Complex chronic conditions (CCC) represent an increasing burden in pediatrics, reflecting both clinical progress and limits of curative care. Those with congenital or acquired heart disease (CAHD) account for the largest subgroup of CCC inpatient deaths. This study aimed to characterize healthcare utilization over the last year of life management and circumstances of death among children with CAHD.

We conducted an observational study at Necker Children's Hospital (Paris, France). Patients aged 1–18 years with CAHD meeting criteria for CCC, with at least one year of follow-up after diagnosis, who died between 2013 and 2023 were included. Data were extracted from a prospectively maintained mortality database and electronic medical records. Collected variables included diagnostic categories, genetic and extracardiac comorbidities, healthcare utilization during the last year of life, end-of-life care, and circumstances of death.

A total of 190 patients were included. Congenital heart disease (CHD) accounted for 57.8% of diagnoses, followed by cardiomyopathies (18.8%), pulmonary arterial hypertension–related disease (10.9%), and rhythm disorders (12.5%). A prenatal diagnosis was documented in 45.3% of patients, predominantly in CHD (70.3%). Genetic syndromes or extracardiac anomaly were reported in 68.8%. During the last year of life, 86% were hospitalized (median cumulative stay 13 days), mainly for acute symptoms (45%) or scheduled procedures (48%). Catheterization (34%) and cardiac surgery (41%) were performed at a median of 38 days before death, with 52% intended to be curative. Care intensity increased markedly in the final two weeks, and 70% of deaths occurred in the ICU following active resuscitation (40%), withdrawal (13%), or non-escalation (12%) of life-sustaining treatment. At death, 41% were intubated, 11% received non-invasive ventilation, 33% inotropes, and 14% were supported with ECMO. Overall, 21% were referred to pediatric palliative care at a median of 24 days before death.

Children with CCC and CAHD follow highly complex care trajectories, in which curative and palliative needs often overlap, making anticipatory planning challenging. Earlier integration of pediatric palliative care beyond the terminal phase may support shared decision-making and improve the quality of care throughout advanced stages of disease.