Coronary-Cameral Fistula: Preliminary Diagnosis According to Colour and Continuous Wave Doppler Echocardiography

A 66-year-old woman with no contributory medical history was worked up for a small left cerebellar infarct. As part of the workup, a 2-dimensional transthoracic echocardiogram was performed. Colour Doppler flow imaging revealed turbulent diastolic flow in the basal inferolateral left ventricular (LV) wall (Fig. 1, A-C; Video 1 , view video online). Continuous wave Doppler indicated diastolic flow signal, but was incompletely visualized (Fig. 1D). Ultrasound enhancing contrast was used to better delineate the Doppler signal (Fig. 1E). It was believed this was consistent with flow from a fistula into the left ventricle. She underwent a cardiac computed tomographic angiography scan and 3-dimensional reconstructed images showed a right coronary artery-LV fistulous connection to the posterior LV wall (Fig. 2, A and B). She later developed an episode of supraventricular tachycardia and proceeded to cardiac catheterization. This confirmed a large right ventricular marginal branch with aneurysmal fistula to LV chamber (Fig. 2C). Ultimately this was believed to be incidental, likely congenital in nature, and she was planned for an electrophysiology study for further characterization of her arrhythmia. Coronary-cameral fistula (CCF) is a fistula connection with cardiac chambers. It is a rare diagnosis seen in approximately 0.1% to 0.2% of patients referred for cardiac catheterization. A preliminary diagnosis made using 2-dimensional transthoracic echocardiogram is exceedingly rare and only documented in case reports. ^{,  ,}  Holo-diastolic flow from the epicardial surface in the left ventricle using continuous wave Doppler imaging has been shown to raise suspicion for CCF, but has never been re-demonstrated. Using a multimodality noninvasive approach a diagnosis of CCF can be conclusively made.