Background
Neonatal and infantile presentation of coronary artery fistulae are rare, and data regarding its treatment, and long term outcome are lacking.
Methods
Retrospective collection of data in all neonates and infants (1 yr and less) who underwent evaluation in the catheterization lab for CAF from Jan 1995 – Dec 2015 were reviewed from the multicenter CAF registry. The CAF type (proximal or distal), size, treatment method and follow-up angiographic data were reviewed to assess coronary remodeling.
Results
A total of 48 patients (pts) were included from 20 centers. The median age and BSA was 0.16 yrs and 0.24 m
2. At presentation, heart failure (HF) symptoms were noted in 28 / 48 (58%) pts; 32 had proximal and 16 distal CAF; 42 were large, 7 medium and 2 small size CAF. The largest and narrowest mean calibers of CAF were 6.6 (2.8-11) and 2.9 (1.1-7.5) mm. Transcatheter closure (TCC) was performed in 24, surgical closure (SC) in 18, and 6 underwent medical observation. Follow-up was available in 37/48 (85%) pts, median 2.3 (0.3-18) yrs. Symptomatic acute coronary events were noted in 2; both had large distal right coronary artery (RCA) fistulae, with myocardial infarction at 9 days post SC and in 24hrs post TCC, respectively. In 17/42 (40%) pts, follow-up angiography showed: favorable remodeling in 11, persistent coronary dilation in 4, asymptomatic coronary thrombosis in 2- 1 with large distal RCA fistula following SC had complete RCA occlusion and remodeling with thread like vessels, 1 with proximal left coronary artery fistula following SC had complete circumflex coronary artery occlusion from thrombus extension and retrograde filling from collaterals. Duration of anticoagulation was < 1 year in 35/38pts; ASA in 35 and ASA + Coumadin in 4 pts. Three of 4 pts with coronary events had no anticoagulation; 3 had large distal CAF, 1 had proximal CAF with dilated aortic sinus.
Conclusions
Neonates and infants with CAF frequently present with HF symptoms and are treated successfully by TCC or SC. Patients with large distal CAF and proximal fistula with severely dilated aortic sinus are at risk for acute and chronic coronary events. Therefore, aggressive anticoagulation and long-term follow-up coronary anatomical and functional evaluation are warranted.