) in heart failure with preserved ejection fraction (HFpEF) is sometimes difficult despite guidelines-derived standardized step-by-step diagnostic algorithms. We therefore explored the added value of lung ultrasound to a previously validated echocardiographic score of right heart catheterization measurements. METHODS: Patients referred for PH underwent a right heart catheterization, echocardiography, and lung ultrasound before and after rapid infusion of 7 mL/kg of saline. A 7-point echocardiographic score based on cardiac chamber dimensions and estimates of filling pressures was implemented for the prediction of precapillary PH. Pulmonary congestion was identified by lung ultrasound B lines. RESULTS: The study enrolled 70 patients with PAH and 77 patients with HFpEF. The PAH patients had a higher echocardiographic score (3.5 ± 1.8 vs 1.6 ± 1.5; P < .001). The HFpEF patients had more B lines both before (8.1 ± 4.2 vs 5.1 ± 3.0; P < .001) and after fluid challenge (14.6 ± 5.4 vs 7.6 ± 3.5; P < .001) and a more important increase (Δ) of B lines after fluid challenge (6.5 ± 2.9 vs 2.5 ± 1.6; P < .001). The sensitivity and specificity of the echocardiographic score (cutoff ≥2) alone for PAH were 0.91 and 0.49, respectively (area under the curve of 0.78). The best diagnostic improvement was observed with addition of ΔB lines + E/e' post-fluid challenge to the echocardiographic score, with a significant increase of the area under the curve (0.98) and (with a cutoff given by the presence of echo score ≥2, ΔB lines <4 and E/e' post < 11) a sensitivity of 0.90 (95% CI, 0.83; 0.97) and specificity of 0.84 (95% CI, 0.76; 0.93). CONCLUSIONS: Lung ultrasound combined with echocardiography at baseline and after fluid challenge has an incremental value for the differential diagnosis between PAH and PH-HFpEF.

Triple Antiarrhythmic Therapy in Newborns with Refractory Atrioventricular Reentrant Tachycardia.

Atrioventricular reentrant tachycardia (AVRT) is the most common form of supraventricular tachycardia in newborns. AVRT is sometimes refractory to conventional antiarrhythmic therapy. We describe our experience about the use of the triple combination of flecainide + propranolol + amiodarone as third-line regimen for refractory and recurrent AVRT in newborns. We considered a series of 14 patients who had failed both first-line and second-line therapy and were treated using the combination of flecainide + propranolol + amiodarone. Transoesophageal electrophysiologic study (TES) was performed to test the effectiveness of medical therapy during hospitalization and to try to reduce the amount of therapy, after amiodarone wash-out, before 1 year of age. TES was repeated at 1 year of age to test the spontaneous resolution of the arrhythmia after treatment discontinuation. Rhythm control was achieved in all 14 patients. At a mean age of 9.3 ± 2 months, AVRT was not inducible by TES in 11/12 amiodarone-free patients. At a mean age of 14.1 ± 3 months, AVRT was still inducible in 7/12 patients after interrupting the entire antiarrhythmic therapy (58.3%). Triple combination was effective as third-line option to suppress AVRT refractory to single and double antiarrhythmic therapy, with no significant adverse events. Our experience suggests that triple therapy could be maintained for a short-term treatment, discontinuing amiodarone before 1 year of age to avoid long-term side effects. Newborns who needed triple therapy appear to have a lower chance of accessory pathway disappearance at 1 year of age. TES could be useful for risk stratification of recurrences at the time of drug discontinuation in infants considered to be at higher risk of recurrent AVRT.

Alert-LDL-2: adherence to guidelines and goals attainment in the treatment of diabetic patients with dyslipidemia.

BACKGROUND: Type 2 diabetes mellitus is associated with a cluster of lipid and apolipoprotein abnormalities which increase the risk for atherosclerotic cardiovascular disease. The aim of this study was to evaluate the adherence to guidelines-oriented dyslipidemia treatment in diabetic patients and to assess the efficacy of a territorial goal-oriented program. METHODS: One thousand seventy-one diabetic patients at very high cardiovascular risk were enrolled in this prospective study. They performed a clinical-laboratory follow-up program, received lifestyle recommendations and optimization of their antihyperlipidemic therapies. At the beginning and the 3-month follow-up visit, LDL-c data were collected, and further therapies were prescribed to the patients that did not reach the target. After 12 month follow-up, LDL-c data were collected again. RESULTS: Diabetic patients significantly improved mean LDL cholesterol levels during one-year follow-up (LDLc mean value 135 mg/dL at baseline, 60 mg/dL at the end of the study), obtaining a greater reduction compared to non-diabetic patients participating in the same program. Accordingly, the percentage of patients that reached the lipid target was significantly higher in diabetic patients after 3-months and 12- follow-ups (P<0.05). Diabetic patients assuming statins, both in monotherapy and in combination with ezetimibe, increased during the follow-up (74.1% at the enrolment vs. 88.2% one year later). GLP1ra-treated patients achieved the greatest reduction in cholesterol levels compared to baseline. CONCLUSIONS: The results of the study recommend encouraging strategies and appropriate treatments to achieve a targeted lipid profile in diabetic patients at very high cardiovascular risk.

