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Objective To describe causes of death in children with congenital or acquired heart disease (CAHD) and provide updated mortality data across CAHD groups.Design Prospective observational cohort study (2010–2024).Setting Tertiary paediatric cardiac care centre.Patients All children with CAHD who died during the study period were classified into congenital heart diseases (CHD), cardiomyopathies (CM) and pulmonary hypertension (PH).Main outcome measures Causes of death and demographic characteristics were reported for the entire cohort and by the CAHD group.Results Among 1137 deaths, 244 (21.5%) occurred under compassionate care. The main cohort consisted of 868 (76.3%) children with CHD, 170 (14.9%) with CM and 50 (4.4%) with PH. The median age at death was 2.6 (IQR 14.2) months. Comorbidities and prematurity were observed in 438 (38.6%) and 348 (30.6%) cases, respectively, with no difference among groups. After excluding compassionate care, heart failure was the leading cause of death affecting 359 (40.2%) children, followed by PH crisis in 105 (11.8%) and infections in 101 (11.4%). In the CHD group, severe complexity was predominant in 516 (59.5%) cases, with functionally univentricular heart (172 (33.3%) cases) being the most common subtype. Surgical deaths constituted 625 (72%) cases, with 64 (7.4%) requiring extracorporeal membrane oxygenation (ECMO) and 69 (0.8%) listed for heart transplant. In the CM group, the dilated CM subtype was predominant, accounting for 78 (45.9%) cases, with 12 (7.1%) requiring ECMO. For the PH group, pulmonary arterial hypertension was observed in 34 (68%) cases, with 9 (18%) requiring ECMO.Conclusions Heart failure is the leading cause of death in CAHD, followed by PH, with most deaths occurring within the first year of life, particularly in complex CHD and premature infants.