Acute Decompensated Heart Failure After Initiation of Amiodarone in a Patient With Anderson-Fabry Disease

A 54-year-old man with the lysosomal storage disorder Anderson-Fabry disease (AFD) and cardiac involvement was placed on amiodarone for treatment of symptomatic paroxysmal atrial fibrillation. Shortly thereafter, he developed symptoms of acute decompensated heart failure, requiring hospital admission. Endomyocardial biopsy demonstrated findings consistent with AFD and possible amiodarone toxicity. Amiodarone was discontinued, and the patient’s heart failure resolved with return to baseline status. Amiodarone is known to alter lysosomal pH and enzyme activity, and this case illustrates how it should be used with considerable caution in patients with AFD.     

Patterns of Care During the Terminal Hospital Admission for Patients With Advanced Heart Failure: A Retrospective Cohort Study

Costs of end of life care for patients who have advanced heart failure (HF) are increasing. There is a perception that many of these patients receive aggressive treatments near the end of life. However, actual patterns of care are unclear. In this article we describe the use of life-sustaining treatments and the timing of goals of care discussions during patients’ terminal admission for HF. We conducted a single-centre retrospective cohort study of patients aged 18 years or older with a most responsible discharge diagnosis of HF who died between April 2012 and December 2013. We identified 133 eligible decedents of whom 67 (50%) received some form of life-sustaining treatment, although only 14 (11%) received cardiopulmonary resuscitation (CPR). The first documented orders for scope of treatment were: CPR for 39 (29%), active medical treatment with no CPR for 81 (61%), and comfort care with no CPR for 11 (8%) patients. The last documented orders were for comfort care in 85 (64%) patients. There were 28 (21%) patients who received palliative care consultation. Median time between palliative care consultation and death was 6 days and between orders for comfort care and death was 24 hours. In contrast to the high mortality risk of our study cohort, palliative care consultation was often absent or in the final days of life, with orders for comfort-oriented care being written only 24 hours before death, suggesting there remain opportunities for earlier integration of palliative and goal-directed approaches to therapy for patients who have advanced HF.     

“NEW” Prehabilitation: A 3-Way Approach to Improve Postoperative Survival and Health-Related Quality of Life in Cardiac Surgery Patients

With advances in health care practices and delivery, the overall life expectancy of the Western population has increased. For those practitioners involved in the care of the patient with advanced cardiac disease, there has been a resultant higher prevalence of increasingly frail and older patients undergoing complex cardiac procedures. The higher rates of comorbid-associated higher vulnerability, with associated deconditioning, predisposes older, frail patients to poorer postoperative outcomes and a complicated recovery process after cardiac surgery. In addition, such patients experience inferior quality of life as a result of reduced ability to independently perform activities of daily living. During the preoperative waiting period, the cardiac symptoms and anxiety induces inactivity that in turn compounds the physical and mental deconditioning. To improve functional capacity and enhance postoperative recovery, prehabilitation, a component of the enhanced recovery after surgery model, might be of particular importance. In some studies, the preoperative improvement of the baseline physical, nutritional, and mental status has been reported to improve postoperative outcomes and enhance recovery after cardiac surgery. To address these domains, a 3-way approach to prehabilitation that is targeted toward improving nutritional status (N), exercise capacity (E) and worry reduction (W) (nutrition, exercise, and worry; “NEW” approach) might facilitate the perioperative management by ameliorating the postoperative outcomes and alleviating the surgical stress-related health deconditioning. In this review, the NEW approach and its potential benefits on postoperative outcomes as well as an implementation model (Promoting Action on Research Implementation in Health Services [PARiHS] framework) to aid institutional level implementation is described.     

Diabetes Mellitus and Cardiovascular Disease: An Evidence-Based Review of Provincial Formulary Coverage

Cardiovascular mortality is the primary cause of death in patients with type 2 diabetes mellitus (T2DM). Recently, clinical trials of the sodium-glucose transport protein 2 (SGLT-2) inhibitors empagliflozin and canagliflozin and of the glucagon-like peptide-1 (GLP-1) agonists liraglutide and semaglutide demonstrated that the agents reduced cardiovascular events. Furthermore, empagliflozin and liraglutide reduced cardiovascular mortality. However, despite the proven cardiac benefits, many but not all provincial formularies have restrictive rules for payment and access for SGLT-2 inhibitors and GLP-1 agonists. These restrictions impede a practitioner’s ability to provide optimal care for patients with T2DM and cardiovascular disease. The 2018 Diabetes Canada Guidelines recommend the use of a glucose-lowering agent with proven cardiovascular benefit (ie, empagliflozin, canagliflozin, liraglutide, or semaglutide) as second-line therapy after metformin, for patients with T2DM and cardiovascular disease who fail to achieve the glycemic target of A1C <7% with metformin. We recognize that provinces must allot resources especially when health care budgets are limited and not able to provide all available treatments. However, today we have glucose-lowering agents that reduce mortality in patients at very high cardiovascular risk. Furthermore, for empagliflozin, the cost effectiveness is highly favourable. Consequently, we urge provincial formularies to re-examine the access requirements for SGLT-2 inhibitors and to consider adding GLP-1 agonists use to reflect current evidence and clinical guideline recommendations.     

