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Purpose of review Therapies for paediatric congenital and acquired heart disease continue to evolve and the appropriateness of pursuing life sustaining interventions at margins of standard therapy is ethically challenging. Recent findings With ongoing emphasis on shared decision making, recent literature explored physician and parental perspectives on communication with families and offering interventions for complex congenital heart disease and advanced heart failure. The inclusion of parental values and views in this process is now widely accepted. Identified outstanding challenges include difficulty with prognostication from the outset, adjusting long-term goals of care to changes in clinical parameters, need for consistency in communication including regular review meetings with family or surrogate decision-makers. Bioethics consultation and multidisciplinary team reviews may be helpful supports. Palliative care involvement in this population improves quality of life and alleviates parental distress but this collaboration is not optimized. Summary Decision to offer, forgo, or discontinue life-sustaining therapies for children with heart disease has nuanced and context-specific considerations, and must integrate burdens of interventions with patient and family values. Thus, decision making remains complex and demands thoughtful review of not only risks and benefits, but views and values, clearly communicated to team and family.

In the course of providing extracorporeal membrane oxygenation (ECMO) and considering more than one ECMO intervention in a single patient, multiple ethical dilemmas will be faced. Solid grounding in ethical considerations and application will help the care provider equip themselves to work through the nuance and context of the unique cases they face. Advanced reasoning regarding medical options should be embedded with a rigorous ethical framework to guide decisions to offer or withhold ECMO. The chapter provides a complex clinical case with discussion of the multiple ethical considerations and application of moral theory and biomedical ethics frameworks to provide the reader an example of how therapies such as ECMO can be ethically justified and appropriately attended to.

Cardiorespiratory symptoms are important in paediatric palliative care, affecting those with cancer as well as children with non-oncological life-limiting illnesses. Breathing is not only a vital function; it also often acts as a barometer of a person’s overall comfort. Breathlessness, often the end point of both cardiac and respiratory compromise, is invariably listed among the most distressing of symptoms. A multimodal approach to treating discomfort related to cardiorespiratory compromise, is essential, allowing for the physical, psychological, social, and spiritual dimensions of the symptoms. The range of possible treatments available continues to expand, encompassing everything from simple non-pharmacological measures to long-term mechanical support of respiratory or cardiac function. This chapter will outline a holistic approach to managing cardiorespiratory symptoms in the child, providing excellent palliative care, where improving quality of life is a primary focus.

Clinicians such as Sir William Osler reinvented Hippocrates and built the image of a noble, lone, professional man (nobility had rather limited horizons in its day) replete with black bag, minister...

Using the narrative about a child with severe respiratory infection and septic shock, this chapter illustrates important learning points around both veno-arterial and veno-venous extracorporeal life support (ECLS) in children. Several important considerations such as patient selection, anticoagulation, management of bleeding complications, and respiratory rehabilitation on ECLS are discussed. The chapter includes a brief discussion of other forms of mechanical circulatory support (e.g. ventricular assist device) and early mobilization while on ECLS. The chapter highlights difficulties with prognostication, ethical decision-making dilemmas in patients on longer-term ECLS support, and provides a useful framework for decision-making.

ECMO has proven to be a life-saving intervention for a variety of disease entities with a high rate of survival in the neonatal population. However, ECMO requires clinical teams to engage in many ethical considerations. Even with ongoing improvements in technology and expertise, some patients will not survive a course of ECMO. An unsuccessful course of ECMO can be difficult to accept and cause a great deal of angst. These questions can result in real conflict both within the care team, and between the care team and the family. Herein we explore a range of ethical considerations that may be encountered when caring for a patient on ECMO, with a particular focus on those courses where it appears likely that the patient will not survive. We then consider how a palliative care approach may provide a tool set to help engage the team and family in confronting the difficult decision to discontinue ECMO.

An infant with pulmonary atresia/ventricular septal defect/major aortopulmonary collateral arteries underwent unifocalization, ventriculoseptal defect closure and placement of a right ventricle to pulmonary artery conduit via median sternotomy. Aortopexy and pulmonary arteriopexy via redo sternotomy were insufficient to allow weaning of continuous positive airway pressure and he required direct tracheobronchopexy via left lateral thoracotomy to alleviate posterior trachealis intrusion along the length of the trachea and left main bronchus. He also underwent laryngotracheoplasty with placement of a posterior costal cartilage graft for bilateral vocal cord paralysis. The patient was weaned from positive pressure and discharged in stable condition.

We reflect upon highlights of a facilitated panel discussion from the 2016 Pediatric Cardiac Intensive Care Society Meeting. The session was designed to explore challenges, share practical clinical experiences, and review ethical underpinnings surrounding decisions to offer intensive, invasive therapies to patients who have a poor prognosis for survival or are likely to be burdened with multiple residual comorbidities if survival is achieved. The discussion panel was representative of a variety of disciplines including pediatric cardiology, cardiac intensive care, nursing, and cardiovascular surgery as well as different health-care delivery systems. Key issues discussed included patient’s best interests, physician obligations, moral distress, and communication in the context of decisions about providing therapy for patients with a poor prognosis.

