Transition and Transfer From Pediatric to Adult Congenital Heart Disease Care in Canada: Call For Strategic Implementation

Dramatic increases in survival to adulthood for persons born with congenital heart disease (CHD) have led rise to a corresponding need to provide age-appropriate and developmentally appropriate care across the lifespan. Health care transition is a multidimensional process that ideally begins in early adolescence in the pediatric setting and continues through young adulthood with input from both pediatric and adult CHD providers. Preparation for transition includes the fostering of adolescents' knowledge of their CHD and of self-management and self-advocacy skills needed for lifelong management of chronic disease. Transfer is the event in time when a patient’s care and ownership of health records is taken over by the adult health care team; this is just one element of the broader transition process. Transfer typically occurs by age 18 throughout much of Canada. Successful transition is a shared responsibility, requiring engaged pediatric and adult providers and partnership with both young adults and their parents, all of whom may struggle with this process. An interdisciplinary approach to transition is recommended, given that health care transition is a complex process that occurs within the broader context of young adults’ lives. This review summarizes existing evidence regarding transition and transfer, offers perspectives from multiple stakeholders, and proposes a transition curriculum of development of CHD education and self-management and self-advocacy skills. Specific recommendations to improve implementation of transition and transfer care within the Canadian context are provided. This review sheds light on the current capacity and challenges of adult CHD providers and proposes directions to move this field forward.     

Variables Associated With Cardiac Surgical Waitlist Mortality From a Population-Based Cohort

Background

Cardiac surgery waitlist recommendations, which were developed based on expert opinion, poorly predict preoperative mortality. Studies reporting risk factors for waitlist mortality have not evaluated the risks including nonadherence to waitlist benchmarks.

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.     

Protein-Losing Enteropathy Following Fontan Palliation

Protein-losing enteropathy (PLE) is a chronic condition involving multiple organ systems that may develop any time following Fontan completion. The pathogenesis of PLE is complex and multifactorial. Chronic venous hypertension, low cardiac output, and abnormal lymphatics may all play a role in the pathogenesis of PLE. Common signs and symptoms include chronic diarrhea, abdominal pain, and ascites. Diagnosis is based on the presence of signs and symptoms in addition to hypoalbuminemia and elevated stool alpha 1 antitrypsin. Early identification and a comprehensive approach to evaluation and treatment are important, as they may affect survival. The initial evaluation should include cardiac catheterization for hemodynamic assessment. Although an evidence base for treatment is lacking, various medical, interventional, and surgical approaches have been described with variable degrees of success. Commonly used therapies include nutritional support, diuretics, subcutaneous unfractionated heparin, budesonide, and sildenafil. Limited data exist for Fontan conversion or takedown. Assessment for heart transplantation should be considered. PLE mortality is high—approximately 50%—but may be mitigated by aggressive investigation and management. The evolving understanding of the role of lymphatics in the pathophysiology of PLE and the emerging role of interventional lymphatic procedures may further improve outcomes in this patient population.     

Acute Coronary Syndromes and Heart Failure Critical Care Units Utilization and Outcomes in Teaching and Community Hospitals: A National Population-Based Analysis

Acute coronary syndromes (ACS) and heart failure (HF) are the leading diagnoses in patients admitted to critical care units (CCUs). Little is known about the differences between CCU resource use and outcomes across hospital types. The Canadian Institute for Health Information was used to identify patients hospitalized with primary diagnoses of ACS or HF. CCUs were categorized as teaching, large community, medium community, and small community hospitals. Outcomes included CCU rates of admission, use of critical care therapy/procedures, and in-hospital mortality. Among 204,900 patients hospitalized with ACS or HF, 73,338 (35.8%, hospital range 0% to 81.4%) were admitted to CCUs, and it varied across hospital types: 41.0% in teaching, 30.0% in large, 45.4% in medium, and 30.9% in small community hospitals (P < 0.001). The percentage of patients admitted to CCUs who received critical care therapies in teaching, large, medium, and small hospitals were as follows: 73.6%, 50.9%, 24.6%, and 8.8% (P < 0.0001). Compared with the in-hospital mortality rate for patients admitted to CCUs in teaching hospitals (8.2%), outcomes were worse for CCU patients in large (11.0%, adjusted odds ratio [aOR] 1.50; 95% CI, 1.19-1.90), medium (10.5%, aOR 1.56; 95% CI, 1.27-1.92), and small community hospitals (9.2%, aOR 1.59; 95% CI, 1.20-2.10). Patients admitted with ACS or HF to teaching hospital CCUs had a higher observed use of critical care therapies and lower mortality compared with community hospitals. These differences highlight the need to examine differences in CCU admission thresholds, resource utilization, and outcomes across hospitals types.     