Cardiac Surgery in HIV Patients: State of the Art

The clinical status of HIV infection has changed dramatically with the introduction of combined antiretroviral therapy. Patients with HIV are now living long enough to be susceptible to chronic illnesses, such as coronary disease and nonischemic cardiomyopathy, which can be consequences of the combined antiretroviral therapy treatment itself. Cardiovascular diseases are a major source of morbidity and mortality in HIV-positive patients. Increasingly, such patients might be candidates for the full range of cardiac surgical interventions, including coronary bypass, valve surgery, and heart transplantation. There has been a shift from offering palliative procedures such as pericardial window and balloon valvuloplasty, to more conventional and durable surgical therapies in HIV-positive patients. We herein provide an overview of the contemporary outcomes of cardiac surgery in this complex and unique patient population. We review some of the ethical issues around the selection and surgical care of HIV-positive patients. We also discuss strategies to best protect the surgical treatment team from the risks of HIV transmission. Finally, we highlight the need for involvement of dedicated infectious disease professionals in a multidisciplinary heart team approach, aiming at the comprehensive care of these unique and complex patients.     

Prognostic Value of Right Ventricular Strain Using Speckle-Tracking Echocardiography in Pulmonary Hypertension: A Systematic Review and Meta-analysis

Background

Right ventricular (RV) strain imaging using speckle-tracking echocardiography (STE) is a quantitative method of assessing RV systolic function that has shown prognostic utility in patients with pulmonary hypertension (PH). However, its prognostic value for a large and mixed PH population remains poorly defined.

Shared Decision-Making as the Future of Emergency Cardiology

Shared decision-making is playing an increasingly large role in emergency cardiovascular care. Although there are many challenges to successfully performing shared decision-making in the emergency department, there are numerous clinical scenarios in which it should be used. In this article, we explore new research and emerging decision aids in the following emergency care scenarios: (1) low-risk chest pain; (2) new-onset atrial fibrillation; and (3) moderate-risk syncope. These decision aids are designed to engage patients and facilitate shared decision-making for specific treatment and disposition (admit vs discharge) decisions. We then offer a 3-step, practical approach to performing shared decision-making in the acute care setting, on the basis of broad stakeholder input and previous conceptual work. Step 1 involves simply acknowledging that a clinical decision needs to be made. Step 2 involves a shared discussion about the working diagnosis and the options for care in the context of the patient's values, preferences, and circumstances. The third and final step requires the patient and provider to agree on a plan of action regarding further medical care. The implementation of shared decision-making in emergency cardiology has the potential to shift the paradigm of clinical practice from paternalism toward mutualism and improve the quality and experience of care for our patients.     

Heart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care

There is mounting recognition that some of the most urgent problems of adult congenital heart disease (ACHD) are the prevention, diagnosis, and management of heart failure (HF). Recent expert consensus and position statements not only emphasize a specific and pressing need to tackle HF in ACHD (ACHD-HF) but also highlight the difficulty of doing so given a current sparsity of data. Some of the challenges will be addressed by this review. The authors are from 3 different centres; each centre has an established subspeciality ACHD-HF clinic and is able to provide heart transplant, multiorgan transplant, and mechanical support for patients with ACHD. Appropriate care of this complex population requires multidisciplinary ACHD-HF teams evaluate all possible treatment options. The risks and benefits of nontransplant ACHD surgery, percutaneous structural and electrophysiological intervention, and ongoing conservative management must be considered alongside those of transplant strategies. In our approach, advanced care planning and palliative care coexist with the consideration of advanced therapies. An ethos of shared decision making, guided by the patient’s values and preferences, strengthens clinical care, but requires investment of time as well as skilled communication. In this review, we aim to offer practical real-world advice for managing these patients, supported by scientific data where it exists.     

