Improving cardiovascular outcomes for patients with heart failure in sub-Saharan Africa: conference proceedings of the 2022 Nigerian Cardiovascular Symposium.

The Nigerian Cardiovascular Symposium is an annual conference held in partnership with cardiologists in Nigeria and the diaspora to provide updates in cardiovascular medicine and cardiothoracic surgery with the aim of optimising cardiovascular care for the Nigerian population. This virtual conference (due to the COVID-19 pandemic) has created an opportunity for effective capacity building of the Nigerian cardiology workforce. The objective of the conference was for experts to provide updates on current trends, clinical trials and innovations in heart failure, selected cardiomyopathies such as hypertrophic cardiomyopathy and cardiac amyloidosis, pulmonary hypertension, cardiogenic shock, left ventricular assist devices and heart transplantation. Furthermore, the conference aimed to equip the Nigerian cardiovascular workforce with skills and knowledge to optimise the delivery of effective cardiovascular care, with the hope of curbing 'medical tourism' and the current 'brain drain' in Nigeria. Challenges to optimal cardiovascular care in Nigeria include workforce shortage, limited capacity of intensive care units, and availability of medications. This partnership represents a key first step in addressing these challenges. Future action items include enhanced collaboration between cardiologists in Nigeria and the diaspora, advancing participation and enrollment of African patients in global heart failure clinical trials, and the urgent need to develop heart failure clinical practice guidelines for Nigerian patients.

Phospholamban Mutation Leading to Recurrent Short-Coupled Polymorphic Ventricular Tachycardia and Fibrillation in Patient without Apparent Structural Heart Disease

Background Phospholamban (PLN), an inhibitor of sarcoplasmic reticulum (SR) Ca2+-ATPase, is a regulator of Ca2+ release during excitation-contraction coupling. We present a case of recurrent polymorphic ventricular tachycardia (PMVT)/ventricular fibrillation (VF) due to a PLN mutation. Case 38 year-old male presents after resuscitation following VF arrest. An ICD was implanted. Seven years later, he presented with VF storm requiring ventricular assist device support and he underwent catheter ablation of PVC triggers of VF arising from the moderator band. Because he had an ECG that was concerning for early repolarization syndrome, he was placed on quinidine and metoprolol. After an episode of VT in 2020 in the setting of COVID infection, whole genome sequencing was obtained and identified a pathogenic PLN mutation. PLN L39Ter has been associated with dilated and hypertrophic cardiomyopathy as well as sudden death. The patient has a history of normal left ventricular function and wall thickness by echocardiography. Decision-making Given the involvement of PLN on SR handling of Ca2+, flecainide may be a more effective therapy for the treatment of PMVT/VF in this patient. Conclusion PLN mutations have been associated with cardiomyopathies. This case illustrates a patient with the pathogenic PLN L39X variant with short-coupled PMVT with no imaging evidence of structural heart disease. Whether a more targeted therapy such as flecainide may be more effective in this patient remains to be determined. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

Selection of the best of 2022 in congenital heart disease

This paper shows a selection of the most relevant articles in congenital heart diseases in the last year. About intervencional procedures, we comment the latest guidelines in 2021, as well as some interesting papers on the implementation of materials and techniques in the field of percutaneous treatment of congenital heart diseases. In cardiac imaging, we focus on articles related to the revolutionary advance of 4D-NMR and 3D echo in the study of these congenital diseases. Respect pediatrics, there are interesting studies about multisystem inflammatory syndrome linked to SARS-CoV-2 (MIS-C) and also in the field of hypertrophic cardiomyopathy. Related to surgery, we comment the hybrid technique for treating hypoplastic left ventricle. Finally, regarding arrhythmias in congenital heart disease, we focus our attention on atrial fibrillation, due to the peculiarities that exist in this type of patients.

Direct Oral Anticoagulants for Stroke and Systemic Embolism Prevention in Patients with Left Ventricular Thrombus.

