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Late-onset cardiac manifestations of Fabry disease are frequently associated with high rates of missed diagnoses and misdiagnoses. We present a case of a 71-year-old male with late-onset Fabry disease whose diagnosis was delayed due to the absence of typical symptoms. The patient has a history of nephrotic syndrome and is currently suffering from end-stage renal disease (ESRD), undergoing maintenance hemodialysis. He was previously diagnosed with diffuse left ventricular hypertrophy and heart block. Upon admission, cardiac examination revealed reduced longitudinal strain of the left ventricle. Fabry disease was suspected due to recurrent heart failure, persistent slight elevation in troponin I (TNI) levels, recurrent syncope, and hearing loss. Subsequent measurement of α-galactosidase A activity and genetic testing confirmed the diagnosis. This case highlights the importance of considering Fabry disease in patients with renal failure, recurrent heart failure, persistent slight elevation in TNI levels, and bilateral interventricular septum syndrome.

As an immune checkpoint inhibitor (ICI), tislelizumab is an anti-programmed cell death protein 1 (PD-1) drug. With the extensive application of ICIs, there is an ever-increasing proportion of immune-related adverse events (irAEs) in clinical settings, some of which may even be life-threatening. Herein, we present a patient with tislelizumab-induced adrenal crisis. The main clinical manifestation was recurrent syncope accompanied by high-grade fever. Timely identification and hormone replacement therapy helped the patient overcome the crisis well. Finally, the patient discontinued tislelizumab and switched to antibody–drug conjugate (ADC) therapy. We report this case to improve our understanding of this situation, identify this kind of disease, and prevent adrenal crisis in time. Eventually, limiting toxicities reduces the interruption of immunotherapy. Since irAEs are multisystem damage with more non-specific symptoms, except for oncologists, general practitioners who endorse the need for taking a holistic approach to the patient should play a vital role in the management of cancer treatment.

Background Dual‐chamber pacemakers equipped with anti‐reflex syncope algorithms are an established therapeutic option for preventing recurrent syncope in selected patients with reflex syncope. However, their efficacy in patients under 40 years old and in non‐type 2B syncope remains uncertain and clinical predictors of recurrent syncope post‐implantation are not well established. Objective To identify clinical factors associated with early recurrence of syncope in patients with reflex syncope who received dual‐chamber pacemakers with anti‐syncope functionality. Methods This retrospective cohort study included 117 patients (65% female) with reflex syncope confirmed by a positive tilt‐table test. All received dual‐chamber pacemakers. The primary endpoint was syncope recurrence within 6 months. Results Among 117 paced patients, 15 (12.8%) had recurrent syncope by 6 months. In the reduced Cox model, all three prespecified variables independently predicted recurrence: female sex (HR: 5.386; 95% CI: 1.689–17.175; p = 0.004), systolic blood pressure differential between the end of the passive phase and the syncope onset (HR: 1.036; 95% CI: 1.008–1.064; p = 0.011), number of prior syncope episodes (HR: 2.950; 95% CI: 1.565–5.561; p = 0.001). ROC‐based cutoffs supported descriptive separation (e.g., ≥ 2.5 prior episodes; ΔSBP ≥ 87.5 mmHg; asystole ≥ 13.5 s), but continuous coding was used for modeling to avoid information loss. Conclusion Within 6 months of dual‐chamber pacemaker implantation, recurrent syncope was more likely in female sex, a larger systolic blood pressure differential between the end of the passive phase and syncope onset, and greater pre‐implantation syncope burdens. In this single‐center retrospective cohort of 117 patients with reflex syncope treated with dual‐chamber pacemakers, 12.8% had syncope recurrence within 6 months. Female sex, higher prior syncope burden, and a larger systolic blood pressure differential identified a high‐risk subgroup requiring closer follow‐up and adjunctive measures.

Background Recurrent syncope with seizure-like stiffening may mimic epilepsy or neurological disorders, and when routine tests are repeatedly normal, intermittent arrhythmia can be easily overlooked. Case presentation We report a case of a 65-year-old man with hypertension, hyperuricaemia, dyslipidaemia, and asthma who experienced recurrent syncopal episodes over one month. He presented several times to emergency departments and tertiary hospitals. Neurological and cardiovascular investigations—including brain MRI, echocardiography, coronary angiography, laboratory tests, and a previous Holter ECG—were consistently unremarkable. The initial diagnosis was hypertensive crisis based on marked post-event blood pressure surges, which was later recognized as a secondary phenomena rather than the primary cause of syncope. On the index admission, continuous emergency monitoring captured a sinus arrest of ~ 17 s with absent arterial pulse waveform, followed by bradyarrhythmia. Post-event blood pressure spiked to 220/110 mmHg. A repeat Holter ECG confirmed intermittent Mobitz II and complete AV block with asystole up to 18.6 s. EEG, performed during this admission to exclude epilepsy, was normal. A dual-chamber permanent pacemaker was implanted with complete resolution of symptoms (Shen et al, Circulation 136(5):e60-e122, 2017; Kusumoto et al, Circulation 140(8): e382-e482, 2019; Brignole et al, Eur Heart J 39(21):1883-1948, 2018). Conclusion This case demonstrates how intermittent AV block may masquerade as seizure or hypertensive crisis, underlining the critical role of emergency department monitoring and prolonged ECG recording in recurrent unexplained syncope.