Remote Monitoring of Cardiac Implantable Electronic Devices

Over the past decade, technological advancements have transformed the delivery of care for arrhythmia patients. From early transtelephonic monitoring to new devices capable of wireless and cellular transmission, remote monitoring has revolutionized device care. In this article, we review the current evolution and evidence for remote monitoring in patients with cardiac implantable electronic devices. From passive transmission of device diagnostics, to active transmission of patient- and device-triggered alerts, remote monitoring can shorten the time to diagnosis and treatment. Studies have shown that remote monitoring can reduce hospitalization and emergency room visits, and improve survival. Remote monitoring can also reduce the health care costs, while providing increased access to patients living in rural or marginalized communities. Unfortunately, as many as two-thirds of patients with remote monitoring-capable devices do not use, or are not offered, this feature. Current guidelines recommend remote monitoring and interrogation, combined with annual in-person evaluation in all cardiac device patients. Remote monitoring should be considered in all eligible device patients and should be considered standard of care.     

Contemporary Emergency Department Management of Patients with Chest Pain: A Concise Review and Guide for the High-Sensitivity Troponin Era

This article synthesizes current best evidence for the evaluation of patients with suspected acute coronary syndrome (ACS) using high-sensitivity troponin assays, enabling physicians to effectively incorporate them into practice. Unlike conventional assays, high-sensitivity assays can precisely measure blood cardiac troponin concentrations in the vast majority of healthy individuals, facilitating the creation of rapid diagnostic algorithms. Very low troponin concentrations on presentation accurately rule out acute myocardial infarction (AMI) and enable the discharge of approximately 20% of patients after a single test, whereas an additional 30%-40% of patients can be safely discharged after short-interval serial sampling in as little as 1 or 2 hours. In contrast, highly abnormal troponin concentrations on presentation (more than 5 times the upper reference limit) or rapidly rising levels on serial testing can rapidly rule in AMI with high specificity. However, approximately one-third of patients remain in a biomarker-indeterminate “observation zone” even after serial sampling. These patients pose a disposition challenge to clinicians because although the differential diagnosis of elevated troponin concentrations is broad, these patients have an increased risk for short-term major adverse cardiac events. Use of repeated serial troponin sampling and structured clinical prediction tools may assist disposition for these patients, because no validated pathways currently exist to guide clinicians. Ongoing research to tailor diagnostic thresholds to individual patient characteristics may enable improved diagnostic accuracy and usher in a new era of personalized medicine in the evaluation of suspected ACS.     

Transcatheter Mitral Valve Intervention for Chronic Mitral Regurgitation: A Plethora of Different Technologies

Chronic mitral regurgitation (MR) remains a common cardiovascular condition resulting in significant morbidity and mortality. With an aging population, increasing trends for both primary (degenerative) and secondary (functional) MR have become apparent. Although the gold standard remains surgical intervention with mitral valve repair/replacement, comorbid conditions have steered the development of less invasive technologies to mitigate perioperative surgical risk. Transcatheter mitral valve repair using a percutaneous edge-to-edge technique is the most widely available choice at present. However, other transcatheter mitral valve repair techniques such as annuloplasty and chordal implantation are notable alternatives. Moreover, emerging technologies in transcatheter mitral valve replacement are rapidly establishing their roles in the field of chronic severe MR therapy. Hence, it is imperative to understand the indications and limitations of these various transcatheter mitral valve interventions to provide the best and most up-to-date clinical care for patients. This review will outline current evidence and patient selection criteria for such device-based therapies.     

Getting to the Heart of the Matter: A Review of Drug Interactions Between HIV Antiretrovirals and Cardiology Medications

The past 20 years have seen remarkable advances in the treatment of HIV such that most people diagnosed with HIV today can live long, healthy lives by taking antiretrovirals which are usually life-long. Advancements in antiretroviral therapy include the availability of well tolerated, single tablet regimens that are associated with a lower risk of drug-drug interactions. Despite this, many people living with HIV infection might be taking antiretroviral agents that are associated with significant drug-drug interactions. Because HIV infection itself is associated with cardiovascular complications and this population is living longer, concomitant use of antiretrovirals and medications to treat cardiovascular-related diseases is often required. For this reason, it is imperative that clinicians are aware of the potential for clinically significant drug-drug interactions between antiretroviral agents and cardiac medications as well as the useful HIV drug interaction resources that might provide guidance. Available data on significant interactions are summarized and suggested guidance regarding management is discussed.     

