Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Qualitative study of facilitators and barriers for continued follow-up care as perceived and experienced by young people with congenital heart disease in Sweden.

In-depth understanding of factors perceived by young people with congenital heart disease (CHD) to affect continued follow-up care is needed to tailor preventive strategies for discontinuation of follow-up care. To identify facilitating factors, low-prevalence settings in terms of discontinuation should be investigated. OBJECTIVE: This qualitative study describes factors affecting continued follow-up as perceived and experienced by young adults with CHD. PARTICIPANTS: Using a mixed purposive sampling technique, 16 young adults with CHD were included. Three participants had discontinued follow-up care and 13 had continued follow-up care after transfer. SETTING: Participants were recruited from all seven university hospitals in Sweden, which is considered a low-prevalence setting in terms of discontinuation. DESIGN: Individual interviews were performed and subjected to qualitative content analysis. RESULTS: The analysis resulted in three main categories, illuminating factors affecting continued follow-up: (1) motivation for follow-up care; (2) participation in care and sense of connectedness with healthcare provider (HCP) and (3) care accessibility. The choice of continuing follow-up or not was multifactorial. Knowledge of your CHD and the importance of continuing follow-up care was a central factor, as well as experiencing CHD-related symptoms and having ongoing or planned medical treatment or interventions. Sensing a clear purpose with follow-up care was facilitating, as was feeling well treated and cared for by HCPs. Practical aspects, such as travel distance was also stressed, as well as active invitations and reminders for visits. CONCLUSION: Factors on both patient, hospital and healthcare system level were raised by participants, stressing the importance of holistic approaches when developing preventive strategies for discontinuation. There is a need for improved skills and competencies among HCPs, as well as a person-centred approach to follow-up care. In addition, specific healthcare needs and remaining transitional needs after transfer to adult care require careful consideration to prevent discontinuation.

Recommendations for advance care planning in adults with congenital heart disease: a position paper from the ESC Working Group of Adult Congenital Heart Disease, the Association of Cardiovascular Nursing and Allied Professions (ACNAP), the European Association for Palliative Care (EAPC), and the International Society for Adult Congenital Heart Disease (ISACHD).

Survival prospects in adults with congenital heart disease (CHD), although improved in recent decades, still remain below expectations for the general population. Patients and their loved ones benefit from preparation for both unexpected and predictable deaths, sometimes preceded by a prolonged period of declining health. Hence, advance care planning (ACP) is an integral part of comprehensive care for adults with CHD. This position paper summarizes evidence regarding benefits of and patients' preferences for ACP and provides practical advice regarding the implementation of ACP processes within clinical adult CHD practice. We suggest that ACP be delivered as a structured process across different stages, with content dependent upon the anticipated disease progression. We acknowledge potential barriers to initiate ACP discussions and emphasize the importance of a sensitive and situation-specific communication style. Conclusions presented in this article reflect agreed expert opinions and include both patient and provider perspectives.

Effects of Massage on Postoperative Pain in Infants With Complex Congenital Heart Disease.

