The Palliative Approach and Terminal Heart Failure Admissions - Are We Getting it Right?

BACKGROUND: Chronic heart failure has a high mortality and early provision of palliative care supports complex decision-making and improves quality of life. AIM: To explore whether and when a palliative approach was adopted during the last 12 months of life in patients who experienced an in-hospital death from heart failure. DESIGN: Retrospective medical record review of all deaths from chronic heart failure (January 2010 to December 2019). PARTICIPANTS: Admissions with chronic heart failure resulting in death were analysed from an Australian tertiary referral centre. RESULTS: The cohort (n=517) were elderly (median age 83.8 years IQR=77.6-88.7) and male (55.1%). Common comorbidities were ischaemic heart disease (n=293 56.7%) and atrial fibrillation (n=289 55.9%). Life sustaining interventions occurred in 97 (18.8%) patients. In 31 (6.0%) patients referral to specialist palliative care occurred prior to, and in 263 (50.9%) during, the terminal admission. Opioids were prescribed to 440 (85.1%) patients. Comfort care was the documented goal in 158 patients (30.6%). A palliative approach was significantly associated with prior admission in the preceding 12 months (OR=1.5 95% CI=1.0-2.1 p<0.043), receiving outpatient care (OR=2.6 95% CI=1.6-4.1 p<0.01), and admissions in the latter half of the decade (OR=1.5 95% CI=1.0-2.0 p<0.038). CONCLUSION: Despite greater adoption of a palliative approach in the terminal admission over the last decade, a significant proportion of patients receive palliative care late, just prior to death.

Fatal Enteroviral Encephalitis in a Patient with Common Variable Immunodeficiency Harbouring a Novel Mutation in NFKB2.

Common variable immunodeficiency is the most prevalent of the primary immunodeficiency diseases, yet its pathogenesis is largely poorly understood. Of the cases that are monogenic, many arise due to pathogenic variants in NFKB1 and NFKB2. Here, we report enteroviral encephalomyelitis as the cause of a fatal neurodegenerative condition in a patient with a novel heterozygous mutation in NFKB2 (c.2543insG, p.P850Sfs36*) that disrupts non-canonical NF-κB signaling. Investigations of primary and secondary lymphoid tissue demonstrated a complete absence of B cells and germinal centers. Despite multiple negative viral PCR testing of cerebrospinal fluid during her disease progression, post-mortem analysis of cerebral tissue revealed a chronic lymphocytic meningoencephalitis, in the presence of Cocksackie A16 virus, as the cause of death. The clinical features, and progression of disease reported here, demonstrate divergent clinical and immunological phenotypes of individuals within a single family. This is the first reported case of fatal enteroviral encephalomyelitis in a patient with NF-κB2 deficiency and mandates a low threshold for early brain biopsy and the administration of increased immunoglobulin replacement in any patient with a defect in this pathway and deterioration of neurological status.