Investigators at Children's Hospital Boston reviewed the medical records of children <21 years old who had advanced heart disease (AHD) and died between 2007 and 2009, to determine lifetime burden of disease, mode of death, and end-of-life experience. Lifetime burden of disease was determined by the number of billed procedures that took place in an operating room or at the bedside over the patient's life. Mode of death was defined as either withdrawal of disease-directed care, death during resuscitation, or comfort care after birth, based on medical record documentation. End-of-life experience was described using the location of death, length of final hospital stay, and presence of family at bedside at the time of death. Medical records were reviewed for the patient's age at death, diagnosis, end-of-life discussions with the family, and the presence of a palliative care consult. The patient's mental status and types of interventions that occurred in the 24 hours preceding death were also recorded.
A total of 111 children were included in the study. Mean age at death was 4.8 months; 76% of study children died in the first year of life. The majority of patients (76%) had a diagnosis of congenital heart disease (CHD), 41% of whom had single-ventricle physiology. Of those without CHD, 10 had cardiomyopathy, 9 had pulmonary hypertension, and 8 had undergone heart transplantation. The median lifetime number of operating room and bedside procedures was 1 for all patients.
The median length of the final hospital stay was 22 days. Discontinuation of disease-directed interventions was the most common mode of death (69%). Death during resuscitation occurred in 25%, and 6% died while receiving comfort care after birth. At the time of death, most patients (87%) were in the cardiac ICU, were completely sedated (90%), and were receiving analgesic medication (92%). Within 24 hours of death, almost all were intubated and almost half were receiving mechanical circulatory support (ECMO or ventricular assist devices). Three quarters of patients had evidence of multisystem organ failure. The patient's parents were present at the bedside at the time of death in 83% of cases. Three quarters of families participated in an end-of-life discussion and 16% of children received a palliative care consult.
The authors conclude that children who die of AHD frequently are cared for in the ICU, have multisystem organ failure, and have received highly technical end-of-life care.
End-of-life care in children with AHD has received little attention. The authors of the current study suggest that this is due to the misconception that children with AHD die “suddenly.” Their study, while limited to 1 institution, demonstrates that this is not the case; many children with AHD have prolonged hospitalizations and numerous intensive medical interventions prior to death.
Previous studies have documented the...