Novel Biomarkers for Predicting Cardiovascular Disease in Patients With Diabetes

It is generally acknowledged that patients with diabetes comprise a high-risk population for the development of cardiovascular disease. However, it is perhaps less well recognized that there actually exists considerable heterogeneity in vascular risk within this patient population, with a sizable subset of individuals seemingly at low risk for major cardiovascular events despite the presence of diabetes. Because traditional clinical risk calculators have shown wide variability in their performance in the setting of diabetes, there exists a need for additional risk predictors in this patient population. In this context, there has been considerable interest in the potential utility of circulating biomarkers as clinical tools that might facilitate risk stratification and thereby guide therapeutic and preventative decision-making. Coupled with the current era of dedicated cardiovascular outcome trials in type 2 diabetes, this interest has spawned a growing literature of recent studies that evaluated potential biomarkers. To date, these studies have identified N-terminal pro-B-type natriuretic peptide, high-sensitivity cardiac troponins, and growth differentiation factor-15 as cardiovascular biomarkers of particular potential in patients with diabetes. Furthermore, recognizing the potential benefit of collective consideration of different biomarkers reflecting distinct pathophysiologic processes that might contribute to the development of cardiovascular disease, there is emerging emphasis on the evaluation of combinations of biomarkers for optimal risk prediction. Although not currently ready for clinical practice, this rapidly-growing topic of biomarker research might ultimately facilitate the goal of individualized risk stratification and thereby enable truly personalized management of diabetes.     

Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology

Eisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease. A large nonrestrictive systemic left-to-right shunt triggers the development of pulmonary vascular disease, progressive pulmonary arterial hypertension, and increasing pulmonary vascular resistance at the systemic level, which ultimately results in shunt reversal. Herein, we review the changing epidemiological patterns and pathophysiology of Eisenmenger syndrome. Multiorgan disease is an integral manifestation of Eisenmenger syndrome and includes involvement of the cardiac, hematological, neurological, respiratory, gastrointestinal, urinary, immunological, musculoskeletal, and endocrinological systems. Standardized practical guidelines for the assessment, management, risk stratification, and follow-up of this very fragile and vulnerable population are discussed. Multidisciplinary care is the best clinical practice. An approach to the prevention and management of a broad spectrum of complications is provided. Relevant therapeutic questions are discussed, including anticoagulation, noncardiac surgery, physical activity, transplantation, and advanced-care planning (palliative care). Advanced pulmonary arterial hypertension therapies are indicated in patients with Eisenmenger syndrome and World Health Organization functional class II or higher symptoms to improve functional capacity, quality of life, and—less well documented—survival. Specific recommendations regarding monotherapy or combination therapy are provided according to functional class and clinical response. The ultimate challenge for all care providers remains early detection and management of intracardiac and extracardiac shunts, considering that Eisenmenger syndrome is a preventable condition.     

Canadian Cardiovascular Society/Canadian Heart Rhythm Society Joint Position Statement on the Cardiovascular Screening of Competitive Athletes

Sudden cardiac death (SCD), especially in a young seemingly healthy individual, is a tragic and highly publicized event, which is often followed by a strong emotional reaction from the public and medical community.” Although rare, SCD in the young is devastating to families and communities, underpinning our society’s desire to avoid any circumstances predisposing to the loss of human life during exertion. The Canadian Cardiovascular Society Position Statement on the cardiovascular screening of athletes provides evidence-based recommendations for Canadian sporting organizations and institutions with a focus on the role of routine electrocardiogram (ECG) screening in preventing SCD. We recommend that the cardiac screening and care of athletes within the Canadian health care model comprise a sequential (tiered) approach to the identification of cardiac risk, emphasizing the limitations of screening, the importance of shared decision-making when cardiac conditions are diagnosed, and the creation of policies and procedures for the management of emergencies in sport settings. Thus, we recommend against the routine (first-line or blanket mass performance of ECG) performance of a 12-lead ECG for the initial cardiovascular screening of competitive athletes. Organization/athlete-centred cardiovascular screening and care of athletes program is recommended. Such screening should occur in the context of a consistent, systematic approach to cardiovascular screening and care that provides: assessment, appropriate investigations, interpretation, management, counselling, and follow-up. The recommendations presented comprise a tiered framework that allows institutions some choice as to program creation.     

Challenges With Severe Coronary Artery Calcification in Percutaneous Coronary Intervention: A Narrative Review of Therapeutic Options

Coronary calcification often complicates atherosclerosis. With an aging population, coinciding with lower thresholds for coronary angiography and percutaneous coronary intervention (PCI), severe calcific coronary stenoses remain a challenge for interventional cardiologists. Although advances in coronary guidewires, percutaneous balloons, and adjunctive procedural devices have improved success of PCI, recalcitrant calcified lesions not amenable to the conventional technique frequently occur. Coronary atherectomy with plaque modification provides a therapeutic alternative. As such, various modalities such as rotational, orbital or laser atherectomy, and more recently shockwave lithoplasty have become therapeutic options for PCI. We provide a summary of the principles, technique, and contemporary evidence for these currently approved devices designed to treat severe coronary calcific lesions.     

