A Rare Case of Profound Sinus Bradycardia in a Patient With Descending Aortic Dissection.

A 34-year-old uncontrolled hypertensive male presented with chest pain radiating to the back. Despite severe pain, he was persistently bradycardic at 38 beats per minute. The workup at the emergency department confirmed the presence of an acute Stanford B aortic dissection. Stanford B dissections are not usually associated with bradycardia. It is Stanford A dissections that are mostly linked with bradycardia because Stanford A dissections can cause concomitant coronary artery extension and involvement of the atrioventricular node. This case demonstrates that sinus bradycardia can exist in the acute setting of any painful aortic dissection, even though it might not necessarily involve the coronary arteries.

Genetic testing in the management of inherited cardiac disorders: two cases of Filamin-C arrhythmogenic left ventricular cardiomyopathy.

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a left ventricle-dominant arrhythmogenic cardiomyopathy (ACM) subtype often associated with malignant ventricular arrhythmias, left ventricular (LV) scar and sudden cardiac death. Awareness about LV involvement is now on the rise. The diagnosis relies on structural abnormalities on cardiac magnetic resonance (CMR) imaging and known ACM-causing genetic mutations. Case summary: A 28-year-old lady (Case 1) was referred for cardiac screening after her father passed away suddenly. Her paternal uncle (Case 2) had been diagnosed with supposed dilated cardiomyopathy prior to referral. Both cases were worked up extensively with an electrocardiogram (ECG), 24-h ambulatory ECG monitor, exercise testing, and CMR imaging. Investigations of Case 1 showed T-wave inversion in the infero-lateral leads and a ventricular ectopic burden of 3% on ambulatory monitoring. Cardiac magnetic resonance imaging revealed moderately reduced LV systolic function (ejection fraction of 40%) with circumferential macroscopic fibrosis. Her uncle (Case 2) also had an impaired and dilated ventricle with extensive scar on CMR. Following the recent introduction of a cardiogenetic service in our unit, both were heterozygous for a pathogenic Filamin-C variant (c.7384+1G>A). Based on CMR findings and genetic results, the diagnosis of both patients was deemed to be ALVC. After years of surveillance, Patient 1 now has an implantable cardioverter defibrillator (ICD) indication. Discussion: The importance of diagnosing patients with ACM lies in the predisposition to sudden cardiac death. Gene-specific treatment algorithms in ACM may alter management strategies, including ICD implantation as primary prevention. An in-depth multidisciplinary discussion and respecting patient autonomy are key factors in any decision pertaining to ICD implantation.

BEAT-IT: A de-novo cardiac screening programme in Maltese adolescents.

AIMS: Sudden cardiac death (SCD) in young individuals is often unexpected, provoking substantial emotional stress for family and friends of the deceased. Cardiac screening may identify individuals who harbour disorders linked to SCD. The feasibility and diagnostic yield of a nationwide cardiac screening programme in adolescents has never been explored. METHODS: All individuals eligible for cardiac screening (students aged 15 years) were systematically invited to enrol. Students were provided with a health questionnaire. ECGs were acquired at school. A physician led consultation was carried out on site. Participants with an abnormal screen were then referred for secondary evaluation to the nation's tertiary centre. Feasibility criteria included a) participation rate >60%, b) adherence to secondary evaluation >80%, and c) cost per individual screened equating to <€100. The diagnostic yield was also evaluated. RESULTS: At the end of enrolment, 2708 students gave consent (mean 15 years, 50.4% male), equating to 67.9% of the eligible cohort. Overall, 109 participants (4.0%) were referred for further evaluation. An abnormal electrocardiogram (ECG) was the most common reason for referral (3.7%). Fifteen individuals (0.6%) were diagnosed with a cardiac condition. Nine (0.3%) had a condition linked to SCD (n = 1 Long-QT syndrome, n = 1 Hypertrophic Cardiomyopathy, n = 5 Wolff-Parkinson White, n = 2 coronary anomalies). The yield was similar in athletes and non-athletes (p = 0.324). The cost per cardiac individual screened equated to €51.15. CONCLUSION: A nationwide systematic cardiac screening programme for adolescent athletes and non-athletes is feasible and cost-efficient, provided that responsible centres have the appropriate infrastructure.

