Turning 18 With Congenital Heart Disease: Prediction of Infective Endocarditis Based on a Large Population

Study Questions:

What is the future risk of development of infective endocarditis for patients with congenital heart disease entering adulthood?


The Dutch CONCOR national registry was used to identify cases of endocarditis in adults with congenital heart disease. A prediction model was derived using the Cox proportional hazards model and bootstrapping techniques. The model was then used to create a risk score to predict a patient’s risk of endocarditis from age 18 up to age 40, as well as to age 60.


Of 10,210 patients in the registry, 233 (2.3%) developed endocarditis over 220,688 patient-years. Predictors of endocarditis included gender, underlying cardiac diagnosis, previous history of endocarditis, previous neurologic event, and history of supraventricular tachycardia or conduction disturbances (decreased risk for endocarditis). When the risk score was applied, up to the age of 40, patients with a low predicted risk (<3%) had an observed incidence of <1%, and those with a high predicted risk (≥3%) had an observed incidence of 6%.


The authors concluded that a risk score consisting of simple clinical parameters can accurately predict patients at relatively low or high risk of infective endocarditis.


This interesting study develops a risk score to determine the risk of young adults with congenital heart disease developing endocarditis up to age 40 and then to age 60. Each congenital heart lesion is given a particular risk weighting, with the lowest for patent ductus arteriosus and atrial septal defect, and the highest for pulmonary atresia with ventricular septal defect. For example, an 18-year-old male with pulmonary atresia and ventricular septal defect and previous endocarditis would have a 56% chance of developing endocarditis by age 60. In contrast, an 18-year-old female with pulmonary stenosis would have a <1% chance of developing endocarditis by age 60. There are some limitations to the study. In constructing the model, all ventricular septal defects were grouped together. One would expect that the endocarditis risk for a patient with completely repaired ventricular septal defect would be lower than a patient with unrepaired defect or repaired defect with residual shunt. Additionally, it is interesting that a history of previous supraventricular arrhythmia or conduction disturbance decreases one’s risk of endocarditis. This model will require validation in additional patient cohorts prior to its widespread clinical application.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Valvular Heart Disease, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias

Keywords: Tachycardia, Supraventricular, Endocarditis, Heart Defects, Congenital, Pulmonary Atresia, Pulmonary Valve Stenosis, Ductus Arteriosus, Patent

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