The importance of early involvement of paediatric palliative care for patients with severe congenital heart disease

Abstract

Growing numbers of patients with severe congenital heart disease (CHD) are surviving into late childhood and beyond. This increasingly complex patient group may experience multiple formidable and precarious interventions, lifelong morbidity and the very real risk of premature death on many occasions throughout their childhood. In this paper, we discuss the advantages of a fully integrated palliative care ethos in patients with CHD, offering the potential for improved symptom control, more informed decision-making and enhanced support for patients and their families throughout their disease trajectory. These core principles may be delivered alongside expert cardiac care via non-specialists within pre-existing networks or via specialists in paediatric palliative care when appropriate. By broaching these complex issues early-even from the point of diagnosis-an individualised set of values can be established around not just end-of-life but also quality-of-life decisions, with clear benefits for patients and their families regardless of outcome.

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

“…Emerging evidence suggests there is some negative life experience and a variable impact on quality of life for those living with a Fontan circulation [392,393], but a positive outlook and focus on remaining healthy are common [392]. Although there is evidence from randomised trials that shows improved quality of life, less depression, and greater spiritual wellbeing with palliative care for those with advanced non-congenital HF [394], there are few data to directly guide palliative care for those with a Fontan circulation [395]. Although not an ideal predictor of death, a suggested pragmatic approach to involving palliative care is to ask clinicians to reflect on the question, ''Would I be surprised if this patient died in the next 12 months?".…”

Cuidado paliativo pediátrico

“…El CPP está indicado en un amplio margen de patologías, aun cuando la cura y la supervivencia a largo plazo, incluso hasta la vida adulta, son posibilidades viables [2,10]. En los niños, las enfermedades malignas (y algunas benignas), tales como fibrosis quística, VIH, mucopolisacaridosis, enfermedades neurodegenerativas, entre otras, tienen un curso crónico, y se han convertido en un problema de salud pública debido a la alta morbimortalidad que representan.…”

“…Como se puede observar, hoy en día, el rango de pacientes que potencialmente se pueden beneficiar del CPP es muy amplio y heterogéneo, justificando su empleo en forma adecuada desde los momentos iniciales de la enfermedad cuando se justifique, y no solo cuando la muerte del niño es el único desenlace posible [10]. En la Tabla 1 se pueden observar ejemplos de enfermedades en las cuales los niños se podrían beneficiar del CPP [13].…”

Improving outpatient advance care planning for adults with congenital or pediatric heart disease followed in a pediatric heart failure and transplant clinic

“…In pediatrics, parents and the healthcare team typically make decisions for patients, but as these patients become adolescents and young adults, they should voice their own values, opinions, and healthcare goals. In no area is this more important than in end‐of‐life care . Without knowing the patient's healthcare preferences in the case of advancing disease or imminent death, the family and healthcare team are forced to make decisions for the patient in an emotional and stressful time and in a culture that promotes life‐sustaining measures.…”

“…In no area is this more important than in end-of-life care. 3,4 Without knowing the patient's healthcare preferences in the case of advancing disease or imminent death, the family and healthcare team are forced to make decisions for the patient in an emotional and stressful time and in a culture that promotes lifesustaining measures.…”