Native T1 and T2 reference values for maltese healthy cohort.

Cardiac Magnetic Resonance (CMR) is increasingly being used for diagnosing various cardiac conditions. Parametric mapping enables quantitative myocardial characterization by directly measuring myocardial T1 and T2 values. However, reference values of parametric mapping are not standardized across different vendors and scanners, causing drawbacks for clinical implementation of this technique across different sites. We assessed the reference ranges of native T1 and T2 values in a healthy Maltese cohort to establish a local parametric mapping service. Healthy subjects [n = 51; mean age 36.0 (range 19-59) years] with normal cardiac function on CMR were recruited. Subjects underwent uniform parametric mapping pulse sequences [MOLLI 5b(3b)3b for native T1 mapping, and gradient echo single shot FLASH readout for T2 mapping] on a 3 T Siemens MAGNETOM Vida scanner. Native T1 and T2 values were measured by placing a region of interest within the interventricular septum at midventricular level. Intra- and inter-observer variability were assessed using Bland-Altman plots. Mean ± 1.96 SD was used as a reference range. Mean native T1 and T2 values were 1200.1 ± 30.7 ms and 39.5 ± 1.8 ms, respectively. There was no significant bias in repeated measurements by the same and different observers. For the first time in Malta, we established the native T1 and T2 parametric mapping reference values for healthy Caucasian Maltese individuals. This will assist cardiologists to establish diagnosis, disease progression, and response to treatment of various myocardial diseases locally.

The juvenile ECG pattern in adolescent athletes and non-athletes in a national cardiac screening program (BEAT-IT).

BACKGROUND: Anterior T wave inversion (TWI) is frequent in healthy adolescent individuals (juvenile ECG pattern), normalising after puberty. Its clinical implications are uncertain. AIM: This study assessed a) national prevalence of anterior TWI, b) ST segment morphology, c) proportion of individuals with a juvenile ECG pattern whose ECG normalises and d) factors predicting TWI persistence >16 years. METHODS: Adolescents (mean 15y) in Malta were systematically invited to enrol in a cardiac screening program. Subjects completed a health questionnaire and an ECG at their school. Participants with TWI were labelled as TWI in V1-V2 or extended TWI (V1-V3/4). The latter were followed at 1 year with a repeat ECG. Those with persistent extended anterior TWI were offered evaluation and surveillance. RESULTS: The prevalence of isolated anterior TWI was 5.0%, commoner in females (6.3%) independent of athletic ability. Extended TWI was commoner in female athletes (4.2%, non-athletes 2.1%). Females often had shallow TWI without overt ST segment abnormalities. Deep TWI and ST segment changes were more frequent in males. Only 0.2% of cases persisted ≥16 years of age. ST segment characteristics were not able to predict T wave normalisation. No events took place during follow up (40 ± 9 months). CONCLUSION: Anterior TWI is a frequent phenomenon in adolescents, especially in females. Female athletes are also more likely to have extended anterior TWI. Only 0.2% of cases have persistent anterior TWI at 16 years of age. Chest wall anatomy may explain this phenomenon in females. It is uncommon in males, hence why surveillance is more prudent.

Automated external defibrillators and basic life support practices in secondary schools: a nationwide study.

AIM: Cardiac arrest prevention in schools has recently gained momentum. The survival benefit in schools who have access to defibrillators is clear, with far better survival outcomes in children or adults who sustain a cardiac arrest on school grounds. The main objectives of this study were to assess sudden cardiac arrest prevention in Maltese schools, specifically the availability of defibrillators and staff competence in delivering resuscitation. METHODOLOGY AND RESULTS: An online-based questionnaire was distributed to all secondary schools across the Maltese archipelago. Data were collected, tabulated, and analysed using SPSS V.23. Most schools (n = 40, 74.1%) completed the questionnaire. Two schools documented a cardiac arrest in the past 10 years. 87.5% agreed that cardiac arrest prevention is an important health topic. Most have a defibrillator on the premises (n = 37, 92.5%). Only one defibrillator is usually available (n = 27, 75.0%). Despite the majority claiming its ease of accessibility (n = 35, 97.2%), most were not available on every floor (n = 37, 97.2%). Only one-third were close to a sporting facility (n = 11, 30.6%). Schools do not organise regular resuscitation courses (n = 21, 58.3%), with eight schools having five or more certified staff members (23.5%). The number of defibrillators did not influence the frequency of resuscitation courses at school (p = 0.607), and there was no association with the number of certified individuals (p = 0.860). CONCLUSION: Defibrillators are not readily available at secondary schools and are often installed in low-risk areas. Most schools have only one staff member certified in resuscitation. These factors should be addressed with urgency.

