Search Results (48)

Background: Among patients with congenital heart disease, particularly those with a history of undergoing the Fontan operation, pregnancy presents a significant maternal–fetal risk, especially when complicated by severe valvular dysfunction. Lung reperfusion syndrome (LRS) is a rare but life-threatening complication occurring following valve intervention. Multidisciplinary management, including by Cardio-Obstetrics teams, is essential for optimizing outcomes in such high-risk cases. Methods: We present the case of a 37-year-old pregnant patient with previously repaired tetralogy of Fallot (via the Fontan procedure) who presented at 24 weeks gestation with worsening severe pulmonary stenosis and right-ventricular dysfunction. The patient had been lost to cardiac follow-up for over a decade. She experienced recurrent arrhythmias, including supraventricular and non-sustained ventricular tachycardia, prompting hospital admission. A multidisciplinary team recommended transcatheter pulmonic valve replacement (TPVR), performed at 28 weeks’ gestation. Results: Post-TPVR, the patient developed acute hypoxia and hypotension, consistent with Lung Reperfusion Syndrome, necessitating intensive cardiopulmonary support. Despite initial stabilization, progressive maternal respiratory failure and fetal compromise led to an emergent cesarean delivery. The neonate’s neonatal intensive care unit (NICU) course was complicated by spontaneous intestinal perforation, while the mother required intensive care unit (ICU)-level care and a bronchoscopy due to new pulmonary findings. She was extubated and discharged in stable condition on postoperative day five. Conclusions: This case underscores the complexity of managing severe congenital heart disease and valve pathology during pregnancy. Lung reperfusion syndrome should be recognized as a potential complication following TPVR, particularly in pregnant patients with Fontan physiology. Early involvement of a multidisciplinary Cardio-Obstetrics team and structured peripartum planning are critical to improving both maternal and neonatal outcomes. Full article

Background: Patients with arrhythmias, particularly paroxysmal supraventricular tachycardia (PSVT), face an increased risk of cardiac complications. Currently, non-parenteral medications for rapid PSVT cessation are lacking. Etripamil, a novel intranasal, short-acting calcium channel blocker, offers a rapid onset and the potential for unsupervised PSVT management. However, data on its use in arrhythmia management remain limited. Aims: We aimed to assess the efficacy and safety of 70 mg of etripamil compared with placebo in the treatment of PSVT. Methods: We systematically searched PubMed, Embase, Web of Science, Scopus, and the Cochrane Library for randomized controlled trials (RCTs) from inception to April 2025. We calculated pooled risk ratios (RRs) with 95% confidence intervals (CIs) using random or common effects models, depending on the heterogeneity. Results: Four RCTs including 540 patients were analyzed. Etripamil demonstrated higher conversion rates to the sinus rhythm at 15 min (RR 1.84 [95% CI: 1.32–2.48]), 30 min (RR 1.80 [95% CI: 1.38–2.35]), and 60 min (RR 1.24 [95% CI: 1.04–1.48]). PSVT recurrence rates were similar between groups (RR 0.52 [95% CI: 0.20–1.34]). Adverse events (AEs) and severe AEs were comparable between etripamil and the placebo. Etripamil was associated with higher rates of nasal discomfort, nasal congestion, rhinorrhea, and epistaxis but not with increased bradyarrhythmia, atrial fibrillation, or non-sustained ventricular tachycardia. Conclusions: Etripamil appears to be a promising treatment for cardiac arrhythmias. Larger long-term RCTs are needed to confirm its safety and efficacy in clinical practice. Full article