In recent years, direct oral anticoagulants (DOAC) have accumulated evidence of efficacy and safety in various clinical scenarios and are approved for a wide spectrum of indications. Still, they are currently used off-label for left ventricular thrombus owing to a paucity of evidence. For the same reason, there is a lack of guideline indication as well. Our work is based on an exhaustive analysis of the available literature and provides a structured and detailed update on the use of DOACs in patients with left ventricle thrombus. The safety and efficacy of DOACs were analyzed in particular clinical scenarios. As far as we know, this is the first paper that analyzes DOACs in this approach.

Heart Transplantation and Covid-19 in the Early Postoperative Period in Hypertrophic Cardiomyopathy: A Clinical Case

Hypertrophic cardiomyopathy (HCM) is a disease that is usually unresponsive to conservative pathogenetic therapy. It does not have clearly developed surgical correction algorithms. Heart transplantation (HTx) is the sole therapeutic option when drug therapy is ineffective and surgical reduction of hypertrophic myocardium is not feasible. There are only sporadic reports in the literature about HTx for HCM. The novel coronavirus disease 2019 (COVID-19) pandemic has significantly affected the work of cardiac surgical units and, in particular, organ transplantation activities. This paper presents a clinical case of an HCM patient who underwent HTx, complicated by COVID-19 infection in the early postoperative period.

Elevated angiotensin-converting enzyme 2 (ACE2) expression in cats with hypertrophic cardiomyopathy.

Angiotensin-converting enzyme 2 (ACE2) is an enzyme within the renin-angiotensin-aldosterone system that plays a role in regulating blood pressure. However, it is also a cellular receptor for infection with SARS coronaviruses. Although most cats develop subclinical or mild disease following infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) acquired from human patients, a previous study has suggested hypertrophic cardiomyopathy (HCM) is a potential risk factor for the development of severe disease in the cat. Herein we investigate the ACE2 protein expression in the lung, heart, and kidney from a small subset of cats with (n = 10) and without HCM (n = 10) by immunohistochemistry. The abundance and intensity of ACE2 expression is slightly elevated in alveoli (p = 0.09; 0.07, respectively) and bronchioles (p = 0.095; 0.37, respectively). However, statistically elevated abundance and intensity of ACE-2 expression was only evident in the heart of cats with HCM (p = 0.032; p = 0.011, respectively). Further investigation did not demonstrate a statistical correlation between the ACE2 expression in the heart in relation to the heart weight to body weight ratio, and the ventricular wall ratio. Current findings suggest an overexpression of ACE2 in HCM cases but follow up study is warranted to understand the pathophysiological process.

Heart Rate Variability And Cardio-Renal Outcomes In Patients With Novel Covid 19 Infection

Studies have shown that SARS-CoV2 can infect the vagal nerve and its connections to the brain stem. This neuronal involvement is seen mostly in the delayed inflammatory phase. It is associated with autonomic nervous system dysfunction, resulting in decreased respiratory ventilation and impaired blood pressure as well as heart rate regulation. The dysautonomia seen in SARSCoV-2 infection can be measured, and heart rate variability (HRV) measurement is one method. Our study explores the relationship between autonomic dysfunction and mortality in patients with COVID- 19 using HRV measurement. In a prospective design, data of consecutive patients with SARS-CoV-2 positive infection admitted to Banner university of Arizona, Tucson, and whose telemetry information was available was collected between August 2020 to November 2021. We attempted to use the telemetry data to measure diurnal heart rate variation by obtaining mean average hourly heart rates, divided into 12-hour day/night periods. The primary outcome measure was mortality. The secondary outcome measured includes incidence of acute kidney injury, transfer to ICU, need for mechanical ventilation, and live discharge from hospital. 334 patients were included in the study. The baseline demographic characteristics, medical history, radiological data, laboratory data, details of medications, and hospital course were reported. Diseases associated with an autonomic dysfunction like diabetes mellitus, chronic renal failure, a history of alcohol abuse, clinical evidence of autonomic neuropathy, or a recent myocardial infarction, documented constrictive or hypertrophic cardiomyopathy, sustained non-sinus dysrhythmias, atrioventricular conduction defects will be excluded in the final analysis. Mortality was high in patients with lower heart rate variability. Compared with survivors, non-survivors were older, were less frequently women, had a higher prevalence of diabetes mellitus, longer stay in the hospital, received an organ transplant, smoking, and higher level of calcitonin. Lower heart rate variability was associated with a higher incidence of acute kidney injury, need for mechanical ventilation, and need for ECMO. This study suggests that analysis of the variability of heart rate may have prognostic implications in patients with COVID19.