A review article of the cardiovascular sequalae in esport athletes: A cause for concern?

The esports industry has grown exponentially and is expected to flourish rapidly in the coming years. Awareness about the cardiovascular sequalae in elite esports athletes has gained considerable momentum. Evidence supporting systematic screening in esports athletes is however lacking. In this narrative review, we discuss potential negative health effects on the cardiovascular system in esports enthusiasts. The pathophysiological mechanisms discussed in this narrative review may put esports athletes at an increased risk of cardiovascular events. Although large studies in esports athletes to substantiate these claims are inexistent, case reports or case-controlled studies have been included to support our hypotheses. Sleep deprivation, psychological stress, altered arterial stiffness, hypertension, and heart rate variability are some of these proposed mechanisms. The available data are unfortunately extrapolated from small cohorts. Nevertheless, the risk of sudden cardiac death is an important health concern, which should be addressed appropriately. This review will raise awareness about the possible negative impact on the cardiovascular system in esports athletes.

A case report of profound atrioventricular block in an endurance athlete: how far do you go?

Background: Athletes presenting with 1st-degree atrioventricular block (AVB) on 12-lead electrocardiogram (ECG) may present a diagnostic conundrum, especially when significantly prolonged and associated with higher degrees of block. A pragmatic stepwise approach to the evaluation of these patients is, therefore, crucial. Case summary: A 19-year-old waterpolo player was referred for assessment of a 1st-degree heart block and one isolated episode of syncope. All other cardiac investigations were within normal limits except for a 24-h ambulatory ECG which showed Mobitz 1 AVB and episodes of 2:1 block occurring in the context of Wenchebach. An electrophysiological study (EPS) was performed which effectively excluded infranodal conductive tissue disease, confirming physiological intranodal block. Discussion: The increase in vagal tone is one of the physiological adaptations to an increased demand in cardiac output in athletes, which explains the presence of 1st-degree AVB in up to 7.5% of athletes. The presence of 2:1 AVB on 24 h ECG raises doubts whether the 1st-degree AVB on resting ECG is pathological or physiological, especially considering this particular patient had suffered an episode of syncope. When this diagnostic uncertainty persists despite non-invasive investigations, including cardiopulmonary exercise testing, invasive EPS may be required to assess the refractoriness of the AV node and at what level within the cardiac conductive system block occurs. The electrophysiological study can effectively rule out infranodal disease by confirming physiological intranodal block using incremental atrial pacing.

Electrocardiographic interpretation in athletes.

Participation in regular exercise of moderate intensity is associated with a plethora of systemic benefits, including a reduction in risk factors for coronary atherosclerosis; however, intensive exercise may paradoxically culminate in sudden cardiac arrest among individuals harboring arrhythmogenic substrates. The precise mechanism for arrhythmogenesis is likely multifactorial, however, surges in catecholamines, electrolyte shifts, acid-base disturbances, increased core temperature and demand myocardial ischemia are potential contributors. Although most deaths occur in middle aged and older males with atherosclerotic coronary artery disease, a significant proportion also affect young athletes with inherited or congenital cardiac abnormalities. The impact of such catastrophes on society, particularly when a young high-profile athlete is affected could be considered a justified reason for identifying individuals who may be at risk. Given the rarity of deaths in young athletes, only the simplest screening test, such as the 12-lead electrocardiography (ECG) may be considered to be cost effective. The ECG is effective for detecting serious electrical diseases in young athletes such as congenital electrical accessory pathways and ion channel diseases but can also identify athletes with potential life-threatening structural diseases such as hypertrophic and arrhythmogenic cardiomyopathy. One of the concerns about ECG screening is that regular intensive exercise results in several physiological alterations in cardiac structure and function that are reflected on the athlete's ECG. Sinus bradycardia, first-degree atrioventricular block, incomplete right bundle branch block, minor J-point elevation and large QRS voltages are common. Conversely, some repolarization anomalies affecting the ST segment, T waves and QT interval may overlap with patterns observed in patients with serious cardiac diseases. The situation is complicated further because age, sex and ethnicity of the athletes also influence the ECG and there is a risk that erroneous interpretation could have serious consequences. This review will describe the normal electrical patterns of the "athlete's heart" and provide insights into differentiation physiological electrical patterns from those observed in serious cardiac disease.

