Kenny, Rose Anne

Sickle cell disease (SCD) is the most common genetic haemoglobinopathy worldwide. Due to advancements in care, SCD patients are living longer. Consequently, there is increased interest in long term sequalae of chronic micro-vascular sickling and resultant end organ damage. Sickle cell cardiomyopathy is an emerging clinical entity characterised by a unique combination of ventricular dilatation, ventricular hypertrophy, diastolic dysfunction and pulmonary hypertension. Additionally, SCD patients have impaired autonomic function which is thought to pre-dispose to vaso-occlusive crises through sympathetic activation and parasympathetic withdrawal during times of physiologic stress. Furthermore, sudden death is a major cause of mortality among patients with SCD, however the mechanism has not been elucidated. This review summarizes the sickle cell cardiomyopathy literature, its relationship with autonomic dysfunction and its association with sudden death.

Abstract Aims Syncope remains a diagnostic challenge despite advancements in testing and treatment. Cardiac syncope is an independent predictor of mortality and can be difficult to distinguish from other causes of transient loss of consciousness (TLOC). This paper explores whether artificial intelligence (AI) can improve the evaluation and management of patients with syncope. Methods and results We conducted a literature review and incorporated the opinions of experts in the fields of syncope and AI. The cause of TLOC is often unclear, hospitalization criteria are ambiguous, diagnostic tests are frequently non-informative, and assessments are costly. Patients are left with unanswered questions and limited guidance. Artificial intelligence (AI) has the potential to optimize syncope evaluation by processing large data sets, detecting imperceptible patterns, and assisting clinicians. However, AI has limitations, including errors, lack of human empathy, and uncertain clinical utility. Liability issues further complicate its integration. We present three viewpoints: (i) AI is crucial for advancing syncope management; (ii) AI can enhance the patient experience; and (iii) AI in syncope care is inevitable. Conclusion Artificial intelligence may improve syncope diagnosis and management, particularly through machine learning–based test interpretation and wearable device data. However, it has yet to surpass human clinical judgment in complex decision-making. Current challenges include gaps in understanding syncope mechanisms, AI interpretability, generalizability, and clinical integration. Standardized diagnostic approaches, real-world validation, and curated data sets are essential for progress. Artificial intelligence may enhance efficiency and communication but raises concerns regarding confidentiality, bias, inequities, and legal implications.

Orthostatic hypotension (OH) is frequently observed with hypertension treatment, but its contribution to adverse outcomes is unknown. The SPRINT (Systolic Blood Pressure Intervention Trial) was a randomized trial of adults, age ≥50 years at high risk for cardiovascular disease with a seated systolic blood pressure (BP) of 130 to 180 mm Hg and a standing systolic BP ≥110 mm Hg. Participants were randomized to a systolic BP treatment goal of either <120 or <140 mm Hg. OH was defined as a drop in systolic BP ≥20 or diastolic BP ≥10 mm Hg 1 minute after standing from a seated position. We used Cox models to examine the association of OH with cardiovascular disease or adverse study events by randomized BP goal. During the follow-up period (median 3years), there were 1170 (5.7%) instances of OH among those assigned a standard BP goal and 1057 (5.0%) among those assigned the intensive BP goal. OH was not associated with higher risk of cardiovascular disease events (primary outcome: hazard ratio 1.06 [95% CI, 0.78–1.44]). Moreover, OH was not associated with syncope, electrolyte abnormalities, injurious falls, or acute renal failure. OH was associated with hypotension-related hospitalizations or emergency department visits (hazard ratio, 1.77 [95% CI, 1.11–2.82]) and bradycardia (hazard ratio, 1.94 [95% CI, 1.19–3.15]), but these associations did not differ by BP treatment goal. OH was not associated with a higher risk of cardiovascular disease events, and BP treatment goal had no effect on OH’s association with hypotension and bradycardia. Symptomless OH during hypertension treatment should not be viewed as a reason to down-titrate therapy even in the setting of a lower BP goal. Clinical Trial Registration URL: https://www.clinicaltrials.gov . Unique identifier: NCT01206062.

The aims of this study were (1) to identify research publications studying noninvasive electrocardiogram (ECG) monitoring devices, (2) to define and categorize current technology in noninvasive ECG recording, and (3) to discuss desirable noninvasive recording features for personalized syncope evaluation to guide technological advancement and future studies. We performed a systematic review of the literature that assessed noninvasive ECG-monitoring devices, regardless of the reason for monitoring. We performed an Internet search and corresponded with syncope experts and companies to help identify further eligible products. We extracted information about included studies and device features. We found 173 relevant papers. The main reasons for ECG monitoring were atrial fibrillation (n = 45), coronary artery disease (n = 10), syncope (n = 8), palpitations (n = 8), other cardiac diseases (n = 67), and technological aspects of monitoring (n = 35). We identified 198 devices: 5 hospital telemetry devices, 12 patches, 46 event recorders, 70 Holter monitors, 23 external loop recorders, 20 mobile cardiac outpatient telemetries, and 22 multifunctional devices. The features of each device were very heterogeneous. There are a large number of ECG-monitoring devices with different features available in the market. Our findings may help clinicians select the appropriate device for their patients. Since there are only a few published articles analyzing their usefulness in syncope patients, further research might improve their use in this clinical setting.