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.     

Atrial Fibrillation and Heart Failure: Untangling a Modern Gordian Knot

Heart failure (HF) and atrial fibrillation (AF) share common risk factors and frequently coexist. Both are highly prevalent in our aging population, and mortality associated with the combination is significantly higher than for each alone. An intricate link exists between AF and HF, including interrelated mechanisms and pathophysiology. Asymptomatic left ventricular systolic or diastolic dysfunction can exacerbate or be exacerbated by AF, resulting in HF with reduced ejection fraction or preserved ejection fraction. A number of treatment strategies have improved symptoms, exercise tolerance, and quality of life for patients with HF, but few have resulted in alteration in prognosis. Sinus rhythm, achieved pharmacologically, has not altered important outcomes, including cardiovascular or total mortality in patients with HF. In recent studies, catheter ablation to achieve sinus rhythm seems to have a significant impact on symptoms, heart function, and possibly mortality. Until future studies can confirm or clarify the impact of catheter ablation on outcomes, the field remains cautious but optimistic that better treatment strategies for patients with HF with reduced ejection fraction or preserved ejection fraction are within reach.     

Subclinical Inflammation in Heart Failure: A Neutrophil Perspective

Although it is widely recognized that inflammation plays a critical role in the development and pathology of heart failure (HF), very little is known about the involvement of one of the most abundant immune cells in the blood, a primary immune response cell: the neutrophil. This review summarizes the current literature on the role of subclinical inflammation, with a focus on the neutrophil in the pathophysiology of the HF syndrome. Some emerging therapeutic strategies are also discussed.     

The State of Affairs for Cardiovascular Health Research in Indigenous Women in Canada: A Scoping Review

Cardiovascular disease (CVD) is the leading cause of death among Indigenous peoples in Canada. As rates of CVD rise, the impacts among the growing population of Indigenous women will emerge as an important health issue. The objective of this scoping review was to advance the state of knowledge about cardiovascular health research in Indigenous women in Canada. Five databases and grey literature (non–peer reviewed works) were searched to identify all studies that reported on the prevalence, pathophysiology, diagnosis, treatment, or interventions for CVD among adult Indigenous women in Canada, including First Nations, Métis, and Inuit. Searching identified 3194 potential articles; 61 of which were included. The most commonly researched topics were the prevalence of CVD, hypertension, and dyslipidemia. Rates of CVD and associated mortality among Indigenous women appear to have surpassed those of their nonindigenous counterparts. Very little research has examined the pathophysiology, diagnosis, and treatment of CVD. Gaps in the research identified the need for sex-based analyses, comparison with nonindigenous women, comprehensive longitudinal data, assessment of diagnosis criteria, development and evaluation of cardiovascular health interventions, and a better understanding of the role of culture and traditions in the prevention and treatment of CVD among Indigenous women. Although comprehensive CVD data are lacking, rates of CVD among Indigenous women in Canada are rising and are nearing or surpassing those of nonindigenous women. This review serves as a call to action to seek further research on the pathophysiology, diagnosis, and treatment of CVD among Indigenous women from across Canada.     

Heart Failure With Preserved Ejection Fraction in Diabetes: Mechanisms and Management

Diabetes mellitus (DM) is a major cause of heart failure in the Western world, either secondary to coronary artery disease or from a distinct entity known as “diabetic cardiomyopathy.” Furthermore, heart failure with preserved ejection fraction (HFpEF) is emerging as a significant clinical problem for patients with DM. Current clinical data suggest that between 30% and 40% of patients with HFpEF suffer from DM. The typical structural phenotype of the HFpEF heart consists of endothelial dysfunction, increased interstitial and perivascular fibrosis, cardiomyocyte stiffness, and hypertrophy along with advanced glycation end products deposition. There is a myriad of mechanisms that result in the phenotypical HFpEF heart including impaired cardiac metabolism and substrate utilization, altered insulin signalling leading to protein kinase C activation, advanced glycated end products deposition, prosclerotic cytokine activation (eg, transforming growth factor-β activation), along with impaired nitric oxide production from the endothelium. Moreover, recent investigations have focused on the role of endothelial-myocyte interactions. Despite intense research, current therapeutic strategies have had little effect on improving morbidity and mortality in patients with DM and HFpEF. Possible explanations for this include a limited understanding of the role that direct cell-cell communication or indirect cell-cell paracrine signalling plays in the pathogenesis of DM and HFpEF. Additionally, integrins remain another important mediator of signals from the extracellular matrix to cells within the failing heart and might play a significant role in cell-cell cross-talk. In this review we discuss the characteristics and mechanisms of DM and HFpEF to stimulate potential future research for patients with this common, and morbid condition.     

The Spoke-Hub-and-Node Model of Integrated Heart Failure Care

Heart failure (HF) is a significant public health concern. Specialized HF clinics provide the optimal environment to address the complex needs of these patients and improve outcomes. The current and growing population of patients with HF outstrips the ability of these clinics to deliver care. Integrated care is defined as health services that are managed and delivered so that people receive a seamless continuum of health promotion, disease prevention, diagnosis, treatment, disease management, rehabilitation, and palliative care services. This approach requires coordination across different levels and sites of care within and beyond the health sector, according to changing patient needs throughout their lives. The spoke-hub-and-node (SHN) model represents an organization of care that works collaboratively with the primary care sector and is highly integrated with community-based multidisciplinary teams of health care professionals and specialty care. The purpose of this article is to analyze the requirements for successful implementation of SHN models. We consider the respective roles of HF clinics, HF nurse specialists, pharmacists, palliative care teams, telemonitoring, and solo practitioners. We also discuss levels of care delivery and the importance of patient stratification and patient flow. The SHN approach has the potential to build on and improve the chronic care model (CCM) to deliver centralized services to preserve high-quality patient-centred care at affordable costs.     