BACKGROUND: Pain management is an essential component of care for pediatric patients following surgery. Massage reduces self-reported postoperative pain in adults with heart disease but has received little attention in postoperative pediatric patients with complex congenital heart disease (CCHD). OBJECTIVES: The aim of the study was to evaluate the effectiveness of massage compared to a rest period on postoperative pain scores and physiological responses in infants with CCHD. METHODS: We used a two-group randomized clinical trial design with a sample of 60 infants with CCHD between 1 day and 12 months of age following their first cardiothoracic surgery. Both groups received standard postoperative care. Group 1 received a daily 30-minute restriction of nonessential caregiving (quiet time), and Group 2 received a daily 30-minute massage. Interventions continued for seven consecutive days. Pain was measured six times daily using the Face, Legs, Activity, Cry, Consolability Pain Assessment Tool (FLACC). Average daily doses of analgesics were recorded. Heart rates (HRs), respiratory rates (RRs), and oxygen saturations (SpO2) were recorded continuously. Daily averages, pre- and postintervention FLACC scores, and physiological responses were analyzed using descriptive statistics, generalized linear mixed models repeated measures, latent growth models, and/or regression discontinuity analysis. Fentanyl-equivalent narcotic values were used as a time-varying covariate. RESULTS: Adjusted pain scores were lower for the massage group on all days except Day 7. Overall, there were no group effects on level of pain or differential rate of change in pain. However, the massage group had lower daily pain scores with small to medium effect size differences, largest at Days 4, 5, and 6, and lower average daily HR and RR. There was little difference between groups in SpO2. Infants demonstrated immediate effects of massage, with HR and RR decreasing and oxygen saturations increasing. DISCUSSION: This study provides beginning evidence that postoperative massage may reduce pain and improve physiological parameters in infants with congenital heart disease. This nonpharmacological adjunct to pain management may provide a particular benefit for this population by reducing demand on the cardiorespiratory system.

Access and Delivery of Adult Congenital Heart Disease Care in the United States: Quality-Driven Team-Based Care.

The landscape of congenital heart disease has changed rapidly over the past few decades. The shift from pediatric to adult congenital heart disease care has stretched resources and the ability to provide high-quality access and delivery of care for the more than 1.5 million adults with congenital heart disease in the United States. Meeting the demand for delivering high-quality care requires a team-based approach, with each member highly specialized. This review describes the deficits and deficiencies in providing care for adults with congenital heart disease in the United States and a team-based approach to improving access and delivery of care.

Molecular Approaches in Single Ventricle Management.

Advances in medical and surgical management have significantly improved early outcomes in single ventricle congenital heart disease over the last 2 decades. Despite these advances, long-term outcomes remain suboptimal and therapeutic options to address systemic ventricular and/or Fontan failure are limited even in the modern era. Intricate molecular biologic techniques have shed light into the mechanisms of development of single ventricle disease. Efforts are underway to leverage this knowledge to improve clinical diagnosis, therapy, and prognostication. Cell-based therapies aimed at inducing cardiomyocyte proliferation and preventing delayed cardiac dysfunction have already entered the clinical realm. Several more novel biological therapies are expected to become available for patients with single ventricle disease in the near future. These scientific advancements provide us hope and reaffirm our faith that molecular medicine will usher in the next generation of therapies for single ventricle management.

Longitudinal Improvements in Radiation Exposure in Cardiac Catheterization for Congenital Heart Disease: A Prospective Multicenter C3PO-QI Study.

BACKGROUND: The C3PO-QI (Congenital Cardiac Catheterization Project on Outcomes - Quality Improvement), a multicenter registry launched in 2015, instituted quality improvement (QI) initiatives to reduce patient radiation exposure. Through regular collaboration, this initiative would allow for harmony among active participants, maximizing efforts and efficiency at achieving radiation best practices. This study sought to report these efforts with a detailed methodology for which institutions can target initiatives, reducing radiation exposure, and increasing patient safety. METHODS: Data were collected prospectively by 8 C3PO-QI institutions between January 1, 2015 and December 31, 2017. Radiation exposure was measured in dose area product per body weight (dose area product/kg; µGy*m2/kg) and reported by expected radiation exposure categories (REC) and institution for 40 published unique procedure types. Targeted interventions addressing selected strategic domains for radiation reduction were implemented in the pediatric catheterization labs of the C3PO-QI institutions. RESULTS: The study consisted of 15 257 unique cases. Median exposure (dose area product/kg) was decreased by 30% for all procedures. Dose area product/kg was reduced in all 3 REC, with the greatest improvement observed in REC I (REC I, -37%; REC II, -23%; REC III, -27%). Although the baseline radiation exposures and exact percent decrease varied across all C3PO-QI sites, each institution demonstrated improvements in radiation dose over time. These improvements occurred with the implementation of institution-specific QI interventions accelerated by participation in the C3PO-QI multicenter collaborative. CONCLUSIONS: Substantial radiation dose reductions can be achieved using targeted QI methodology and interventions. Participation in a multicenter QI collaborative may accelerate improvement across all centers due to enhanced engagement and shared learning between sites.