Background: Apical hypertrophic cardiomyopathy (ApHCM) is a distinct variant of hypertrophic cardiomyopathy (HCM). Few studies have focused on the genetic determinants of this subtype. We aimed to investigate the genetic basis of apical hypertrophy in a Swedish cohort. Methods–Results: Longitudinal data on 58 unrelated index patients with ApHCM from the Southeast healthcare region in Sweden from 2010 to 2024 were assessed retrospectively. Additionally, the original raw data from genetic testing were re-evaluated using AI-based Emedgene software. Patients were 47 ± 14 years old, and 60% males. A total of 72.4% had the pure apical type and the remaining had the mixed phenotype, dominant distal. In the cohort, 50/58 (86.2%) underwent genetic testing, of whom 7/50 (14%) were considered genotype positive for a pathogenic/likely pathogenic variant, mainly in MYH7 (43%) and in the non-sarcomeric ALPK3 gene (28.6%). A re-evaluation of the original data from genetic testing identified a previously unreported variant in the skeletal muscle α-actin (ACTA1) gene. Overall, 21 of 58 patients (36.2%) had HCM-related events during their disease course: 10% had a stroke, and 12% had heart failure. Atrial fibrillation was present in 41.4% and non-sustained ventricular tachycardia occurred in 29.3% of the patients. Apical aneurysm was observed in 17.2% of cases. Patients with a positive genotype were more likely to have a positive family history of HCM compared to those with a negative genotype (p = 0.020). Conclusions: In ApHCM, a positive genotype was found less frequently compared to classic HCM. Only 14% of patients with ApHCM were found to be genotype positive, indicating that apical hypertrophy represents a genetically unique population with low risk of mortality. Nevertheless, patients with ApHCM faced higher rates of atrial fibrillation, ventricular arrhythmias, and apical aneurysms. Full article

Background and Objectives: Aortic stenosis (AS) is a frequent valvular disease characterized by the obstruction of left ventricular outflow. The resulting hemodynamic and structural changes create an arrhythmogenic substrate, with sudden cardiac death (SCD) often caused by ventricular arrhythmias (VAs) being a feared complication. This review examines the relationship between severe AS and VA, detailing the epidemiology, pathophysiological mechanisms, risk factors, and management approaches prior to aortic valve replacement (AVR). Materials and Methods: We conducted a comprehensive narrative review of the historical and contemporary literature investigating ventricular arrhythmias in severe aortic stenosis. Literature searches were performed in PubMed, MEDLINE, and Scopus databases using keywords, including “aortic stenosis”, “ventricular arrhythmia”, “sudden cardiac death”, and “aortic valve replacement”. Both landmark historical studies and modern investigations utilizing advanced monitoring techniques were included to provide a complete evolution of the understanding. Results: The prevalence of ventricular ectopy and non-sustained ventricular tachycardia increases with AS severity and symptom onset. Left ventricular hypertrophy, myocardial fibrosis, altered electrophysiological properties, and ischemia create the arrhythmogenic substrate. Risk factors include the male sex, concomitant aortic regurgitation, elevated filling pressures, and syncope. Diagnostic approaches range from standard electrocardiography to continuous monitoring and advanced imaging. Management centers on timely valve intervention, with medical therapy serving primarily as a bridge to AVR. Conclusions: Ventricular arrhythmias represent a consequence of valvular pathology in severe AS rather than an independent entity. Their presence signals advanced disease and a heightened risk for adverse outcomes. Multidisciplinary management with vigilant monitoring and prompt surgical referral is essential. Understanding this relationship enables clinicians to better identify high-risk patients requiring urgent intervention before life-threatening arrhythmic events occur. Full article

Introduction: Arrhythmias are a frequent complication of pulmonary hypertension (PH). Supraventricular tachycardias (SVT) are predominantly reported and are associated with clinical deterioration and an increased mortality. In contrast, the prevalence and clinical relevance of bradycardias is largely unclear. Therefore, the aim of the present study was to determine a prevalence of bradycardias in PH patients and to outline their clinical relevance. Material and methods: Between January 2000 and June 2013, consecutive PH patients were pro- and retrospectively enrolled in two cohorts. Patients received either a 24 h or 72 h Holter ECG. Results: A total of 314 patients (58% female, mean age: 63 years) from PH groups 1–5 (39%, 11%, 19%, 28%, 3%) were included. Basic heart rhythm was sinus rhythm in 87% of patients (9% atrial fibrillation, 2% atrial flutter and 2% paced rhythm). Further arrhythmias were detected in 34% of patients (SVT: 12%, non-sustained ventricular tachycardia: 16%) with a 6% prevalence of relevant bradycardias. Atrioventricular block was revealed in 5% of patients (seven first-degree, one and three second-degree Wenckebach and Mobitz type, respectively, four third-degree), and 1% revealed sinoatrial block (one second-degree, third-degree and unspecified each). Conclusions: The prevalence of bradycardias appears to be about 5–10% in PH patients. Most of them are short and self-limiting. However, some patients experience syncope or clinical deterioration and, therefore, need specific treatment. To find these patients, long-term ECG monitoring combined with ECG-symptom correlation may be useful. Bradycardic medication should be excluded as a cause. Full article