British Cardiovascular Society Annual Conference, '100 years of Cardiology'

The proceedings contain 226 papers. The topics discussed include: mitral valve abnormalities in decedents of sudden cardiac death due to hypertrophic cardiomyopathy and idiopathic left ventricular hypertrophy;prevalence and diagnostic significance of novel 12-lead ECG patterns following COVID-19 infection in elite soccer players;clinical outcomes and myocardial recovery in energetics, perfusion and contractile function after valve replacement surgery in severe aortic stenosis patients with diabetes comorbidity;brain phenotype of takotsubo syndrome;improving the diagnostic accuracy of apical hypertrophic cardiomyopathy;investigating a novel role for nesprin-1 and the linc complex in cardiomyocyte mechanotransduction;transcatheter aortic valve implantation in patients with right bundle branch block: should prophylactic pacing be undertaken?;and disease penetrance in asymptomatic carriers of familial cardiomyopathy variants.

COVID-19 infection and myocarditis after surgical left ventricle reconstruction in patient with hypertrophic cardiomyopathy

It has now been shown that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causing a new COVID-19 infection, can penetrate the myocardium and have both direct damaging and toxic effects. The main cardiac manifestations of this disease are fulminant myocarditis, atrial and ventricular arrhythmias, and heart failure. Cardiac arrhythmias are common complication not only in adult patients, but also in children. In this paper, we present a clinical case of the young female patient who successfully underwent operation because of a submitral (diffuse-generalized) form of HCM, with a long event-free postoperative course, and with the rapid cardiac dysfunction, syncopes and cardiac arrhythmias after COVID-19 infection. Syncopes were caused by self-terminating short runs of ventricular tachycardia that did not trigger ICD shock. After the medical treatment of myocarditis, she has a clinical improvement and further favorable postoperative period.

Apical Hypertrophic Cardiomyopathy Prompting Aneurysm, Thrombus, and Cardiac Arrest in a 56-Year-Old Female.

Hypertrophic cardiomyopathy (HCM) is the most prevalent genetic cardiac disease while apical hypertrophic cardiomyopathy (apHCM) is a rare subset of HCM. The significance of this case report is to present apHCM, its chronological course, and its association with left ventricular aneurysm, thrombosis, and cardiac arrest. We present the case of a 56-year-old female with a past medical history of apHCM who was admitted for substernal chest pain, developed a ventricular storm (VT), and subsequently suffered cardiac arrest; resuscitation of spontaneous circulation (ROSC) was eventually achieved after 10 minutes. It was initially thought that her arrhythmia and hemodynamic decompensation were purely secondary to cocaine use at a party six hours prior to her presentation. During hospitalization, cardiac magnetic resonance imaging demonstrated a severe apHCM apical aneurysm, thrombosis, and a re-entrant circuit as a likely cause of this patient's decompensation and eventual cardiac arrest. After several days of hemodynamic stability and decreased dependence on intravenous antiarrhythmic medication infusions, she was extubated and transitioned to oral amiodarone and beta-blocker therapy with the implantation of a cardioverter-defibrillator (ICD). In this case, we analyze the continuum of apHCM, a rare subset of HCM once thought to be benign but with the emergence of complications, including aneurysm, thrombus formation, resistant ventricular tachycardia, and cardiac arrest. Recognition and management of apHCM with medical and/or surgical intervention are therefore critical to prevent the aforementioned sequela.

Impact of SARS-Cov-2 infection in patients with hypertrophic cardiomyopathy: results of an international multicentre registry.