Abnormal ECG Findings in Athletes: Clinical Evaluation and Considerations.

PURPOSE OF REVIEW: Pre-participation cardiovascular evaluation with electrocardiography is normal practice for most sporting bodies. Awareness about sudden cardiac death in athletes and recognizing how screening can help identify vulnerable athletes have empowered different sporting disciplines to invest in the wellbeing of their athletes. RECENT FINDINGS: Discerning physiological electrical alterations due to athletic training from those representing cardiac pathology may be challenging. The mode of investigation of affected athletes is dependent on the electrical anomaly and the disease(s) in question. This review will highlight specific pathological ECG patterns that warrant assessment and surveillance, together with an in-depth review of the recommended algorithm for evaluation.

Left Ventricular Trabeculations in Athletes: Epiphenomenon or Phenotype of Disease?

PURPOSE OF REVIEW: Excessive trabeculation attracting a diagnosis of left ventricular noncompaction cardiomyopathy (LVNC) has been reported in ostensibly healthy athletes. This review aims to explain why this occurs and whether this represents a spectrum of athletic physiological remodelling or unmasking of occult cardiomyopathy. RECENT FINDINGS: Genetic studies have yet to identify a dominant mutation associated with the LVNC phenotype and reported gene mutations overlap with many distinct cardiomyopathies and ion channel disorders, implying that the phenotype is shared across different genetic conditions. Large contemporary cohort studies indicate that current LVNC imaging criteria are oversensitive and not predictive of adverse clinical outcomes. The majority of excessive LV trabeculation, as assessed by current quantification methods, is not due to cardiomyopathy but forms part of the normal continuum in health with potential contributions from cardiac remodelling processes. The study of rare, severe LVNC phenotypes may yield insights into an underlying molecular pathogenesis but in the absence of a universally accepted definition, contamination with aetiologically distinct conditions expressing a similar phenotype will remain an issue. Automated, objective quantification of trabeculation will help to define the normal distribution using big data without the constraint of wide interobserver variation.

Exercise training in heart failure.

Exercise training (ET) in heart failure (HF) has long been established as an important part of HF care. ET is known to improve quality of life and functional capacity in a number of ways. Despite its proposed benefits, evidence supporting its routine inclusion in standard rehabilitation programme is at times conflicting, partly because of the significant heterogeneity present in available randomised controlled trials. There is lack of evidence with regard to the duration of the overall benefit, the optimal exercise regimen and whether certain types of HF aetiologies benefit more than others. The aim of this review is to provide an update to date literature review of the positive and negative evidence surrounding ET in HF, while proposing an efficient novel in-hospital exercise-based rehabilitation programme for patients with HF in addition to a pre-existing HF clinic.

Cardiac troponin: more than meets the eye.

Exercise is known to have a vast array of health benefits. It may however confer delirious effects on most body systems, with the cardiovascular system taking particular prominence. Athletes in particular are known to be at a higher risk for sudden cardiac death as a result of several cardiac adaptations which take place. Myocardial damage as a result of extreme exertional activities is thought to play a very important role in this risk. Cardiac troponin I is widely known to be an excellent diagnostic marker which is used in patients suspected of having acute coronary syndrome. Its release during exercise has been routinely studied, with many hypotheses currently being proposed as to its role and potential complications once released. Whether or not it implies that myocardial damage is taking place as a result of exercise is debatable, but its release might have some role in the development of cardiotoxic states which predisposes athletes to significant cardiac risk. This review aims to discuss the proposed mechanisms in exercise-induced troponin release, while also goes into its clinical relevance and potential early and late sequelae.