The incidence of congenital heart disease is 0.8% of live births [1Dolk H., Loane M., Garne E., European Surveillance of Congenital Anomalies (EUROCAT) Working Group Congenital heart defects in Europe: prevalence and perinatal mortality, 2000 to 2005 Circulation 2011 ;  123 (8) : 841-849 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti]. According to the data in the literature, our neighboring countries have already examined mortality according to the type of heart disease, the perioperative context or even the age at death [2Gilboa S.M., Salemi J.L., Nembhard W.N., Fixler D.E., Correa A. Mortality resulting from congenital heart disease among children and adults in the United States, 1999 to 2006 Circulation 2010 ;  122 (22) : 2254-2263 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 3GBD 2017 Congenital Heart Disease Collaborators Global, regional, and national burden of congenital heart disease, 1990–2017: a systematic analysis for the Global Burden of Disease Study 2017 Lancet Child Adolesc Health 2020 ;  4 (3) : 185-200

Cliccare qui per andare alla sezione Riferimenti, 4Jortveit J., Øyen N., Leirgul E., Fomina T., Tell G.S., Vollset S.E., e al. Trends in mortality of congenital heart defects Congenit Heart Dis 2016 ;  11 (2) : 160-168 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 5Kaltman J.R., Burns K.M., Pearson G.D., Goff D.C., Evans F. Disparities in congenital heart disease mortality based on proximity to a specialized pediatric cardiac center Circulation 2020 ;  141 (12) : 1034-1036 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 6Mat Bah M.N., Sapian M.H., Jamil M.T., Alias A., Zahari N. Survival and associated risk factors for mortality among infants with critical congenital heart disease in a developing country Pediatr Cardiol 2018 ;  39 (7) : 1389-1396 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 7Morris C.D., Menashe V.D. 25-year mortality after surgical repair of congenital heart defect in childhood. A population-based cohort study JAMA 1991 ;  266 (24) : 3447-3452 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti]. However, the distribution of causes of death in children with heart disease has not been described.

Main objective is to conduct an epidemiological analysis of the distribution of causes of death in children with heart disease in an important center.

It is a retrospective descriptive study, single-center: pediatric cardiology at Necker hospital. Method is collect data from the medical file. Inclusion criterions are: age 0 to 18 at death, deceased between 2010 and 2020 and heart disease (congenital or not). This represents 973 patients. Classification of the causes of mortality established on data from the literature and on professional experience in: accompaniment, sudden death, heart failure, pulmonary arterial hypertension, rhythmic or conductive disorder, multi-organ failure, chronic respiratory failure, brain death, septic shock, hemorrhagic shock, thrombotic accident. It was also collected: term of birth, sex, place of death (home or hospital), type of heart disease, date of surgery/catheterization, number of surgeries, comorbidities, assistance (ECMO or Berlin Heart) at the time of death.

We observed as expected that the mortality is mainly before the age of one year, during the perioperative period. However, a peak was not observed after the age of 5 in the cohort studied (Fig. 1).

Excluding accompaniment, the main cause of death from all heart disease is heart failure and secondarily pulmonary arterial hypertension (Fig. 2).

In the light of what is known, it appears interesting to bring the experience of a reference center in the analysis of mortality in children with heart disease to enrich our knowledge and discuss unexpected results.