Management of the adult Fontan patient.

Adults with congenital heart disease who underwent the Fontan operation to palliate single ventricle heart defects-by direct connection of caval venous return to the pulmonary circulation-have improved survival due to advancements in surgical and interventional techniques. However, cardiac and non-cardiac comorbidities often coexist, complicating management, and contributing to premature morbidity and mortality. Cardiac issues include heart failure symptoms related to systolic and diastolic dysfunction, atrial and ventricular arrhythmias and systemic atrioventricular valve regurgitation. Structural issues may be related to obstruction of the Fontan pathway, or to branch pulmonary artery stenosis, both of which exacerbate symptoms. Non-cardiac complications in adults involve hepatic congestion, fibrosis and cirrhosis, hepatocellular carcinoma, chronic kidney disease, stroke, venous stasis, lymphatic issues and involvement of other organ systems. 'Fontan failure' refers to circulatory dysfunction, either cardiac, non-cardiac, or both, diagnosed after exclusion or treatment of reversible contributors such as structural issues and arrhythmias. Counselling about reproductive health, mental health, perioperative management and overall wellness are paramount for patients' well-being. Fontan patients are typically managed in highly specialised adult congenital heart centres, but may present to cardiologists or other practitioners with cardiac and non-cardiac emergencies or urgencies, sometimes after being out of care. In this review, we discuss the management of the adult Fontan patient, including surveillance, cardiac and non-cardiac complications, reproductive health, and advanced therapies.

Improving growth of infants with congenital heart disease using a consensus-based nutritional pathway.

OBJECTIVE: Infants with congenital heart disease (CHD) often experience growth failure prior to surgery, which is associated with increased paediatric-intensive-care unit length of stay (PICU-LOS) and post-operative complications. This study assessed the impact of a pre-operative, consensus-based nutritional pathway (including support from a multi-disciplinary team) on growth and clinical outcome. DESIGN: Single-centre prospective pilot study. SETTING: Tertiary paediatric cardiology surgical centre. PATIENTS: Infants with CHD. INTERVENTION: Infants with CHD were followed for up to 4-months-of-age before cardiac surgery and then to 12-months-of-age following the implementation of the consensus-based nutritional-pathway (Intervention group: November 2017-August 2018), with outcomes compared to a historic control group. The nutrition pathway involved a dietitian contacting parents of infants with the highest risk of growth failure weekly; reviewing weight gain and providing feeding support. MAIN OUTCOME MEASURE: Growth (weight-for-age, WAZ, and height-for-age-z-score, HAZ) at 4 and 12 months-of-age. RESULTS: 44 infants in the intervention group were compared to 38 in the control group. Median (inter quartile range) change in WAZ from birth to 4 months-of-age (-0.9 (-1.5, 0.7)) and from birth to 12 months-of-age (-0.09 (-1.3, 1.1)) in the intervention group compared to the control group (-1.5 (-2.0, -0.4) (p = 0.04)) at 4 months-of age and at 12 months-of-age (-0.4 (1.9, 0.2) (p = 0.03)). HAZ at 4 months-of-age was -0.7 (-1.4, -0.1) vs. -1.0 (-1.9, -0.3) (p = 0.6) in the intervention and control groups respectively, and at 12 months-of-age HAZ was -0.7 (-1.9, -0.07) in the intervention group vs.-1.6 (-2.6, -0.4) in the control group (p = 0.04). Duration of PICU-LOS was 8.2 ± 11.6 days intervention vs. 18.3 ± 24.0 days control (p = 0.006). CONCLUSION: Overall weight was well maintained and growth improved in infants who followed the pre-operative nutritional-pathway. The duration of PICU-LOS was significantly lower in the intervention group, which may be due to improved nutritional status, although this requires further investigation.