AIMS: To describe the natural history of SARS-CoV-2 infection in patients with hypertrophic cardiomyopathy (HCM) compared with a control group and to identify predictors of adverse events. METHODS AND RESULTS: Three hundred and five patients [age 56.6 ± 16.9 years old, 191 (62.6%) male patients] with HCM and SARS-Cov-2 infection were enrolled. The control group consisted of 91 131 infected individuals. Endpoints were (i) SARS-CoV-2 related mortality and (ii) severe clinical course [death or intensive care unit (ICU) admission]. New onset of atrial fibrillation, ventricular arrhythmias, shock, stroke, and cardiac arrest were also recorded. Sixty-nine (22.9%) HCM patients were hospitalized for non-ICU level care, and 21 (7.0%) required ICU care. Seventeen (5.6%) died: eight (2.6%) of respiratory failure, four (1.3%) of heart failure, two (0.7%) suddenly, and three (1.0%) due to other SARS-CoV-2-related complications. Covariates associated with mortality in the multivariable were age {odds ratio (OR) per 10 year increase 2.25 [95% confidence interval (CI): 1.12-4.51], P = 0.0229}, baseline New York Heart Association class [OR per one-unit increase 4.01 (95%CI: 1.75-9.20), P = 0.0011], presence of left ventricular outflow tract obstruction [OR 5.59 (95%CI: 1.16-26.92), P = 0.0317], and left ventricular systolic impairment [OR 7.72 (95%CI: 1.20-49.79), P = 0.0316]. Controlling for age and sex and comparing HCM patients with a community-based SARS-CoV-2 cohort, the presence of HCM was associated with a borderline significant increased risk of mortality OR 1.70 (95%CI: 0.98-2.91, P = 0.0600). CONCLUSIONS: Over one-fourth of HCM patients infected with SARS-Cov-2 required hospitalization, including 6% in an ICU setting. Age and cardiac features related to HCM, including baseline functional class, left ventricular outflow tract obstruction, and systolic impairment, conveyed increased risk of mortality.

Sudden cardiac death in the pediatric and adolescent population

Each year nearly 7,000 children, adolescents, and young adults in the United States as well as tens of thousands worldwide die unexpectedly. Nearly 50% of these deaths occur with little or no warning. This discussion reviews the epidemiology, presentation, diagnosis, and management of sudden cardiac death (SCD). SCD is predominantly due to previously unrecognized structural abnormalities of the heart and underlying or acquired conditions that predispose persons, especially when involved in exercise, to sudden death. Topics considered include SCD presenting signs, symptoms, diagnoses, and when applicable, management of common conditions associated with SCD.

USE OF REMDESEVIR CAUSING SINUS BRADYCARDIA AND EVENTUALLY ASYMPTOMATIC MOBITZ TYPE 1 ATRIOVENTRICULAR BLOCK

Background: Remdesivir has emerged as a novel treatment in hospitalized COVID19 patients not requiring mechanical ventilation. Though there have been several case reports of remdesivir-associated sinus bradycardia, this association is still unclear. Furthermore, remdesivir's interaction with beta blockers has not been studied. Case: A 70-year-old woman with apical hypertrophic cardiomyopathy (HCM), heart failure with reduced ejection fraction (HFrEF) and atrial fibrillation (AF) status post ablation presented with shortness of breath. She was tachycardic to 115 beats per minute (BPM) and hypoxemic to the 80’s, requiring supplemental oxygen via a non-rebreather mask. She was found to have COVID19 pneumonia, for which dexamethasone and remdesivir were started. She developed marked bradycardia and eventually asymptomatic Mobitz type 1 atrioventricular block (AVB). Decision-making: Once COVID19 pneumonia was diagnosed, dexamethasone and remdesivir were started. She immediately became bradycardic and remdesivir and beta blockade were held. Of note, she was taking metoprolol succinate at home for HFrEF. Bradycardia and AVB resolved with cessation of remdesivir and she was discharged home safely on metoprolol succinate. Conclusion: Patients on remdesivir, especially those with underlying cardiomyopathy, are at higher risk for bradyarrhythmia. Remdesivir may potentiate the effects of beta blockers and their concomitant use requires judicious monitoring. [Formula presented]

A Case of Severe Left-Ventricular Noncompaction Associated with Splicing Altering Variant in the FHOD3 Gene.

