A 70-year-old woman with atypical exertional chest pain history and suspicion of vascular malformation was referred to our institution for further diagnosis and treatment. Chest X-ray routinely performed by the general practitioner demonstrated a circularly enlarged shadow of the left border of the heart (Figure 1A).
Computed tomography angiography (CTA) and magnetic resonance (MR) revealed a coronary artery-pulmonary artery fistula arising from the proximal part of the left anterior descending (LAD) artery and emptying into the pulmonary trunk via a giant coronary aneurysm (65 mm × 52 mm × 50 mm, mass 95 g) with a large intraluminal thrombus (Figure 1B, C). Coronary angiography (CAG) confirmed the fistula and demonstrated no significant stenosis of the coronary arteries (Figure 1D, Supplementary material, Videos S1, S2).
A retrospective analysis of CTA performed two years earlier for other indications revealed the presence of an unrecognized fistula with an aneurysm measuring 47 mm × 49 mm × 43 mm.
Considering the progression of the aneurysm size, by the decision of the Heart Team, the patient was qualified for the endovascular closure of the fistula with endovascular coiling.
The procedure was performed using the right radial approach with the EBU 3.5 7 F guide catheter. Four coils (Optima Coil System, BALT USA LLC) were implanted into the fistula resulting in complete occlusion of the fistula feeding vessel (Figure 1E, Supplementary material, Video S3). The periprocedural period was uneventful, and the patient was discharged from the hospital the next day.
Review, six months after the procedure, showed full resolution of symptoms and return to normal activities. CTA and MRI demonstrated a reduction in the aneurysm size to 61 × 49 × 48 mm (mass 75 g). Coronary angiography showed complete closure of the connection between the fistula and the aneurysm, but with the preserved minor coronary artery-pulmonary artery connections, possibly as a result of pre-existing multiple drainage sites to the pulmonary artery (Figure 1F, Supplementary material, Video S4). Considering the reduction of symptoms and the size of the aneurysm, we decided on conservative treatment and further patient follow-up.
The prevalence rates for coronary-pulmonary artery fistulas (CPAF) range from 0.32% to 0.68% and most commonly terminate in the main pulmonary artery [1, 2]. Although CPAFs can be found incidentally and remain asymptomatic, in some cases, they have a significant hemodynamic effect and lead to complications, e.g., the formation of giant coronary artery aneurysms (GCAA), as in the case of our patient. Presentations of GCCA include cardiac symptoms of angina and myocardial infarction, probably caused by compression of the coronary artery by aneurysm and a coronary artery steal phenomenon [3, 4]. Moreover, pulmonary artery embolism due to aneurysmal thrombus formation and signs of mediastinal mass and cardiac compression, such as superior vena cava syndrome, may also occur [5].
Currently, we do not have recommendations for the treatment of CPAF and GCAA. The decision should be made as a team, based on the hemodynamic significance of the fistula and the risk of procedure-related complications.
Supplementary material
Supplementary material is available at https://journals.viamedica.pl/kardiologia_polska.
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