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.

Steal syndrome is characterized by the disruption of blood flow from specific vascular territories due to perfusion via collateral vessels bypassing an obstructed artery segment. In the neurovascular setting, this can lead to entities such as subclavian steal syndrome or the less prevalent double steal phenomenon, primarily associated with atherosclerosis. We present a case of an early 80s patient with cerebrovascular insufficiency who exhibited neurological deficits during mastication. The angiographic evaluation showed occlusion at the origins of the left common carotid and of the brachiocephalic trunk. This configuration caused the left vertebral artery to be the only vessel filling anterograde. The flow in the right vertebral artery is retrograde, filling the subclavian and the right common carotid artery. Endovascular revascularization of the brachiocephalic trunk was successfully performed using a Gore Viabahn VBX Balloon Expandable Stent (W.L. Gore & Associates, Flagstaff, AZ, USA), leading to symptom resolution.

The study assesses the burden and costs of recurring unexplained syncope and injuries and the effectiveness of implantable loop recorders. The English national hospital database (Hospital Episode Statistics) was retrospectively analyzed. 12,002 patients were identified with repeated syncope hospitalizations. 25% of patients were hospitalized at least once again for syncope, 9% of the patients were hospitalized at least once for an injury, causing substantial costs. In the second analysis: 10,902 patients implanted with an implantable cardiac monitor were tracked. By year 3, hospitalizations due to syncope had dropped by 60% versus pre-implantable cardiac monitor (ICM) levels. This study shows a high rate of recurrent syncope admissions and a parallel burden of hospitalizations for injuries. Use of an ICM appears to reduce syncope hospitalizations.

Despite advances in syncope evaluation strategies and risk stratification, the high cost of syncope is largely driven by extensive and often repetitive testing. This analysis of a large deidentified US claims database compared the use of diagnostic tests, therapeutic procedures, and the recurrence rate of acute syncope events before and after placement of an insertable cardiac monitor (ICM) in syncope patients. The patients had a minimum of 1 year of continuous enrollment before and 2 years after ICM placement. Among 2140 patients identified, a statistically significant reduction in the use of 14 out of 18 tests was observed during follow-up compared with pre-ICM testing. During the 2-year follow-up, 28.3% of patients underwent cardiac therapeutic interventions after a median of 127 days. Significantly fewer patients experienced acute syncope events during the 1st and 2nd years of ICM follow-up compared with the 1-year pre-ICM period, and the frequency of events per patient also decreased. In conclusion, reductions in diagnostic testing and acute syncope events were observed after ICM placement in a large real-world cohort of unexplained syncope patients. Further studies are needed to prospectively assess the impact of ICM vs. short-term monitoring on patient outcomes and healthcare utilization.

Cardioneuroablation (CNA) represents a promising therapy for recurrent vasovagal syncope (VVS), extrinsically driven atrioventricular block (AVB) and sinus node dysfunction (SND). However, effectiveness in patients aged 50 and above is not well-established. In this prospective study of patients referred for CNA, we compared syncope and pacemaker implantation free survival, heart rate (HR) variability (HRV) and quality of life between two age groups: group A (< 50 years) and B (≥ 50 years). A total of 50 patients were included (17 Group A and 33 Group B). The etiologies comprised VVS (56%), AVB (22%), and SND (22%). After a median follow-up of 17.0 (12.5–26.0) months, there were no differences of the combined endpoint of syncope or pacemaker implantation free-survival between groups (29.4.% vs. 21.2%; p-log-rank = 0.736). 84% of the entire cohort remained free from syncope, with a better but not significant syncope free survival in the older group (23.5% vs. 12.1%; p-log rank = 0.486). There were no differences in pacemaker implantation rate (A 5.9% vs. B 17.6%; p log-rank = 0.658). Notably, older patients had lower HR values post-procedure compared to younger patients (68.9 ± 13.3 vs. 80.4 ± 16.3 bpm; p  = 0.012). Both groups exhibited a decrease in HRV parameters and an improvement in quality of life. In conclusion, CNA has comparable clinical benefits for patients aged 50 and above in terms of syncope or pacemaker implantation free survival and HRV reduction when compared to younger patients.

An 84-year-old man presented with a history of repeated syncope and decreased heart rate and blood pressure over the last month. On physical examination, a mass sized ~3×3 cm was palpable in the left submandibular area; the mass was hard, poorly mobile, without tenderness or local skin irritation. The computed tomography angiography examination revealed a soft tissue mass in the neck, at the level of the left carotid bifurcation and above. The left common carotid artery bifurcation and internal and external carotid artery segment were embedded in the mass, and there were multiple enlarged lymph nodes in the left neck. The diagnosis of diffuse large B-cell non-Hodgkin lymphoma was confirmed by a percutaneous biopsy of the left submandibular mass. To the best of our knowledge, this is the first reported case of non-Hodgkin lymphoma involvign the carotid space.