Left ventricular noncompaction (LVNC) is a highly heterogeneous primary disorder of the myocardium. Its clinical features and genetic spectrum strongly overlap with other types of primary cardiomyopathies, in particular, hypertrophic cardiomyopathy. Study and the accumulation of genotype-phenotype correlations are the way to improve the precision of our diagnostics. We present a familial case of LVNC with arrhythmic and thrombotic complications, myocardial fibrosis and heart failure, cosegregating with the splicing variant in the FHOD3 gene. This is the first description of FHOD3-dependent LVNC to our knowledge. We also revise the assumed mechanism of pathogenesis in the case of FHOD3 splicing alterations.

COVID-19 in Adults With Hypertrophic Cardiomyopathy.

Background: Individuals with cardiovascular disease are considered high risk for severe COVID-19. However, the clinical impact of COVID-19 in patients with hypertrophic cardiomyopathy (HCM) is unknown. The purpose of this study was to describe the clinical course and outcomes of COVID-19 in patients with HCM. Methods: This retrospective observational study included adults with HCM and positive PCR/antibody test for SARS-CoV-2 at a large urban hospital system in the New York from January, 2020 to January, 2021. Results: Seventy individuals were included, with a mean (SD) age of 60.1 (15.1) years, 39 (55.7%) of whom were male, and 42 (60%) white. Forty-five (65.3%) patients had obstructive HCM. Hypertension and obesity (BMI ≥ 30) were present in 45 (64.3%) and 37 (52.9%) patients, and the prevalence of atrial fibrillation, obstructive sleep apnea and diabetes was high. Common symptoms of COVID-19 were fever, cough, shortness of breath and fatigue, affecting 33 (47.1%), 33 (47.1%), 28 (40.0%), and 28 (40.0%) patients, respectively. Fourteen (20%) patients were hospitalized. The majority (45 [64.3%] patients) recovered without intervention. Two patients had non-fatal pulmonary embolisms, 1 had atrial fibrillation requiring electrical cardioversion and 1 had acute decompensated heart failure. Three (4.3%) patients required mechanical ventilation, two of whom died (case fatality rate 2.9%). A total of 15 (21.4%) patients were asymptomatic. Conclusions: Our data suggest that in this diverse and high-risk group of patients with HCM, established risk factors for severe COVID-19, such as obesity, may be more important drivers of morbidity and mortality than the presence of HCM alone.

Mechanisms of troponin release into serum in cardiac injury associated with COVID-19 patients.

Serum levels of thin filament proteins, cardiac troponin T (cTnT) and cardiac troponin I (cTnI) employing high sensitivity antibodies provide a state-of-the art determination of cardiac myocyte injury in COVID-19 patients. Although there is now sufficient evidence of the value of these determinations in patients infected with SARS-CoV-2, mechanisms of their release have not been considered in depth. We summarize the importance of these mechanisms with emphasis on their relation to prognosis, stratification, and treatment of COVID-19 patients. Apart from frank necrotic cell death, there are other mechanisms of myocyte injury leading to membrane fragility that provoke release of cTnT and cTnI. We discuss a rationale for understanding these mechanisms in COVID-19 patients with co-morbidities associated with myocyte injury such as heart failure, hypertension, arrythmias, diabetes, and inflammation. We describe how understanding these significant aspects of these mechanisms in the promotion of angiotensin signaling by SARS-CoV-2 can affect treatment options in the context of individualized therapies. Moreover, with likely omic data related to serum troponins and with the identification of elevations of serum troponins now more broadly detected employing high sensitivity antibodies, we think it is important to consider molecular mechanisms of elevations in serum troponin as an element in clinical decisions and as a critical aspect of development of new therapies.

The relevance of specific heart failure outpatient programs in the COVID era: an appropriate model for every disease.