Multifocal ectopic Purkinje-related premature contractions (MEPPC) syndrome is a recently recognized rare form of arrhythmia involving the entire His–Purkinje system and often coinciding with dilated cardiomyopathy (DCM). Certain variants in the SCN5A gene may be linked to MEPPC syndrome. We present a case of a 32-year-old Caucasian female who exhibited a high burden of premature ventricular contractions (PVCs) and non-sustained episodes of ventricular tachycardia (NSVT) with an alternating QRS pattern, and who was resistant to traditional medical therapy and radiofrequency catheter ablation (RFCA), necessitating implantation of a cardioverter-defibrillator (ICD). A positive family history (father’s death at the age of 40 years) and the rapid deterioration of left ventricular function parameters echocardiographically during recurrent arrhythmic episodes raised concern about a potentially complex disease scenario. Genetic testing revealed a heterozygous variant of the SCN5A gene, c.2440C>T, p.(Arg814Trp), confirming the diagnosis of MEPPC syndrome. Treatment with a combination of class I antiarrhythmic drugs, flecainide and mexiletine, concomitant with beta blockers, led to symptomatic improvement, a reduction of PVCs (from 66 491 (44%) to 858 (1%)), and the restoration of left ventricular function (LV EF from 44% to 53%). A lack of defined diagnostic criteria hampers timely diagnosis, leading to ineffective interventions and delayed initiation of treatment with antiarrhythmic drugs. MEPPC patients remain at significant risk for severe heart failure and sudden cardiac death. Our clinical case report underscores the importance of accurate and timely diagnosis, which allows effective treatment with a combination of antiarrhythmic drugs and mitigates the risk associated with MEPPC syndrome. Full article

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by structural abnormalities, arrhythmias, and a spectrum of genetic and clinical manifestations. Clinically, ARVC is structurally distinguished by right ventricular dilation due to increased adiposity and fibrosis in the ventricular walls, and it manifests as cardiac arrhythmias ranging from non-sustained ventricular tachycardia to sudden cardiac death. Its prevalence has been estimated to range from 1 in every 1000 to 5000 people, with its large range being attributed to the variability in genetic penetrance from asymptomatic to significant burden. It is even suggested that the prevalence is underestimated, as the presence of genotypic mutations does not always lead to clinical manifestations that would facilitate diagnosis. Additionally, while set criteria have been in place since the 1990s, newer understanding of this condition and advancements in cardiac technology have prompted multiple revisions in the diagnostic criteria for ARVC. Novel discoveries of gene variants predisposing patients to ARVC have led to established screening techniques while providing insight into genetic counseling and management. This review aims to provide an overview of the genetics, pathophysiology, and clinical approach to ARVC. It will also focus on clinical presentation, ARVC diagnostic criteria, electrophysiological findings, including electrocardiogram characteristics, and imaging findings from cardiac MRI, 2D, and 3D echocardiogram. Current management options—including anti-arrhythmic medications, device indications, and ablation techniques—and the effectiveness of treatment will also be reviewed. Full article