Spiritually Sensitive Intervention to Mitigate Depressive Symptoms Among Hospitalized Children Affected With Congenital Heart Diseases: Insights for Holistic Pediatric Nursing.

This article reports on an experiment in 28 pediatric hospitals across 14 African and Asian cities. The aim was to examine the effect of a customized spiritually sensitive intervention (SSI) on mitigating depressive symptoms among hospitalized children with congenital heart diseases (CHD). Results showed that post-SSI, 1,139 treatment group children were less depressed vis-à-vis the control group. The SSI was more effective for children from African cities, boys, middle class, Christians and Buddhists, those undergoing medical care for ventricular septal defects, children who stayed in the hospital for 2 to 3 weeks and who regularly self-practiced the lessons after hospital discharge. The SSI could be effectively used for holistic pediatric nursing and health care across contexts with CHD affected children, with some culture-specific variations for greater impact.

Alternative approach to pediatric cardiac quality assessment for low-volume centers.

BACKGROUND: In pediatric cardiac care, many centers participate in multiple, national, domain-specific registries, as a major component of their quality assessment and improvement efforts. Small cardiac programs, whose clinical activities and scale may not be well-suited to this approach, need alternative methods to assess and track quality. METHODS: We conceived of and piloted a rapid-approach cardiac quality assessment, intended to encompass multiple aspects of the service line, in a low-volume program. The assessment incorporated previously identified measures, drawn from multiple sources, and ultimately relied on retrospective chart review. RESULTS: A collaborative, multidisciplinary team formed and came to consensus on quality metrics pertaining to 3 chosen areas of clinical activity in the program. Despite the use of multiple different data sources and the need for manual chart review in data collection, a rich assessment of these program components was completed for presentation in 6 weeks. CONCLUSIONS: While small programs may not participate in the spectrum of cardiac care registries available, these same centers can benefit from them by adapting some of their validated metrics for use in internal, self-maintained quality reports. Our pilot of this alternative approach revealed opportunities for improved quality assessment practices; the product can serve as a baseline for future prospective assessment and reporting, as well as longitudinal internal benchmarking.

[Relevant aspects of the ESC guidelines for the management of cardiovascular diseases during pregnancy for obstetric anaesthesia (update 2018)]. / Anästhesiologisch relevante Aspekte der ESC-Leitlinien für das Management von kardiovaskulären Erkrankungen während der Schwangerschaft (Update 2018).

The current update of the ESC (European Society of Cardiology) guidelines on managing cardiovascular diseases during pregnancy provides instructions for doctors in daily practice. Heart diseases are the most common reason for maternal death during pregnancy in western countries. Among other things, the following topics are dealt with: congenital heart disease, pulmonary hypertension, aortic and valvular diseases as well as arrhythmias and hypertensive disorders. Compared to the guidelines from 2011 some changes have been made regarding the recommendations to classify maternal risk according to the modified World Health Organization (mWHO) classification or in recommendations on anticoagulation for low-dose and high-dose requirements of vitamin K antagonists. The main focus of this summary of recent recommendations is the impact on the anesthesia management in order to provide responsible anesthesiologists with relevant background knowledge.

Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory.