Heart Failure (HF) is characterized by an elevated readmission rate, with almost 50% of events occurring after the first episode over the first 6 months of the post-discharge period. In this context, the vulnerable phase represents the period when patients elapse from a sub-acute to a more stabilized chronic phase. The lack of an accurate approach for each HF subtype is probably the main cause of the inconclusive data in reducing the trend of recurrent hospitalizations. Most care programs are based on the main diagnosis and the HF stages, but a model focused on the specific HF etiology is lacking. The HF clinic route based on the HF etiology and the underlying diseases responsible for HF could become an interesting approach, compared with the traditional programs, mainly based on non-specific HF subtypes and New York Heart Association class, rather than on detailed etiologic and epidemiological data. This type of care may reduce the 30-day readmission rates for HF, increase the use of evidence-based therapies, prevent the exacerbation of each comorbidity, improve patient compliance, and decrease the use of resources. For all these reasons, we propose a dedicated outpatient HF program with a daily practice scenario that could improve the early identification of symptom progression and the quality-of-life evaluation, facilitate the access to diagnostic and laboratory tools and improve the utilization of financial resources, together with optimal medical titration and management.

Chronic Stress, Exercise and Cardiovascular Disease: Placing the Benefits and Risks of Physical Activity into Perspective.

Chronic stress, which has been exacerbated worldwide by the lingering COVID pandemic, has been strongly linked to cardiovascular disease (CVD). In addition, autonomic dysregulation via sustained sympathetic activity has been shown to increase the risk of arrhythmias, platelet aggregation, acute coronary syndromes and heart failure. Fortunately, effective coping strategies have been shown to attenuate the magnitude of hyperarousal associated with the stress response, including moderate-to-vigorous lifestyle activity and/or structured exercise. A good-to-excellent level of cardiorespiratory fitness also appears to be highly cardioprotective. These beneficial effects have been substantiated by numerous studies that have evaluated the levels of stress reactivity and stress recovery in physically active individuals versus matched sedentary controls, as well as before and after exercise interventions. On the other hand, unaccustomed strenuous exercise in habitually sedentary persons with underlying CVD is associated with a disproportionate incidence of acute cardiac events. Moreover, extreme exercise regimens appear to increase coronary calcification and the likelihood of developing atrial fibrillation. This review summarizes these relations and more, with specific reference to placing the benefits and risks of physical activity into perspective.

Association of COVID-19 infection with large thrombi in left and right atrial appendages.

BACKGROUND: Multiple intra-atrial thrombi are found rarely except in the presence of prosthetic valves, intra-cardiac devices, structural connections like foramen ovale and thrombophilia. CASE PRESENTATION: We reported acute thrombosis formation in right and left atrial appendages of a 66-year old man admitted due to progressive dyspnea since 7 days earlier. He had a history of prior laryngeal Squamous Cell Carcinoma, apical hypertrophic cardiomyopathy (HCM), and atrial fibrillation (AF). Infection with COVID-19 was confirmed thereafter. Cardiac Magnetic Resonance Imaging (CMR) suggested the diagnosis of atrial clot superior to neoplasm. After surgical removal of the thrombi, symptoms as well as imaging features of pneumonia were resolved. CONCLUSIONS: We should focus on different presentations and complications of systemic inflammation especially in the setting of COVID-19 infection. Although risk factors of thrombosis are present in some of these patients, rapid progression as well as unusual types of involvement may indicate to a new trigger.

Severe SARS-CoV-2 Infection in a Cat with Hypertrophic Cardiomyopathy.

Coronavirus disease 19 (COVID-19), has claimed millions of human lives worldwide since the emergence of the zoonotic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in China in December 2019. Notably, most severe and fatal SARS-CoV-2 infections in humans have been associated with underlying clinical conditions, including diabetes, hypertension and heart diseases. Here, we describe a case of severe SARS-CoV-2 infection in a domestic cat (Felis catus) that presented with hypertrophic cardiomyopathy (HCM), a chronic heart condition that has been described as a comorbidity of COVID-19 in humans and that is prevalent in domestic cats. The lung and heart of the affected cat presented clear evidence of SARS-CoV-2 replication, with histological lesions similar to those observed in humans with COVID-19 with high infectious viral loads being recovered from these organs. The study highlights the potential impact of comorbidities on the outcome of SARS-CoV-2 infection in animals and provides important information that may contribute to the development of a feline model with the potential to recapitulate the clinical outcomes of severe COVID-19 in humans.