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.     

A Population-Based Study of Adherence to Guideline Recommendations and Appropriate-Use Criteria for Implantable Cardioverter Defibrillators

Studies evaluating physician adherence to guideline recommendations for implantable cardioverter defibrillator (ICD) therapy are sparse, and none exist for the application of appropriate-use criteria (AUC) in clinical practice. As part of a quality improvement initiative, a review of all ICD procedures was performed from January 1, 2015 to December 31, 2016 in Alberta, Canada, to evaluate the proportion of patients receiving appropriate ICD therapy and to identify reasons for nonadherence. Our device-implant process involves an electrophysiologist or implanting cardiologist evaluation, reminders of ICD eligibility criteria on the device requisition, and peer-review consensus. Implants were classified according to the 2008 American College of Cardiology/American Heart Association/Heart Rhythm Society (ACC/AHA/HRS) ICD guidelines, 2013 Canadian Cardiovascular Society (CCS) Cardiac Resynchronization Therapy (CRT) guidelines, and 2013 AUC. There were 1,300 ICD procedures performed, and the mean age was 63.8 ± 12.9 years; 79% were male; the mean ejection fraction was 0.32 ± 0.13, and 69% were for primary prevention. Among all implants, < 1% were discordant with American College of Cardiology/American Heart Association/Heart Rhythm Society (ACC/AHA/HRS) recommendations. Among CRT implants, 10% were inconsistent with Canadian Cardiovascular Society (CCS) recommendations. According to AUC, 92% of implants were appropriate. Reasons for nonadherence to ACC/AHA/HRS recommendations included QRS width < 120 msec (n = 3), LVEF > 0.35 (n = 2) and recent myocardial infarction (MI) (n = 1). The most common reason for nonadherence to AUC was the absence of criteria for classification (n = 57, 4%). In this population-based study, we found that a process of specialist evaluation, eligibility reminders on device forms, and peer-review consensus may improve adherence to guideline recommendations and AUC for ICD therapy.     

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.     

Decision Support Tools: Realizing the Potential to Improve Quality of Care

Delivering evidence-based, personalized care that engages patients requires profound changes in the structure, process, and organization of care, along with revised incentives to support such changes. Health care providers must absorb and apply a vast, usually overwhelming, amount of scientific information to provide high-quality patient care. Accordingly, care remains inconsistent, with unintentional adverse consequences. Decision support tools can provide patient-specific assessments that support clinical decisions, improve prescribing practices, reduce medication errors, improve the delivery of primary as well as secondary prevention, and improve adherence to standards of care. Decision support tools are created using an individual patient’s genetic, sociodemographic, and clinical characteristics to improve the delivery of precise, personalized care. Implementation requires ease of use for busy clinicians; uptake improves with active education, and gradual adoption of a tool integrated into care processes without disrupting clinical work flow. As health care systems continue to evolve and computerized support increases, increased implementation of decision support tools that are provided automatically as part of usual work flow, with clinically actionable recommendations at the point of care, requiring accountability for deviations from recommended therapy, represent an important opportunity to enhance quality of care by tailoring treatment to risk, improving the consistency of health care delivery, increasing patient knowledge and engagement, and avoiding specific therapeutic interventions in patients who will receive no benefit. However, successful implementation also requires strategies to engage providers in accepting and using these tools to improve care.     

Cardiovascular Efficacy and Safety of PCSK9 Inhibitors: Systematic Review and Meta-analysis Including the ODYSSEY OUTCOMES Trial

Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors are efficacious lipid-lowering agents, but more precise estimates of their effects on major adverse cardiovascular events (MACE), mortality, and safety are needed. We systematically reviewed and meta-analyzed randomized controlled trials with durations ≥ 6 months comparing MACE, mortality, and safety with PCSK9 inhibitors vs control. We searched CENTRAL, Embase, MedLine and the grey literature to November 7, 2018. From 2048 articles, we included 23 trials (n = 60,723). PCSK9 inhibitors reduced MACE (relative risk, 0.83; 95% confidence interval, 0.78-0.88), but did not clearly reduce mortality (relative risk, 0.93; 95% confidence interval, 0.85-1.02) or increase adverse events. In conclusion, PCSK9 inhibitors reduce nonfatal MACE, are well tolerated, but effects on mortality remain unclear.     