OBJECTIVES: The purpose of this study was to evaluate the association between the method of procedural sedation and outcomes for congenital cardiac catheterization procedures. BACKGROUND: The safety of operator-directed sedation (ODS) in the pediatric/congenital cardiac catheterization laboratory has been questioned. To our knowledge, the relative safety of ODS versus general anesthesia (GA) in these cases has not to date been critically evaluated. METHODS: A single-center retrospective cohort study was performed to compare the relative safety, cost, and times of catheterization procedures performed with ODS and those performed with GA from a cardiac anesthesiologist. The risk of adverse outcomes was compared using propensity-score-adjusted models. Using the same propensity score, procedure times and relative charges were also compared. RESULTS: Over the study period, 4,424 procedures in 2,547 patients were studied. Of these, 27% of cases were performed with ODS. ODS procedures were 70% diagnostic procedures, 17% device closure of patent ductus arteriosus, 5% balloon pulmonary valvuloplasty, and 3% pulmonary artery angioplasty. The risk of adverse event in adjusted models for ODS cases was significantly lower than in GA cases (odds ratio: 0.66; 95% confidence interval: 0.45 to 0.95; p = 0.03). Total room time and case time were also significantly shorter (p < 0.001). Professional (charge ratio: 0.88; p < 0.001) and hospital (charge ratio: 0.84; p < 0.001) charges for ODS cases were also lower than those for GA cases. CONCLUSIONS: This study demonstrates that clinical judgment can identify subjects in whom ODS is not associated with increased risk of adverse events. The use of ODS was associated with reduced case times and charges. In combination, these findings suggest that the selective use of ODS can allow for greater efficiency and higher value care without sacrificing safety.

Improving health perception through a transition care model for adolescents with congenital heart disease.

AIMS: The aim of this study was to assess the impact of a transition clinic model on adolescent congenital heart disease (CHD) patients' health perception outcomes. The transition clinic model consists of multidisciplinary standardized interventions to educate and support CHD patients and represents a key element in the adequate delivery of care to these individuals during their transition from childhood to adulthood. Currently, empirical data regarding the impact of transition clinic models on the improvement of health perceptions in CHD adolescent patients are lacking. METHODS: A quasi-experimental design was employed. Quality of life, satisfaction, health perceptions and knowledge were assessed at the time of enrolment (T0) and a year after enrolment (T1), respectively. During the follow-up period, the patients enrolled (aged 11-18 years) were involved in the CHD-specific transition clinic model (CHD-TC). RESULTS: A sample of 224 CHD adolescents was enrolled (60.7% boys; mean age: 14.84 ±â€Š1.78 years). According to Warnes' classification, 22% of patients had simple heart defect, 56% showed moderate complexity and 22% demonstrated severe complexity. The overall results suggested a good impact of the CHD-TC on adolescents' outcomes, detailing in T1 the occurrence of a reduction of pain (P < 0.001) and anxiety (P < 0.001) and an improvement of knowledge (P < 0.001), life satisfaction (P < 0.001), perception of health status (P < 0.001) and quality of life (P < 0.001). CONCLUSION: The CHD-TC seems to provide high-quality care to the patient by way of a multidisciplinary team. The results of the present study are encouraging and confirm the need to create multidisciplinary standardized interventions in order to educate and support the delivery of care for CHD adolescents and their families.

Advanced care planning in adult congenital heart disease: Transitioning from repair to palliation and end-of-life care.

As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2]. The scientific adult CHD (ACHD) community has been working hard to identify variables related to worse outcomes, modifying those where possible in order to improve survival. Indeed, survival in adults with CHD has increased, but consequently, on top of CHD-related complications, patients are increasingly exposed to the standard cardiovascular risk factors. Therefore, a program for lifelong coaching on health behavior and life style management becomes indispensable. More emerging is that a substantial number of patients, in particular those with complex heart defects, will eventually end up in a stage with hardly any medical or interventional options left. Our healthcare provision has to be prepared to organize care for this specific group of patients who will die prematurely and require the timely development and establishment of advanced care planning. Advanced care planning should preferentially be set-up in expert CHD centers. The long-lasting relationship in ACHD care with healthcare providers offers an excellent basis with regards to prognosis, advanced care planning and end-of-life issues.

Historical investigation of medical treatment for adult congenital heart disease: A Canadian perspective.