Optimizing Risk Stratification and Noninvasive Diagnosis of Ischemic Heart Disease in Women

Ischemic heart disease (IHD) is an important and previously underappreciated cause of significant morbidity and mortality in women. Key differences exist in the pathophysiology, sex-specific risk factors, and clinical presentation in women compared with men, which influence diagnostic accuracy and utility of pretest risk assessments and noninvasive testing. Women are disproportionately affected by ischemia from microvascular dysfunction as evidenced by having less obstructive coronary artery disease on angiography, contributing to the challenge in diagnosis and prognosis of IHD in women via conventional methods, which tend to emphasize detection of epicardial stenoses. In this article, we review the utility, evidence for, and challenges of currently available risk assessments and noninvasive cardiac diagnostic tests in women. We propose an approach to investigation of the symptomatic woman with suspected IHD and selection of the appropriate testing modality. Finally, we explore opportunities for future research and highlight the urgent need for updated, evidence-based, Canadian guidelines specific to women with IHD.     

Prediction of Early Adverse Events in Emergency Department Patients With Acute Heart Failure: A Systematic Review

Background

Acute heart failure (AHF) accounts for a substantial proportion of Emergency Department (ED) visits and hospitalizations. Previous studies have shown that emergency physicians' clinical gestalt is not sufficient to stratify patients with AHF into severe and requiring hospitalization vs nonsevere and safe to be discharged. Various prognostic algorithms have been developed to risk-stratify patients with AHF, however there is no consensus as to the best-performing risk assessment tool in the ED.

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.     

Arrhythmias and Sudden Cardiac Death in End Stage Renal Disease: Epidemiology,Risk Factors,and Management

Patients with end-stage renal disease (ESRD) are predisposed to heart rhythm disorders resulting in significant morbidity and mortality. Bradyarrhythmia appears to be more prevalent than ventricular tachyarrhythmias. There is also a high incidence of sudden cardiac death (SCD) in this group of patients, which cannot be explained only by traditional cardiac risk factors. The reported incidence and prevalence of arrhythmias and SCD is quite variable mainly because of the different study populations and recording techniques. The mechanism of SCD in patients with ESRD is also not clear. Although traditionally the thinking has been that ventricular arrhythmias are the main contributor to SCD, recent studies with implantable loop recorders have highlighted the role of bradyarrhythmias. The pathophysiological processes resulting in arrhythmia and SCD in patients with ESRD are unique. Some of the risk factors, including dialysate composition, timing, and frequency, are modifiable and hence provide an option for interventions to potentially reduce SCD. In addition, there might be a relationship with the timing of dialysis with SCD tending to occur during the long interdialytic period. Patients with ESRD have a higher likelihood of requiring pacemaker implantation; however, they also have a higher risk of device-related complications. The limited data available regarding the role of the implantable cardioverter defibrillator to prevent SCD in patients with ESRD have shown conflicting results. Future research is needed to develop appropriate risk stratification tools to identify patients who will benefit from such interventions and to assess their safety and efficacy.     

Automated 3-Dimensional Single-Beat Real-Time Volume Colour Flow Doppler Echocardiography in Children: A Validation Study of Right and Left Heart Flows

Background

Novel quantification of stroke volume according to mitral inflow and aortic outflow using automated real-time 3-dimensional volume colour flow Doppler echocardiography (3D-RT-VCFDE) is more accurate than 2-dimensional echocardiography and has excellent correlation with cardiac magnetic resonance imaging-based flows in adults. This technology is applied for the first time to the right heart and in children.

Effects of Riociguat on Right Ventricular Remodelling in Chronic Thromboembolic Pulmonary Hypertension Patients: A Prospective Study

Background

The effects of riociguat treatment on right ventricular (RV) metabolism, perfusion, and output in patients with chronic thromboembolic pulmonary hypertension (CTEPH) are unknown. In this study, RV changes associated with riociguat therapy were investigated.

Cardiac Complications Associated With Checkpoint Inhibition: A Systematic Review of the Literature in an Important Emerging Area

Background

Immune checkpoint inhibitors, including programmed cell death-1, programmed cell death ligand-1 and cytotoxic lymphocyte antigen-4 inhibitors, have emerged as important therapeutic alternatives for advanced malignancies. This drug class upregulates T-cell activity, leading to an immune response against cancer cells. However, the increased activity of T cells can lead to autoimmune reactions.

Anabolic Androgenic Steroid Use as a Cause of Fulminant Heart Failure

A 39-year-old male weightlifter presented in fulminant heart failure. An echocardiogram revealed severe global biventricular failure. Left ventricular (LV) systolic function was estimated at 15%. His dilated cardiomyopathy was attributed to his use of both testosterone and boldenone in the 3-month period before his presentation. Although the deleterious effects of androgenic- anabolic steroids on diastolic function are well known, the effects of these drugs on systolic function is an area of ongoing investigation. Our case suggests that androgenic anabolic steroid use should be considered in the differential diagnosis of patients presenting with acute heart failure.