Background: The wearable cardioverter defibrillator (WCD) has emerged as a valuable tool used for temporary protection from sudden cardiac death. However, since the WCD uses surface electrodes to detect arrhythmias, it is susceptible to inappropriate detection. Although shock conversion rates for the WCD are reported to be high for detected events, its efficacy in clinical practice tends to be degraded by patient noncompliance. Reasons for this include wearer discomfort and frequent false alarms, which may interrupt sleep and generate anxiety. Up to now, data on the incidence of false alarms emitted by the WCD and their predictors are rare. Objectives: The aim of our study was to assess the relationship between both artifact sensing and episode misclassification burden and wearing compliance in patients with a WCD (ZOLL LifeVest™ 4000 system, ZOLL CMS GmbH, Cologne, Germany). Methods and Results: We conducted a single-center retrospective observational study, analyzing patients with a WCD prescribed at our institution. A total of 134 patients (mean age 51.7 ± 13.8 years, 79.1% male) were included. Arrhythmia recordings were analyzed and categorized as non-sustained ventricular tachycardia, sustained ventricular tachycardia or fibrillation, artifact sensing or misclassified episodes. Indication for WCD prescription was both primary and secondary prophylaxis. A total of 3019 false WCD alarms were documented in 78 patients (average number of false alarms 38.7 ± 169.5 episodes per patient) over a mean WCD wearing time of 71.5 ± 70.9 days (daily WCD wearing time 20.2 ± 5.0 h). In a total of 78 patients (58.2% of the study population), either artifact sensing (76.9%), misclassified episodes (6.4%), or both (16.7%) occurred. Misclassified episodes included sinus tachycardias, atrial flutter, atrial fibrillation, premature ventricular contractions (PVCs), and intermittent bundle branch block. A multiple linear regression identified loop diuretics (regression coefficient [B] −0.11; 95% CI −0.21–(−0.0001); p = 0.049), angiotensin receptor–neprilysin inhibitors (ARNIs) (B −0.11; 95% CI 0.22–(−0.01); p = 0.033), and a higher R-amplitude of the WCD baseline electrocardiogram (ECG) (B −0.17; 95% CI −0.27–(−0.07); p = 0.001) as independent predictors for a lower number of artifact episodes per day. In addition, atrial fibrillation (B 0.05; 95% CI 0.01–0.08; p = 0.010), and calcium antagonists (B 0.07; 95% CI 0.02–0.12; p = 0.012) were independent predictors for increased numbers of misclassified episodes per day, while beta-blockers seemed to reduce them (B −0.06; 95% CI −0.10–(−0.01); p = 0.013). Patients terminated 61.0% of all false alarms manually by pressing the response button on average 1.9 times per false alarm with overall 3.6 manual terminations per affected patient per month. Conclusions: In conclusion, false alarms from the ZOLL LifeVest™ system were frequent, with artifact sensing being the most common cause. Hence, the occurrence of false alarms represents a significant side effect of WCD therapy, and efforts should be made to minimize false alarms. Full article

Background/Objectives: Troponin level elevation without an obstructive culprit lesion is caused by heterogenous entities. The effect of aging on this condition has been poorly investigated. Methods: After screening 24,775 patients between 2010 and 2021, this study included a total of 373 patients with elevated troponin levels without an obstructive culprit lesion or suspected myocardial infarction with non-obstructive coronary arteries (MINOCAs) categorized into four age groups containing 78 patients (<51 years), 72 patients (51–60 years), 81 patients (61–70 years), and 142 patients (>70 years). This study analyzed the baseline characteristics, the in-hospital complications, in-hospital mortality, and the long-term outcomes. Results: The older patients exhibited a higher rate of major adverse cardiovascular in-hospital events than those of the other age groups (15.4% in the <51-year-old group vs. 36.1% in the 51–60-year-old group vs. 33.3% in the 61–70-year-old group vs. 47.2% in the >70-year-old group; p < 0.001). However, the rate of non-sustained ventricular tachycardia (nsVT) was higher in the 51–60-year-old patients than those of the other age groups (5.6% in the 51–60-year-old group vs. 1.3% in the 61–70-year-old group vs. 0.7% in the >70-year-old group; p = 0.027). At the 11-year follow-up, cardiovascular mortality was higher among the older patients compared to that of the younger patients (3.9% in the 61–70-year-old group vs. 4.2% in the >70-year-old group, p = 0.042), while non-cardiovascular mortality was comparable between the age groups. Conclusions: The older patients with troponin level elevation without an obstructive culprit lesion experienced a higher incidence of major adverse cardiovascular events during hospitalization compared to that of the younger groups. Additionally, higher cardiovascular mortality rates were revealed in the older patients at a long-term follow-up. Full article