A chimney femoral artery graft for peripheral extracorporeal membrane oxygenation can potentially cause hyperperfusion and subsequent venous congestion in the ipsilateral leg, especially in the context of septic shock and higher flow requirement. This report describes a novel technique to use an additional leg venous cannula to avoid leg congestion as well as to achieve higher total flow.

Patients with critical congenital heart disease are exposed to significant lifetime morbidity and mortality. Prenatal diagnosis can provide opportunities for anticipatory co-management of patients between palliative subspecialists and the cardiac care team. The benefits of palliative care include support for longitudinal decision-making and avoidance of interventions not consistent with family goals. Effectively counseling families requires an up-to-date understanding of outcomes and knowledge of provider biases. Patient-proxy reported quality of life (QOL) is highly variable in this population and healthcare providers need to be aware of limitations in their own subjective assessment of QOL.

Extracorporeal cardiopulmonary resuscitation may be defined as the use of extracorporeal membrane oxygenation for the support of patients who do not respond to conventional cardiopulmonary resuscitation. Data from national and international paediatric databases indicate that the use of extracorporeal cardiopulmonary resuscitation is increasing. Guidelines from the American Heart Association suggest that any patient with refractory cardiopulmonary resuscitation and potentially reversible causes of cardiac arrest is a candidate for extracorporeal cardiopulmonary resuscitation. One possible framework for selection of patients for extracorporeal cardiopulmonary resuscitation includes dividing patients on the basis of favourable or unfavourable characteristics. Favourable characteristics include cardiac disease, witnessed event in the intensive care unit, ability to deliver effective cardiopulmonary resuscitation, active patient monitoring present, favourable arterial blood gases, and early institution of extracorporeal membrane oxygenation. Unfavourable characteristics potentially include non-cardiac disease, an unwitnessed cardiac arrest, ineffective cardiopulmonary resuscitation, and severely acidotic arterial blood gases. Considering the significant resources and cost involved in the use of extracorporeal cardiopulmonary resuscitation, its use needs to be critically examined to improve outcomes, assess neurological recovery and quality of life, and help identify populations and other factors that may help guide in the selection of patients for successful extracorporeal cardiopulmonary resuscitation.

IntroductionThe effective exchange of clinical information is essential to high-quality patient care, especially in the critical care unit (CCU) where communication failures can have profoundly negative impacts on critically ill patients with limited physiological capacity to tolerate errors. A comprehensive systematic characterisation of information exchange within a CCU is needed to inform the development and implementation of effective, contextually appropriate interventions. The objective of this study is to characterise when, where and how healthcare providers exchange clinical information in the Department of Critical Care Medicine at The Hospital for Sick Children and explore the factors that currently facilitate or counter established best rounding practices therein.Methods and analysisA convergent parallel mixed-methods study design will be used to collect, analyse and interpret quantitative and qualitative data. Naturalistic observations of rounds and relevant peripheral information exchange activities will be conducted to collect time-stamped event data on workflow and communication patterns (time–motion data) and field notes. To complement observational data, the subjective perspectives of healthcare providers and patient families will be gathered through surveys and interviews. Departmental metrics will be collected to further contextualise the environment. Time–motion data will be analysed quantitatively; patterns in field note, survey and interview results will be examined based on themes identified deductively from literature and/or inductively based on the data collected (thematic analysis). The proactive triangulation of these systemic, procedural and contextual data will inform the design and implementation of efficacious interventions in future work.Ethics and disseminationInstitutional research ethics approval has been acquired (REB #1000059173). Results will be published in peer-reviewed journals and presented at relevant conferences. Findings will be presented to stakeholders including interdisciplinary staff, departmental management and leadership and families to highlight the strengths and weaknesses of the exchange of clinical information in its current state and develop user-centred recommendations for improvement.

The purpose of this review is to discuss the management of the low cardiac output syndrome (LCOS) following surgery for congenital heart disease. The LCOS is a well-recognized, frequent post-operative complication with an accepted collection of hemodynamic and physiologic aberrations. Approximately 25% of children experience a decrease in cardiac index of less than 2 L/min/m2 within 6-18 hours after cardiac surgery. Post-operative strategies that may be used to manage patients as risk for or in a state of low cardiac output include the use of hemodynamic monitoring, enabling a timely and accurate assessment of cardiovascular function and tissue oxygenation; optimization of ventricular loading conditions; the judicious use of inotropic agents; an appreciation of and the utilization of positive pressure ventilation for circulatory support; and, in some circumstances, mechanical circulatory support. All interventions and strategies should culminate in improving the relationship between oxygen supply and demand, ensuring adequate tissue oxygenation.

Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder diagnosed in childhood. It affects ≈1 in every 5000 live male births (≈20 000 new cases worldwide each year).1,2 This results in a US prevalence of 1.3 to 1.8 per 10 000 males 5 to 24 years of age. DMD is caused by mutations in the gene encoding the dystrophin protein. The loss of dystrophin results in a cascade of events leading to progressive loss of muscle function. Without supportive care, young men with DMD typically die in their late teens and early 20s. Historically, the most common cause of death has been respiratory failure. However, with improved respiratory support, an increasingly important source of morbidity and mortality is cardiomyopathy leading to heart failure and arrhythmias.3,4 There are important differences in DMD cardiomyopathy compared with other types of pediatric dilated cardiomyopathy.5 DMD cardiomyopathy is similar to the cardiomyopathy seen in some forms of limb girdle muscular dystrophy and congenital muscular dystrophy. In particular, a shared cardiomyopathic process is seen in those disorders in which the primary mutation alters components that directly or indirectly interact with dystrophin. There is less left ventricular (LV) enlargement at diagnosis in DMD. Only 30% of boys with DMD have cardiac symptoms at diagnosis (far fewer than other dilated cardiomyopathy). DMD cardiomyopathy is less often treated at the time of diagnosis. However, treatment rates have increased over time. Finally, there is a higher mortality for DMD cardiomyopathy than for other dilated cardiomyopathies. The DMD Care Considerations published in 2010 addressed cardiac care recommendations based on minimal surveillance standards with echocardiography.6,7 However, echocardiography has known limitations in DMD patients.8 Since the 2010 publication of the DMD Care Considerations,6,7 there have been significant advances in the understanding …

The use of extracorporeal membrane oxygenation in infants and children with cardiac disease who develop refractory cardiogenic shock, cyanosis, or cardiac arrest is increasing. Early mortality in children with cardiac disease who require extracorporeal membrane oxygenation remains an important issue, as only 40% of cannulated patients survive to discharge from the hospital. However, it is encouraging that 90% children who are discharged alive from the hospital after extracorporeal membrane oxygenation are still alive at intermediate-term follow-up. Surviving patients are at risk for long-term dysfunction of multiple organ systems related to their underlying cardiac disease, non-cardiac comorbidities, treatment-related complications, and exposure to extracorporeal membrane oxygenation. Among the most important acute complications related to support with extracorporeal membrane oxygenation is injury to the central nervous system, which may contribute to adverse neurodevelopmental outcomes. All of these factors, in turn, influence quality of life. Many survivors remain medically complex related to their underlying cardiac disease, comorbidities, and sequelae of complications acquired over their lifetime. Neurological morbidity clearly plays an important role in approximately one-third of survivors, with significant deficits in approximately 10%. The limited data about quality of life data that are available for survivors of cardiac extracorporeal membrane oxygenation suggests that approximately 15–30% of survivors have at least moderately decreased quality of life. Overall, published data support the ongoing use of support with extracorporeal membrane oxygenation in children with acute cardiac failure, most of whom would die without it. However, programmatic efforts to improve the selection of patients and the preservation of the function of end organs during extracorporeal membrane oxygenation are clearly needed in order to improve long-term outcomes.

The objective of this study was to determine if cognitively normal children with epilepsy have higher accidental injury rates than their age- and sex-matched friends without epilepsy and what factors may predict this. Patients 5 to 16 years old, with a developmental quotient >70, without major motor or sensory impairments, with a 1-year history of epilepsy and who either had a seizure or had been on antiepileptic drugs within the past year, were identified from the pediatric neurology database of the Royal University Hospital. Twenty-five of 31 cases and their best friend controls agreed to participate. Seizure-related factors including type, duration, frequency, timing, date of diagnosis, antiepileptic drug initiation and discontinuation, and specific types and total antiepileptic drugs used were assessed by interview. Questionnaires about accidental injury including type, number, severity, and, if applicable, injuries resulting from seizures, as well as general safety practices, activity restrictions, and presence of attention-deficit hyperactivity disorder, were completed by cases and controls. No significant differences in injury numbers (specific types or total) or severity were found, although a small number of epileptic children were very predisposed to injury. Seizure-related factors did not predict injury in cases. Safety practices were similar, and restrictions in cases were not excessive. Children with attention-deficit hyperactivity disorder had a higher injury rate, both in cases and controls. Cognitively normal children with epilepsy do not have a higher injury rate than their nonepileptic peers. If consciousness is impaired in seizures, extra supervision for swimming and bathing and restricted climbing heights are suggested. All other safety restrictions for epileptic children should follow those appropriate to nonepileptic children to allow a normal lifestyle. (J Child Neurol 2001;16:100-104).