Implantable Loop Recorder with Long Sensing Vector: Safety, Acceptability, and Sensing Performance in Pediatric Patients.

Implantable loop recorders (ILRs) are effective tools for detecting arrhythmias by long-term continuous heart rhythm monitoring. Benefits have been demonstrated even in pediatric patients. ILR with a long sensing vector has recently been designed to improve signal quality in terms of P wave visibility and R wave amplitude. However, there are no data on its use in pediatric patients. We considered a series of pediatric patients implanted with a long sensing vector ILR. Sensing performance, including R wave amplitude and P wave visibility, device-related complications, and diagnostic yield were collected. During follow-up, each patient guided by his/her parents/guardians was also asked to complete a brief questionnaire to assess patient acceptability of the device. Twenty-five consecutive pediatric patients (mean age 11.3 ± 3.5 years, 72% male) were enrolled. The insertion success rate was 100% on the first attempt with no complications. The median amplitude of the R wave was 1.15 mV (interquartile range, 1.01-1.42) with no significant differences between patients aged ≤ or > 10 years (p = 0.726) and between female and male (p = 0.483). P wave was classified as 'always visible' in 24/25 patients (96%). ILR was generally well accepted and tolerated by all involved patients. During a median follow-up of 297 days (117-317), we achieved in 5 patients a correlation between symptoms and rhythm disorders (20%) and ruled out significant arrhythmias in 6 symptomatic children (24%). Long sensing vector ILR showed to be well accepted, with good signal quality and an excellent safety profile even in pediatric patients.

Association Between Circulating CD4+ T Cell Methylation Signatures of Network-Oriented SOCS3 Gene and Hemodynamics in Patients Suffering Pulmonary Arterial Hypertension.

Pathogenic DNA methylation changes may be involved in pulmonary arterial hypertension (PAH) onset and its progression, but there is no data on potential associations with patient-derived hemodynamic parameters. The reduced representation bisulfite sequencing (RRBS) platform identified N = 631 differentially methylated CpG sites which annotated to N = 408 genes (DMGs) in circulating CD4+ T cells isolated from PAH patients vs. healthy controls (CTRLs). A promoter-restricted network analysis established the PAH subnetwork that included 5 hub DMGs (SOCS3, GNAS, ITGAL, NCOR2, NFIC) and 5 non-hub DMGs (NR4A2, GRM2, PGK1, STMN1, LIMS2). The functional analysis revealed that the SOCS3 gene was the most recurrent among the top ten significant pathways enriching the PAH subnetwork, including the growth hormone receptor and the interleukin-6 signaling. Correlation analysis showed that the promoter methylation levels of each network-oriented DMG were associated individually with hemodynamic parameters. In particular, SOCS3 hypomethylation was negatively associated with right atrial pressure (RAP) and positively associated with cardiac index (CI) (|r|≥ 0.6). A significant upregulation of the SOCS3, ITGAL, NFIC, NCOR2, and PGK1 mRNA levels (qRT-PCR) in peripheral blood mononuclear cells from PAH patients vs. CTRLs was found (P ≤ 0.05). By immunoblotting, a significant upregulation of the SOCS3 protein was confirmed in PAH patients vs. CTRLs (P < 0.01). This is the first network-oriented study which integrates circulating CD4+ T cell DNA methylation signatures, hemodynamic parameters, and validation experiments in PAH patients at first diagnosis or early follow-up. Our data suggests that SOCS3 gene might be involved in PAH pathogenesis and serve as potential prognostic biomarker.

Relevance of comorbidities on initial combination therapy in pulmonary arterial hypertension.

Rationale: Demographic characteristics of pulmonary arterial hypertension (PAH) patients have changed over time, but the effects of cardiovascular risk factors on risk status and pulmonary vascular resistance (PVR) reduction with initial oral combination therapy are not known. Therefore, we tested the relevance of cardiovascular comorbidities in this setting. Methods: The study enrolled 181 treatment-naive PAH patients with a 6-month (IQR 144-363 days) right heart catheterisation and risk assessment after initial oral combination therapy. Results: Group A included 96 (53.0%) patients without cardiac comorbidities; Group B included 54 (29.8%) patients with one cardiac comorbidity; Group C included 31 (17.1%) patients with two cardiac comorbidities or more. Group C patients were older with a balanced sex distribution. There was a significant difference in PVR reduction moving from the absence to one or at least two cardiac comorbidities, respectively: median -45.0%, -30.3%, -24.3%. A European Respiratory Society/European Society of Cardiology low-risk status was present at first follow-up in 50 (52.0%) patients in Group A, 19 (35.1%) in Group B and 9 (29.0%) in Group C; a REVEAL 2.0 low-risk status was present at first follow-up in 41 (42.0%) patients in Group A, 15 (27.7%) in Group B and 7 (22.6%) in Group C. Group A patients were 2.3 times more likely to achieve/maintain a low-risk status compared with Group B and C (OR 2.27, 95% CI 1.15-4.54, p=0.02). No significant difference was observed between patients with non-cardiac comorbidities and those without comorbidities. Conclusion: Initial oral combination therapy seems associated with a less effective response for patients with cardiovascular comorbidities compared with the others, related to the magnitude of treatment-induced decrease in PVR.

