Defining endocarditis in congenital heart disease

Document Type



Internal Medicine


PURPOSE: Two-thirds of the world's population resides in the developing world. In developing regions such as south Asia <2% of children with congenital heart disease (CHD) are surgically treated. The underlying CHD in patients complicated with infective endocarditis (IE) in these countries may be different in comparison to the West. This case-series attempts to describe IE in patients with CHD in Pakistan.
Patients with CHD and endocarditis admitted to Aga Khan University between 1988–2001 were retrospectively studied.
Forty-eight patients with CHD had IE by Modified Duke's Criteria. Twenty-two (46%) patients were >16 years old. Uncorrected ventricular septal defect (33%), teratology of fallot (12.5%), atrial septal defect (6.3%), patent ductus arteriosus (5%), coarctation of aorta (5%) and congenital mitral valve disease (12.5%) were the most common underlying defects. Patients with cyanotic defects, particularly of the complex type, were underrepresented (6.3%). Only 11 (22.9%) patients had a previous palliative or corrective surgery. Mean age at surgery was 4.6 +/- 3.5 years. Streptococci (33%), Staphylococcus epidermidus (8.4%) and pseudomonas spp. (4.2%) were the most common microorganisms. Twenty-two (45.8%) patients had culture negative endocarditis. Patients with cyanotic heart disease were more likely to have positive cultures (78% vs. 48.7%), renal failure (28% vs.8.3%) and thrombocytopenia (60% vs. 11.7%), although the first two failed to reach statistical significance. However, there were no differences in heart failure, need for surgery and mortality. Surgery was required in nine (18.7%) patients and 13 (27.1%) patients died. Patients with heart failure (p <0.001, 95% CI 3.6–84), pseudomonal infection (p 0.06, 95%CI 2.5–7) and nosocomial endocarditis (p 0.06, 95% CI 2.5–7) had increased mortality.
In contrast to developed countries, IE in Pakistan complicates uncorrected left-to-right shunts or TOF. Complex cyanotic defects are underrepresented probably because they are usually incompatible with life when not corrected.