Infective endocarditis in congenital heart disease: Japanese national collaboration study

Objective: To provide pure cohorts of paediatric and adult patients with congenital heart disease (CHD) and infective endocarditis (IE) for making future guidelines. Design: Japanese nationwide survey. Setting: 66 Japanese institutions. Patients: 170 children, mean (SD) age 7.4 (5.7) years ( range 14 days to 17 years), and 69 adults, age 32.5 (14.1) years ( range 18 - 69) who developed IE between 1997 and 2001 ( one in 240 admissions with CHD). Main outcome measures: Clinical presentation of IE. Results: 119 patients including 88 with cyanotic CHD had previous cardiac surgery. Procedures preceding IE were dental (12%) followed by cardiovascular surgery (8%). Sites of infection were left sided in 46% and right sided in 51%. Vegetation with diameter of 11 mm was documented in 151 ( 63%). Frequent complications were embolic events ( stroke 11%, other emboli 20%) and cardiac failure (23%). The most common microorganisms were streptococci (50%) and staphylococci (37%) with methicillin resistant Staphylococcus aureus in 7.5%. Empirical treatments were penicillins ( alone or with other antibiotics 57%) followed by cephems (22%) and vancomycin ( 11%). Surgery during active IE was common (26%), with vegetation (45%) and heart failure (29%) as the most frequent indications. Mortality was 8.8%: 8.0% among patients who received medical treatment alone and 11.1% among those with active IE who underwent surgery. The causes of death ( n = 21) were surgery ( 7), infection ( 7), cardiac failure ( 6), and renal failure ( 1). Conclusions: Because of a recent increase in the incidence of IE and high mortality and complication rate, it is mandatory to establish well formulated recommendations for management of IE in paediatric and adult patients with CHD based on a large cohort. Results of this nationwide multicentre database should be helpful in establishing guidelines.