Subcutaneous finger cardioverter-defibrillator in low weight paediatric patients: a case series.

Placement of traditional transvenous implantable cardioverter defibrillator (ICD) system in low-weight children is often difficult because of their vessel size, the elevated risk of lead malfunction and failure, children's growth and various anatomic constraints, creating the need for alternative solutions. Subcutaneous array leads combined with an abdominally placed ICD device can minimize the surgical approach. In this case series, we analyse the data behind indications for subcutaneous finger cardioverter defibrillator (SFCD) and discuss the preliminary clinical experience in low-weight children. We considered 4 consecutive children (mean age 3.9 years, range 3-5.5 years, mean body weight 17.6 Kg, range 14-23 Kg) who underwent SFCD implant from April 2016 to August 2020. All patients showed a good compliance to the device system with no complications (infections or skin erosions). No patients experienced in the observation period (mean time 44.5±21.5 months) sustained ventricular arrhythmias requiring shocks. No inappropriate shocks released by the device occurred. No significant changes were observed in LET (lowest energy tested) performed around 24 months of follow-up. All patients showed a good compliance and stable atrio-ventricular sensing and pacing thresholds. In smaller children in whom a transvenous approach is not feasible or not possible for anatomic reasons, the SFCD appears to be a safe method to prevent SCD with little surgical trauma and preservation of an intact vascular system, providing an adequate bridge to transvenous ICD or subcutaneous ICD implant late in the life.

Wearable cardioverter-defibrillator (life-vest): A feasible bridging treatment in adult congenital heart disease.

BACKGROUND: Wearable cardioverter-defibrillators (WCDs) are currently used in patients at temporarily heightened risk for sudden cardiac death (SCD) who are temporarily unable to receive an implantable cardioverter-defibrillator (ICD). WCD can safely record and terminate life-threatening arrhythmias through a non-invasive electrode-based system. The current clinical indications for WCD use are varied and keep evolving as experience with this technology increases. METHODS: We reviewed and explored the data behind indications for WCD use and discuss its usefulness in congenital heart disease (CHD) patients. RESULTS: We considered 8 consecutive patients (mean age 35.25 years, range 18-51 years, average duration of WCD use 4 months, range 3-6 months) with complex CHD, in which a WCD was used between June 2018 and January 2022. No sustained ventricular arrhythmias requiring shocks were recorded in the observation period. No inappropriate shocks were recorded. All the patients showed a good compliance and a very high mean wear time per day (21.2 ± 1 h a day). Four patients implanted a permanent device (3 CRT-D, 1 ICD), three underwent cardiac surgery at the end of the WCD period and one is still on the waiting list for the operation. CONCLUSIONS: Larger trial could confirm the possible conceivable benefit from an extended use of the WCD in certain populations with complex CHD as in our case series, especially in patients with life-treating ventricular arrhythmias waiting for surgery for residual cardiac defects or in the early phases following the surgical/hemodynamic interventions, patients with tachycardiomyopathy expected to improve after the arrhythmias are removed and patients awaiting implantation of an ICD at high risk due to active infection.

The paradox of pulmonary arterial hypertension in Italy in the COVID-19 era: is risk of disease progression around the corner?

OBJECTIVE: The coronavirus disease 2019 (COVID-19) outbreak has led to significant restrictions on routine medical care. We conducted a multicentre nationwide survey of patients with pulmonary arterial hypertension (PAH) to determine the consequences of governance measures on PAH management and risk of poor outcome in patients with COVID-19. MATERIALS AND METHODS: The present study, which included 25 Italian centres, considered demographic data, the number of in-person visits, 6-min walk and echocardiographic test results, brain natriuretic peptide/N-terminal pro-brain natriuretic peptide test results, World Health Organization functional class assessment, presence of elective and non-elective hospitalisation, need for treatment escalation/initiation, newly diagnosed PAH, incidence of COVID-19 and mortality rates. Data were collected, double-checked and tracked by institutional records between March 1 and May 1, 2020, to coincide with the first peak of COVID-19 and compared with the same time period in 2019. RESULTS: Among 1922 PAH patients, the incidences of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and COVID-19 were 1.0% and 0.46%, respectively, with the latter comparable to that in the overall Italian population (0.34%) but associated with 100% mortality. Less systematic activities were converted into more effective remote interfacing between clinicians and PAH patients, resulting in lower rates of hospitalisation (1.2% versus 1.9%) and related death (0.3% versus 0.5%) compared with 2019 (p<0.001). A high level of attention is needed to avoid the potential risk of disease progression related to less aggressive escalation of treatment and the reduction in new PAH diagnoses compared with 2019. CONCLUSION: A cohesive partnership between healthcare providers and regional public health officials is needed to prioritise PAH patients for remote monitoring by dedicated tools.