ISSN : 1301-5680
e-ISSN : 2149-8156
Turkish Journal of Thoracic and Cardiovascular Surgery     
Clinical characteristics and outcomes in patients with endocardial fibroelastosis associated with critical aortic stenosis with biventricular circulation
Vehbi Doğan1, Senem Özgür1, İlker Ertuğrul1, Tamer Yoldaş1, Utku Arman Orun1, Murat Koç2, Selmin Karademir1
1Departments of Pediatric Cardiology, Dr. Sami Ulus Maternity and Children Research and Training Hospital, Ankara, Turkey
2Departments of Cardiovascular Surgery, Dr. Sami Ulus Maternity and Children Research and Training Hospital, Ankara, Turkey
DOI : 10.5606/tgkdc.dergisi.2015.10934
Background: This study aims to assess the characteristics, results and prognosis of patients with endocardial fibroelastosis secondary to critical aortic stenosis.

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.

Keywords : Aortic valvuloplasty; critical aortic stenosis; endocardial fibroelastosis; newborn
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