Krasic Stasa, Butera Gianfranco, Topic Vesna, Vukomanovic Vladislav
Cardiology Department, Mother and Child Health Institute of Serbia, R. Dakica St. 6-8, 11070 Belgrade, Serbia.
Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia.
J Cardiovasc Dev Dis. 2024 Sep 25;11(10):298. doi: 10.3390/jcdd11100298.
Coronary artery fistulas (CAFs) are rare congenital anomalies with an occurrence rate of 0.002-0.3%. The right coronary artery (RCA) is reportedly the most common site of origin of CAFs, but fistulas draining to the left atrium (LA) are rare. We presented a three-year-old boy with a symptomatic congenital RCA-to-LA fistula, which was successfully percutaneously occluded with an Amplatzer vascular plug 4 (AVP4).
The diagnosis was made by echocardiography when he was two months old. During the follow-up period of 2 years, a progressive dilatation of the RCA and enlargement of the left ventricle was detected, so treatment for congestive heart failure was initiated. At the age of three, the patient presented with a history of occasional mild central chest pain and discomfort and mild dyspnea on exertion. On a 24 h ECG Holter monitor, the depression of ST segments was registered. CT angiography highlighted a large type B RCA fistula to the LA, which extended along the atrioventricular sulcus. The proximal RCA diameter was 7 mm. The fistula was tortuous, with segmental narrowing and three curves. Cardiac catheterization was performed across the right femoral artery on the three-year-old boy (body weight: 13 kg). Across the 4F Judkins right guiding catheter, an AVP4 of 5 mm was placed in the distal part of the CAF connected with the delivery cable. After 15 min, ECG changes were not registered, so the device was released. Immediate post-deployment angiography demonstrated complete CAF occlusion, with satisfying flow in the distal coronary artery. The patient was discharged after four days. In the short-term follow-up period, the boy was symptom-free.
In our experience, given the existence of the left-to-left shunt and the more pronounced exercise-induced coronary steal phenomenon that occurs in medium-sized and large CAFs, occlusion is necessary to prevent the further progression of clinical signs and symptoms.
冠状动脉瘘（CAF）是一种罕见的先天性异常，发生率为0.002 - 0.3%。据报道，右冠状动脉（RCA）是CAF最常见的起源部位，但引流至左心房（LA）的瘘很少见。我们报告了一名3岁男孩，患有有症状的先天性RCA至LA瘘，成功地通过Amplatzer血管封堵器4（AVP4）进行了经皮封堵。
他2个月大时通过超声心动图确诊。在2年的随访期间，发现RCA进行性扩张和左心室扩大，因此开始进行充血性心力衰竭治疗。3岁时，患者有偶尔轻度中央胸痛和不适以及劳力性轻度呼吸困难的病史。24小时心电图动态监测显示ST段压低。CT血管造影突出显示一个大的B型RCA至LA瘘，沿房室沟延伸。RCA近端直径为7mm。瘘管迂曲，有节段性狭窄和三处弯曲。对该3岁男孩（体重：13kg）经右股动脉进行了心导管检查。通过4F Judkins右引导导管，将一个5mm的AVP4放置在与输送电缆相连的CAF远端部分。15分钟后，未记录到心电图变化，因此释放了该装置。封堵后即刻血管造影显示CAF完全封堵，冠状动脉远端血流满意。4天后患者出院。在短期随访期间，该男孩无症状。
根据我们的经验，鉴于存在左向左分流以及在中大型CAF中发生的更明显的运动诱发冠状动脉窃血现象，为防止临床体征和症状进一步进展，进行封堵是必要的。