A narrative review was performed, analyzing peer-reviewed articles from databases such as PubMed, EMBASE, Scopus, and Web of Science to examine the mechanistic links between sleep-disordered breathing (SDB), and cardiac arrhythmias, emphasizing intermittent hypoxia, autonomic imbalance, and intrathoracic pressure swings as key pathways. Studies, including the DREAM and CESAAR trials, consistently demonstrate that SDB patients face elevated risks: more than doubling the likelihood of overall arrhythmias (OR 2.24; 95% CI 1.48–3.39), quadrupling the risk of AF (OR 4.02; 95% CI 1.03–15.74), and tripling the risk of non-sustained ventricular tachycardia (NSVT) with higher apnea-hypopnea index (AHI) values. Additionally, SDB doubles the risk of bradyarrhythmia, such as sinus pause, second and third-degree atrioventricular block, and intraventricular conduction delay (OR 2.50; 95% CI 1.58–3.95). According to meta-analytical findings, continuous positive airway pressure (CPAP) therapy is a pivotal intervention, significantly reducing AF recurrence by 42% and lowering VA incidence by 58%. Moreover, CPAP therapy diminishes sinus bradycardia and occurrences of sinus pause and may reduce the necessity for pacemaker implantation. Recognizing SDB as a modifiable risk factor for cardiac arrhythmias highlights the importance of early diagnosis and effective management, primarily through CPAP therapy, to improve cardiovascular outcomes. Full article

Background: Approximately half of all women develop palpitations during pregnancy, with a quarter experiencing arrhythmias. While most presentations are benign, some cases can result in sudden cardiac death or serious symptom development. Considering such clinical presentation, healthcare providers must acquire knowledge in this area to provide comprehensive prenatal, perinatal, and postnatal care. However, no study could be located that focused on women’s life experiences of such complications during or in the post-pregnancy period. Objectives: The study aims to share the results of a study that explored the life experience of one woman who developed non-sustained ventricular tachycardia during her third pregnancy that lasted into the postpartum period. Methods: Using narrative inquiry self-study methodology, a woman’s experiences were explored to uncover the challenges she faced in coping with such complications during a period of transition for herself and her family. This methodology allowed for an in-depth understanding of how these complications could affect all aspects of her life. Results: Four narrative threads were produced: (1) diagnostic challenges and delayed recognition; (2) impact on maternal identity and family dynamics; (3) navigating healthcare systems and treatment decisions; and (4) long-term adaptation and resilience. Conclusions: The intention was to add to this topic area to ensure future researchers, current and future healthcare providers, and patients have literature they can refer to when studying, providing care for, or experiencing similar health complications. Acquiring this knowledge can aid healthcare professionals to ensure appropriate care is provided, risks are minimized, and their recovery is well supported. Full article

A 67-year-old man started treatment due to frequent asymptomatic premature ventricular complexes (PVCs) accidentally being registered during a preventive examination by a specialist, because of which he was referred to cardiologist. During the initial 24-hour (h) ECG monitoring, 4.5% PVCs and one episode of asymptomatic non-sustained ventricular tachycardia (NSVT) with three PVCs in row, at a frequency of 150 beats per minute (bpm), were detected. After the introduction of beta blockers into therapy, a lower number of PVCs, without NSVT, were recorded in the control 24 h Holter ECG, while transthoracic echocardiography (TTE) showed normal left ventricular (LV) systolic function without cardiomyopathy. So, an exercise test was indicated, and it was interrupted in the third minute at 120 beats per minute (bpm) due to fatigue and pain in the hips, without malignant arrhythmias, angina or dyspneic complaints. During the rest period, a significant inferolateral depression of the ST junction was observed, which recovered in the ninth minute. Immediately after the ECG monitoring stopped, the patient lost consciousness; his pulse was not palpable, but breathing was audible, so cardiac massage was started. After he had regained consciousness, the ECG showed alternating sinus and junctional rhythm with the lowest frequency of 33 bpm, which was accompanied by marked hypotension (80/50 mmHg). The patient was immediately hospitalized; coronary angiography and repeated TTE were completely normal, while continuous ECG monitoring did not confirm malignant rhythm disorders or asystole. It was concluded that it was vasovagal syncope (VVS), most likely caused by the Bezold–Jarisch reflex (BJR). Full article

