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Coronary-cameral fistula masquerading as a ventricular septal defect

Transthoracic echocardiography in an 8-year-old girl with fatigue and cyanotic spells since birth, revealed situs solitus, levocardia, atrioventricular concordance and double-outlet right ventricle (RV), a nonrestrictive subaortic ventricular septal defect (VSD) shunting bidirectionally, and a posterior muscular VSD (Figure 1). She had severe infundibular and valvular pulmonary stenosis with a peak gradient of 64mm Hg. The pulmonary annulus was adequate in size (15 mm) and ventricular function was normal. On computed tomography angiography, no additional VSDwas seen, the left circumflex artery (LCA) was enlarged, and there was a doubtful communication between the LCA and the RV (Figures 2 and 3). Intraoperatively, we found a subaortic VSD and muscle bands obstructing the RV outflow tract. In the posterior muscular area, there was an opening in the septum that communicated with the LCA but not the left ventricle. On infusion of cardioplegia into the aortic root, we noted immediate efflux of cardioplegia solution from the opening, confirming a coronary-cameral fistula. There was no additional VSD in the posterior septum. We routed the subaortic VSD to the aorta, performed infundibular resection, and closed the fistula Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(8) 992–993 The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313498757 aan.sagepub.com