Abstract 20256: Clinical Presentation, Management and Longitudinal Follow-Up of Neonatal and Infants With Coronary Artery Fistula - A Multicenter Study From the Coronary Artery Fistula Registry
Background: Neonatal coronary artery fistula is rare, and data regarding its clinical presentation, treatment, and long term outcome are lacking.
Methods: Data in all neonates and infants who underwent evaluation in the catheterization lab for coronary artery fistula (CAF) from Jan 1995 - Jan 2014 were reviewed from the multicenter CAF registry. The CAF were classified as proximal and distal for analysis. Results: A total of 21 patients were included from 12 centers.
Results: The median age and BSA was 0.26 yrs and 0.26 m2, respectively. At presentation, heart failure (HF) symptoms were noted in 14 / 21 (61%) pts; 11 had proximal and 10 had distal CAF. The largest and narrowest mean calibers of CAF were 6.9 and 3.3mm. Eleven pts underwent transcatheter closure (TCC), 7 surgical closure (SC), and 3 had medical observation only.
Procedural success was 100%,; intentional partial closure was performed in 1 patient with large distal fistula. Follow-up with median 2.3 ( 0.3-18 ) years in 90% pts revealed coronary events in 3 patients; 1 pt with large distal RCA fistula had myocardial infarction at 9 days post SC with ventricular dysfunction requiring thrombolytics and there was recovery of ventricular function. In 2/9 pts, follow-up angiography showed asymptomatic coronary thrombosis in 2; 1 with large distal RCA fistula and SC had complete RCA occlusion and remodeling with thread like vessels in the RCA at 2.5 yrs follow-up angiography. One patient with proximal LCA to left atrium fistula with severely dilated coronary sinus had SC, angiography 4 years later showed complete LCX occlusion from thrombus extension and retrograde filling from collaterals.
Conclusions: Neonates and infants with CAF frequently present with HF symptoms. They are treated successfully by transcatheter or SC, however, large distal CAF and proximal fistula with severely dilated coronary sinus are at risk for acute and chronic coronary events. Therefore, long-term follow-up coronary anatomical and functional evaluation is imperative for its evaluation and management. Partial closure of the large distal CAF may prevent coronary events following closure.
Author Disclosures: S.T. Gowda: None. S. Kutty: None. A. Jeremy: None. L. Prieto: None. Z. Chen: None. M. Miura: None. T. Atsuhito: None. A. Qureshi: None.
- © 2014 by American Heart Association, Inc.