Electrophysiological aspects of cardiac amyloidosis (CA) are still poorly explored compared to other aspects of the disease. However, electrocardiogram (ECG) abnormalities play an important role in CA diagnosis and prognosis and the management of arrhythmias is a crucial part of CA treatment. Low voltages and a pseudonecrosis pattern with poor R-wave progression in precordial leads are especially common findings. These are useful for CA diagnosis and risk stratification, especially when combined with clinical or echocardiographic findings. Both ventricular and supraventricular arrhythmias are common in CA, especially in transthyretin amyloidosis (ATTR), and their prevalence is related to disease progression. Sustained and non-sustained ventricular tachycardias’ prognostic role is still debated, and, to date, there is a lack of specific indications for implantable cardiac defibrillator (ICD). On the other hand, atrial fibrillation (AF) is the most common supraventricular arrhythmia with a prevalence of up to 88% of ATTR patients. Anticoagulation should be considered irrespective of CHADsVA score. Furthermore, even if AF seems to not be an independent prognostic factor in CA, its treatment for symptom control is still crucial. Finally, conduction disturbances and bradyarrhythmias are also common, requiring pacemaker implantation in up to 40% of patients. Full article

Background: Premature ventricular complexes (PVCs) are common electrocardiographic abnormalities and may be a prognosticator in predicting mortality in patients with structurally normal hearts or chronic heart diseases. Whether PVC burden was associated with mortality in patients with chronic atrial fibrillation (AF) remained unknown. We investigated the prognostic value of PVC burden in patients with persistent AF. Methods: A retrospective analysis of 24 h Holter recordings of 1767 patients with persistent AF was conducted. Clinical characteristics, 24 h average heart rate (HR), and PVC measures, including 24 h PVC burden and the presence of consecutive PVCs (including any PVC couplet, triplet, or non-sustained ventricular tachycardia) were examined for the prediction of all-cause and cardiovascular mortality using the Cox proportional hazards model. Results: After a median follow-up time of 30 months, 286 (16%) patients died and 1481 (84%) patients survived. Multivariate analysis revealed that age, heart failure, stroke, angiotensin-converting enzyme inhibitor/angiotensin receptor blocker, beta-blocker, digoxin, oral anticoagulant use, and estimated glomerular filtration rate were significant baseline predictors of all-cause mortality and cardiovascular mortality. Twenty-four-hour PVC burden and the presence of consecutive PVCs were significantly associated with all-cause and cardiovascular mortality after adjusting for significant clinical factors. When compared to the first quartile of PVC burden (<0.003%/day), the highest quartile (>0.3%/day) was significantly associated with an increased risk of all-cause mortality (hazard ratio, 2.46; 95% CI, 1.77–3.42) and cardiovascular mortality (hazard ratio: 2.67; 95% CI, 1.76–4.06). Conclusions: Twenty-four-hour PVC burden is independently associated with all-cause and cardiovascular mortality in patients with persistent AF. Full article

Background: Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) are common valvular abnormalities that have been associated with ventricular arrhythmias (VA). Cardiac magnetic resonance imaging (CMR) has a key role in risk stratification of VA, including assessment of late gadolinium enhancement (LGE). Methods: Single-center retrospective analysis of patients with MVP or MAD who had >1 CMR and >1 24 h Holter registration available. Data are presented in detail, including evolution of VA and presence of LGE over time. Results: A total of twelve patients had repeated CMR and Holter registrations available, of which in four (33%) patients, it was conducted before and after minimal invasive mitral valve repair (MVR). After a median of 4.7 years, four out of eight (50%) patients without surgical intervention had new areas of LGE. New LGE was observed in the papillary muscles and the mid to basal inferolateral wall. In four patients, presenting with syncope or high-risk non-sustained ventricular tachycardia (VT), programmed ventricular stimulation was performed and in two (50%), sustained monomorphic VT was easily inducible. In two patients who underwent MVR, new LGE was observed in the basal inferolateral wall of which one presented with an increased burden of VA. Conclusions: In patients with MVP and MAD, repeat CMR may show new LGE in a small subset of patients, even shortly after MVR. A subgroup of patients who presented with an increase in VA burden showed new LGE upon repeat CMR. VA in patients with MVP and MAD are part of a heterogeneous spectrum that requires further investigation to establish risk stratification strategies. Full article