Geographical Differences in Comorbidity Burden and Outcomes in Adults With Syncope Hospitalizations in Canada

A recent study found that rates of hospitalization for syncope vary across provinces; however, it is unknown whether differences in comorbidity burden and outcomes also exist. The Canadian Institute for Health Information Discharge Abstract Database was used to identify primary syncope hospitalizations (ICD-10 code R55) from 2004 to 2013 for all provinces (except Quebec). Charlson comorbidity score was calculated from comorbidities at the time of hospitalization. Outcomes were defined as in-hospital mortality, 30-day readmission for any cause, and syncope. Logistic regression models were constructed for odds ratios (ORs) and 95% confidence intervals (CIs) to estimate interprovincial differences in outcomes. The interprovincial range (IPR) for mean age was 61.1 ± 17.5 to 73.7 ± 16.3 years, and at least half were male patients. There were significant differences in comorbidity burden across provinces (P < 0.01); however, the majority of patients had a Charlson comorbidity score = 0 (IPR, 53.9%- 71.9%). In multivariable analysis, compared with Ontario, in-hospital mortality was higher for British Columbia (OR, 1.59; 95% CI, 1.22-2.06), Nova Scotia (OR, 1.67; 95% CI, 1.05-2.65), and Newfoundland (OR, 2.27; 95% CI, 1.29-4.00); 30-day readmission for any cause was higher for British Columbia (OR, 1.15; 95% CI, 1.06-1.26), Alberta (OR, 1.19; 95% CI, 1.07-1.31), Manitoba (OR, 1.36; 95% CI, 1.18-1.56), and Prince Edward Island (OR, 1.38; 95% CI, 1.0-1.89), and all outcomes were higher in Saskatchewan. There is significant interprovincial heterogeneity in comorbidity burden and outcomes for hospitalizations for syncope. Future research evaluating whether standardized practices for management of syncope reduce variability and improve healthcare utilization and costs is needed.     

Resource Use and Burden of Hospitalization,Outpatient, Physician,and Drug Costs in Short- and Long-term Care After Acute Myocardial Infarction

Background

Little is known about the resource use and cost burden of acute myocardial infarction (AMI) beyond the index event. We examined resource use and care costs during the first and each subsequent year, among patients with incident AMI.

Cardiorenal Syndrome and Heart Failure—Challenges and Opportunities

Cardiorenal syndromes (CRS) describe concomitant bidirectional dysfunction of the heart and kidneys in which 1 organ initiates, perpetuates, and/or accelerates decline of the other. CRS are common in heart failure and universally portend worsened prognosis. Despite this heavy disease burden, the appropriate diagnosis and classification of CRS remains problematic. In addition to the hemodynamic drivers of decreased renal perfusion and increased renal vein pressure, induction of the renin-angiotensin-aldosterone system, stimulation of the sympathetic nervous system, disruption of balance between nitric oxide and reactive oxygen species, and inflammation are implicated in the pathogenesis of CRS. Medical therapy of heart failure including renin-angiotensin-aldosterone system inhibition and β-adrenergic blockade can blunt these deleterious processes. Renovascular disease can accelerate the progression of CRS. Volume overload and diuretic resistance are common and complicate the management of CRS. In heart failure and CRS being treated with diuretics, worsening creatinine is not associated with worsened outcome if clinical decongestion is achieved. Adjunctive therapy is often required in the management of volume overload in CRS, but evidence for these therapies is limited. Anemia and iron deficiency are importantly associated with CRS and might amplify decline of cardiac and renal function. End-stage cardiac and/or renal disease represents an especially poor prognosis with limited therapeutic options. Overall, worsening renal function is associated with significantly increased mortality. Despite progress in the area of CRS, there are still multiple pathophysiological and clinical aspects of CRS that need further research to eventually develop effective therapeutic options.     

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.     

Quality of Cardiac Care in Canada: Recommendations for Building a Sustainable Future

Cardiovascular (CV) disease continues to present a significant disease and economic burden in Canada. To improve the quality of care and ensure sustainability of services, a national quality improvement initiative is required. The purpose of this analysis was to review the evidence for public reporting (PR) and external benchmarking (EB) to improve patient outcomes, and to recommend a strategy to improve CV care in Canada. To incorporate recent literature, the Canadian Cardiovascular Society (CCS) commissioned the Institute of Health Economics to provide a rapid update on the literature of PR and EB. The review showed that EB is more likely to promote positive effects, such as improved mortality, morbidity, and evidence-based clinical practice, and to limit negative effects, such as access restrictions or unintended provider behaviour associated with some forms of “top-down” PR. On the basis of these findings, this we recommend the following: (1) secure funding for the provincial collection of CV quality indicators and the creation of annual National CV Quality Reports; (2) enhance the culture of using CV quality indicator data for continuous quality improvement and opportunities for national or regional EB and sharing best practices; and (3) implement ongoing evaluation and revision of CCS clinical practice guidelines incorporating key quality indicators. This is already under way to a limited extent by the CCS with its Quality Project, but intentional, sustained support needs to be secured to enhance this ongoing effort and improve the quality of CV care for all Canadians.