Insights on Atrial Fibrillation in Congenital Heart Disease

Patients with congenital heart disease (CHD) have been surviving late into adulthood, with atrial arrhythmias being the most common long-term complication. In recent reports, atrial fibrillation (AF) tended to be the most common form of arrhythmias among groups of patients with adult CHD (ACHD) older than 50 years of age. When compared with their adult counterparts without CHD, AF in patients with ACHD has been characterized by a higher incidence and prevalence, younger age of onset, and a greater risk of progression to persistent AF. Risk factors for the development of AF are not well known but include older age, left atrial dilation, systemic hypertension, and multiple cardiac surgeries. Data on management options such as optimal antiarrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation are limited. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the growing ACHD patient population with AF. This report will provide a contemporary review of the epidemiology, pathophysiology, and management options for AF in this complex patient population.     

Why Do Patients With Well-Controlled Vascular Risk Factors Develop Progressive Chronic Kidney Disease?

Cardiovascular disease and chronic kidney disease (CKD) share several common risk factors, and CKD itself is an independent and graded risk factor for cardiovascular disease. Although control of vascular risk factors is associated with improved kidney outcomes, certain patients still show CKD progression, highlighting that examination of other factors is warranted. In this review we explore how blood pressure and glycemic targets appear to differ for macro- vs microvascular disease. Furthermore, factors such as obstructive sleep apnea and obesity are associated with CKD progression. There is increasing recognition of how sex, gender, ethnicity, and socioeconomic position all factor into CKD progression. Uncertainty exists as to what is the optimal diet to prevent loss of kidney function. Last, complications of CKD might directly or indirectly contribute to progression of kidney disease. In conclusion, control of vascular risk factors reduces the risk of CKD progression, and careful consideration of these additional factors might ultimately result in improved cardiovascular and CKD outcomes.     

Mechanisms of Arrhythmia and Sudden Cardiac Death in Patients With HIV Infection

Long-term survival of HIV-infected patients has significantly improved with the use of antiretroviral therapy (ART). As a consequence, cardiovascular diseases are now emerging as an important clinical problem in this population. Sudden cardiac death is the third leading cause of mortality in HIV patients. Twenty percent of patients with HIV who died of sudden cardiac death had previous cardiac arrhythmias including ventricular tachycardia, atrial fibrillation, and other unspecified rhythm disorders. This review presents a summary of HIV-related arrhythmias, associated risk factors specific to the HIV population, and underlying mechanisms. Compared with the general population, patients with HIV have several cardiac conditions and electrophysiological abnormalities. As a result, they have an increased risk of developing severe arrhythmias, that can lead to sudden cardiac death. Possible explanations may be related to non-ART polypharmacy, electrolyte imbalances, and use of substances observed in HIV-infected patients; many of these conditions are associated with alterations in cardiac electrical activity, increasing the risk of arrhythmia and sudden cardiac death. However, clinical and experimental evidence has also revealed that cardiac arrhythmias occur in HIV-infected patients, even in the absence of drugs. This indicates that HIV itself can change the electrophysiological properties of the heart profoundly and cause cardiac arrhythmias and related sudden cardiac death. The current knowledge of the underlying mechanisms, as well as the emerging role of inflammation in these arrhythmias, are discussed here.     

Telehealth for Remote Stroke Management

Stroke is a leading cause of adult disability and the fourth leading cause of death in Canada. Most strokes are ischemic and functional outcome is highly time-dependent, making fast diagnosis and treatment initiation crucial. This poses a challenge in vast geographical areas where stroke neurology expertise is only available in urban centres. In this article we review the rationale for telestroke networks and their current implementation in Canada. Telestroke networks enable stroke-specific procedures to be performed by less experienced physicians under the guidance of stroke neurology experts. We also present evidence that the safety and effectiveness of intravenous alteplase in community hospitals in a telestroke network seems to be comparable with that achieved in dedicated stroke centres. It is thus a viable option to guarantee an aging population access to stroke care across large geographic regions with faster treatment and access to more advanced treatment options by means of transfer to a comprehensive centre if necessary. Although telestroke networks have an upfront implementation cost, they can lead to reduced direct and indirect costs for the health care system by reducing days spent in the hospital as well as disability with the need for long-term care. Telestroke networks can also be used for identification and enrollment of patients into emergency stroke trials and thus provide a more representative sample of the population and increase recruitment. Standardization of regional telestroke networks could lead to collaborations with larger data acquisitions for research purposes and quality control in the future.