Death in Early Childhood Due to Severe Congenital Heart Disease

Abstract

Source: Wik G, Jortveit J, Sitras V, et al. Unexpected death in children with severe congenital heart defects in Norway 2004-2016. Arch Dis Child. 2021;106(10):961-966; doi:10.1136/archdischild-2020-319936Investigators from Oslo University Hospital, Oslo, Norway, and Sorlandet Hospital Arendal, Arendal, Norway, conducted a retrospective study to determine rates of unexpected deaths in children with severe congenital heart defect (CHD), with a focus of deaths that occurred unrelated to cardiac interventions. Study participants were children with severe CHD born between 2004 and 2016 and identified by reviewing the Oslo University Hospital Clinical Registry for Congenital Heart Defects. Data from this registry were linked to the Norwegian Cause of Death Registry to obtain information about date and causes of death. Cardiac lesions classified as severe included cyanotic CHDs, coarctation of the aorta, atrioventricular septal defects (AVSD), aortic stenosis, Epstein’s anomaly, and coronary artery anomalies. Ventricular septal defects were classified as severe if treated by intervention.The medical records of the identified children were reviewed with information on dates of cardiac procedures and clinical status abstracted. The main study outcome was unexpected death before the age of 2 years, defined as a death in a study patient who was not receiving palliative care because of a surgically uncorrectable cardiac lesion. Unexpected deaths were classified as pre- or postoperative in-hospital mortality, or unrelated to cardiac interventions, occurring before a planned intervention or following discharge for an intervention. Potential risk factors associated with unexpected deaths that were assessed included staged surgeries for a lesion, residual defect after surgery, persistent low oxygen saturation (<95%), comorbidity, and infection at the time of death.Data were analyzed on 2,359 children with severe CHD, of whom 2,123 (90%) were still alive at 2 years of age. Among the 236 children who died before 2 years of age, 2 were excluded from further analysis because of parental request, 109 (46%) died after palliative care, 58 (46%) were classified as pre-/postoperative in- hospital deaths, and 67 (54%) had an unexpected death unrelated to cardiac intervention. The group of 67 children with unexpected deaths unrelated to cardiac intervention (2.8% of all patients with severe CHD) included 26 (39%) who died prior to a planned intervention. Causes of death in these children were presumed to be cardiac in 11 (42%), related to comorbidity in 3 (12%), associated with infection in 7 (27%), and due to comorbidity and cardiac causes in 5 (19%). Among the 41 children who died after discharge following cardiac intervention, risk factors associated with the death included comorbidity in 22 (54%), persistent low oxygen saturation in 23 (56%), staged surgery in 21 (51%), and residual defect in 21 (51%).The authors conclude that the rate of unexpected death unrelated to cardiac intervention in children <2 years old with severe CHD without comorbidity is low. The numbers of unexpected deaths unrelated to cardiac intervention in children <2 years of age were low.Dr Bratton has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.Congenital heart disease is the most common congenital defect, occurring in 1% of live births, and almost half of deaths among infants with CHD occur within the first year of life.1 The authors of the present study evaluated a small group of Norwegian infants with CHDs. Among 67 infants, deaths were unexpected and either occurred before a planned cardiac procedure or after hospital discharge from a cardiac procedure and were classified as unexpected death unrelated to a cardiac procedure.However, conclusions regarding rates of death among infants with specific CHDs cannot be compared as the current authors do not present data on the infants at risk of death who survived. Therefore, neither risk of death with specific heart defects nor population-based mortality of children ≤2 years with and without severe CHDs can be calculated. Surgical mortality is typically defined as death within 30 days after surgery regardless of cause or hospital status.3 Marked increases in surgical survival for CHD over the past 20 years are largely due to increased survival among infants with hypoplastic left heart syndrome, but these infants require staged surgical procedures to palliate the single ventricle physiology.4Children with severe CHD infrequently die unexpectedly within the first 2 years of life. Those with hemodynamically important residual cardiac defects, co-morbid conditions, infections, and desaturation likely have greater rates of death compared to those who do not.