OBJECTIVE: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time? DESIGN/METHODS: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order to articulate a contextualized understanding of the evolution of adult CHD treatment in Canada. The informants recruited were currently or previously located in and/or involved in treatment in seven provinces and two territories across Canada including patients, families, advocates, researchers, and practitioners located in disciplines that included cardiology, cardiovascular surgery, nursing, psychology, dietetics, and kinesiology. RESULTS: Alongside findings that highlighted the significant demographic shift, the findings highlight key themes related to temporal shifts in treatment, emergence of organizational structures and use of evidence, comprehensive approaches to care, and future directions. A critical finding that requires immediate attention is the significantly disproportionate resources to the number of adults living with CHD, and as a result, the real risk of premature death for this population. CONCLUSIONS: The insights provided behoove the community of stakeholders to think creatively on how to draw attention to the inadequacy of resources and the unique and diverse needs of this population.

Cost analysis of combining congenital cardiac catheterization and electrophysiology procedures in an outpatient setting.

BACKGROUND: Patients with congenital heart disease require multiple procedures over their lifetime. These procedures increase cost and time commitment. Previous studies in the field of medicine have shown that combining procedures is an effective method to reduce cost and time. There has been no such study to evaluate the cost and efficiency of combining pediatric cardiac procedures. OBJECTIVE: The objective of this study was to compare the cost and time commitment of combined cardiac catheterization (cath) and electrophysiology (EP) outpatient procedures against separate cath and EP procedure. METHODS: Outpatient combination procedures performed in the pediatric cardiac cath lab from 2013 to 2016 were matched to a control population of two or three similar single outpatient procedures from 2009 to 2016 for patients of similar age and cardiac anatomy. Procedure duration, recovery duration, length of stay, equipment charges, physician charges, all other hospital charges, and total admission charges were analyzed. The two groups were compared using an unpaired t-test. RESULTS: A total of 92 subjects, 32 study subjects and 60 control subjects, were included in this study. Study group procedures had a significantly shorter recovery duration (P = 0.04) and length of stay (P = 0.01). Study group procedure duration trended shorter on average but statistically insignificant (P = 0.20). The total median savings for patients undergoing combined procedures in the study group was $13,181 (interquartile range $423.8-$26710). CONCLUSIONS: Combining cath and EP outpatient procedures reduces the time commitment and provides some economic advantage.

Feasibility and Outcomes in a Pilot Randomized Controlled Trial of a Psychosocial Intervention for Adults With Congenital Heart Disease.

BACKGROUND: North American adults with congenital heart disease (CHD) are known to be at elevated risk of mood and anxiety disorders. This was the first trial of a group psychosocial intervention targeting this patient population. METHODS: Within this feasibility study, we conducted a 2-arm pilot randomized controlled trial (RCT) in which patients were randomized to Usual Care or an 8-session group psychosocial intervention (Adult Congenital Heart Disease-Coping and Resilience [ACHD-CARE]). Here, we report feasibility outcomes in accordance with published recommendations: (1) process, (2) resources, (3) management, (4) acceptability of the intervention, and (5) scientific outcomes (for which the primary outcome measures were anxiety and depression symptoms). RESULTS: Forty-two patients were randomized in the pilot RCT. The study was executable within a realistic timeline and revealed no significant human and data-management problems. The intervention was determined to be acceptable and highly valued by participants who participated in the ACHD-CARE program. The main challenges were practical barriers (eg, transportation, scheduling group sessions in-person given competing schedules) and retention. With regard to scientific outcomes, there were no adverse outcomes, and treatment fidelity was confirmed. Although not powered to test efficacy, there was a medium effect size (in favour of the intervention group) for depression symptoms. CONCLUSIONS: We determined it would be feasible to conduct a full-scale trial of a psychosocial intervention targeting adults with CHD, although with modifications to address practical barriers to participation. Should this intervention prove effective, a manualized intervention could be made be available.