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Canadian Journal of Cardiology

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

Published:February 05, 2018DOI:https://doi.org/10.1016/j.cjca.2018.01.090
      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.
      • Saboo S.
      • Steigner M.
      • Ghosh N.
      • et al.
      Multimodality non-invasive imaging of a coronary cameral fistula.
      A preliminary diagnosis made using 2-dimensional transthoracic echocardiogram is exceedingly rare and only documented in case reports.
      • Saboo S.
      • Steigner M.
      • Ghosh N.
      • et al.
      Multimodality non-invasive imaging of a coronary cameral fistula.
      • Maragkoudakis S.
      • Patrianakos A.
      • Kallergis E.
      • et al.
      Echocardiographic demonstration of coronary artery to left ventricle fistulas: case report and review of literature.
      • Li Y.
      • Xie M.
      • Wang X.
      • et al.
      Two giant right coronary artery aneurysms with fistula to the left ventricle: preliminary diagnosis by echocardiography.
      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.
      • Li Y.
      • Xie M.
      • Wang X.
      • et al.
      Two giant right coronary artery aneurysms with fistula to the left ventricle: preliminary diagnosis by echocardiography.
      Using a multimodality noninvasive approach a diagnosis of CCF can be conclusively made.
      Figure thumbnail gr1
      Figure 1Transthoracic and Doppler echocardiography. (A) Apical 4-chamber view with side by side comparison of 2-dimensional images. (B) Parasternal short-axis view. (C) Off-axis parasternal long view with side by side comparison of 2-dimensional images with colour Doppler flow present in diastole (arrow). (D) Non-contrast-enhanced continuous-wave signal. (E) Contrast-enhanced Doppler signal. LV, left ventricle; RA, right atrium; RV, right ventricle.
      Figure thumbnail gr2
      Figure 2Comparison of 3-D computed tomography with invasive coronary angiography images. (A) and (B) Computed tomography images of the RCA illustrating drainage into the LV with large aneurysm present (arrow). (C) Angiography showing the RCA with a large aneurysm at the LV insertion site (arrow). LA, left atrium; LV, left ventricle; RA, right atrium; RCA, right coronary artery; RV, right ventricle.
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