Methods: The study included 22 newborns (17 boys, 5 girls; mean age 7.5 days; range 0 to 28 days) having endocardial fibroelastosis associated with critical aortic stenosis between January 2008 and July 2014. Patients’ demographic, echocardiographic and cardiac catheterization, and angiography findings were reviewed.

Results: Patients’ mean weight at presentation was 3.3 kg (range 2 to 4.8 kg). Left ventricular systolic dysfunction with varying degrees (ejection fraction <55%) was present in 90% of the patients. Mean ejection fraction and fractional shortening were 34±13% and 17±6.5%, respectively. All patients were performed balloon aortic valvuloplasty. Peak-to-peak aortic valvular gradients before and after valvuloplasty were 56±23 and 22±15 mmHg, respectively. Early mortality rate was 31%.

Conclusion: Mortality rate is high in newborns with endocardial fibroelastosis associated with critical aortic stenosis. Identifying the patients whose chance of survival is higher with univentricular paliation rather than valvotomy or valvuloplasty may provide superior early results.