Pediatric coronary cameral fistula in a structurally normal heart: a case report and review of the literature

Abstract

Introduction: Coronary cameral fistulas (CCFs) are rare congenital anomalies characterized by abnormal connections between a coronary artery and one of the cardiac chambers. These abnormal connections can lead to significant clinical implications, including heart failure and myocardial ischemia, necessitating timely diagnosis and intervention.

Case presentation: A 5-year-old Iranian boy was brought to the emergency room at Heart Hospital Center complaining of chest pain and dyspnea on exertion. He had experienced similar episodes over the past 4 months, which had not been evaluated. Physical examination revealed stable vital signs and no remarkable findings. Transthoracic echocardiography demonstrated a dilated left coronary artery with a large aneurysm and a small orifice to the right ventricular body. The left ventricular ejection fraction was 55%. Cardiac computed tomography angiography confirmed the diagnosis. Cardiac angiography showed a dilated left coronary artery and a coronary cameral fistula to the right ventricular . The aneurysm was successfully occluded using two Amplatzer™ devices.

Conclusion: This case underscores the critical role of multimodal imaging in diagnosing and managing coronary cameral fistulae. Early detection and appropriate intervention are paramount in preventing the progression of symptoms and potential complications such as heart failure and myocardial ischemia. The successful closure with Amplatzer™ devices highlights the efficacy of minimally invasive techniques in treating complex cardiovascular anomalies. Regular follow-up and careful monitoring are essential to ensure long-term success and to manage any potential recurrences.

Clinical key message: Timely identification and management of coronary cameral fistulae are crucial to prevent complications. Advances in imaging techniques and minimally invasive treatments, such as transcatheter closure, offer effective solutions. A multidisciplinary approach and regular follow-up are essential for comprehensive care and successful long-term management.

Keywords: Coronary angiography; Coronary artery anomalies; Coronary cameral fistula.

Fig. 1

(A) and (B) Three-dimensional cardiac computed tomography angiography (CTA) images showing a coronary cameral fistula with a dilated left coronary artery (LCA) and a large aneurysm. Both views (A and B) illustrate the aneurysmal dilation of the LCA, with the arrows indicating the location of the fistula and aneurysm

Fig. 2

Cardiac computed tomography angiography (CTA) image showing a coronary cameral fistula (indicated by the arrow). The CTA highlights the connection between the coronary artery and a cardiac chamber, providing a clear view of the anatomical anomaly

Fig. 3

Cardiac angiography:A shows the coronary cameral fistula (indicated by the arrow). B illustrates two coronary cameral fistula orifices to the right ventricular (RV) cavity (indicated by the arrow). C shows the coronary cameral fistula immediately after the use of the Amplatzer™ device (indicated by the arrow). D shows the coronary cameral fistula 2 minutes after using the Amplatzer™ device, with successful occlusion (indicated by the arrow)

Fig. 4

Chest x-ray taken 1 day after the procedure, showing no signs of complications. The heart and lungs appear normal, and the Amplatzer™ devices are in place and visible (yellow arrow)