Table 1.

Figure 1.

Figure 2. AP: pulmonary artery. DA: Left anterior descending coronary artery

Figure 3.

INTRODUCTION

Coronary fistulas are rare anomalies that amount to 0.3% of congenital heart diseases (1-3). To this day, the incidence rate of coronary-pulmonary fistulas (CPF) has increased due to the widespread use of cardiac computed tomography and represents 15% to 30% of all coronary fistulas reported (4) In the last clinical practice guidelines published by the American College of Cardiology on congenital heart diseases (2018) no consensus has been achieved on the management of this condition (5). For some authors the therapeutic approach should be based on the size of the fistula, presence of symptoms, anatomy of the fistula, age of the patient, and presence of associated heart diseases (6).

CLINICAL CASE

We present the case of a 58-year-old woman (former smoker) with cardiovascular risk factors and no significant past medical history and NYHA functional class III dyspnea of 2-month clinical evolution.

Previous studies:

ECG: sinus rhythm, 80 bpm, no pathological findings.

Echocardiogram: no pathological findings.

Exercise echocardiography: positive for myocardial ischemia due to hypermotility of inferomedial and apical segments on maximum workload (900 kgm/min). During the exercise the patient showed signs of dyspnea.

It was decided to perform a coronary angiography:

Coronary angiography (Figure 1). Left main coronary artery: left main coronary artery of good caliber without angiographically significant stenosis. Left anterior descending coronary artery: of good caliber, reaches the apex, and there are not traces of angiographically significant stenosis. The coronary-pulmonary fistula (CPF)—of significant blood flow—originates in its proximal segment. Its first segment has a linear course of approximately 3 mm in diameter and 5 cm in length. Also, it shows another sinuous segment that runs into the pulmonary artery trunk. Circumflex artery: non-dominant, of good caliber with no signs of significant stenosis on the angiography performed. Right coronary artery: dominant, of good caliber and without angiographically significant stenosis.

After diagnosing CPF a cardiac computed tomography is performed to complete de anatomical assessment.

Tomography (Figure 2)

The case was studied by the hospital heart team. Since it was a high-flow large-caliber CPF, it was decided to proceed with its endovascular closure.

The right radial access was used. A total of 7000 IU of sodium heparin were administered. The left main coronary artery was selectively catheterized using a 6-Fr extra-backup guide catheter. The Progreat microcatheter (Terumo) was placed at distal level with respect to the linear trajectory of the CPF. Embolization was attempted through the controlled release of two 3 mm x 5 cm detachable coils (AZUR detachable coil system, Terumo, United States). Fifteen minutes later, the coronary angiography performed reveals the partial closure of the CPF that remains patent with blood flow through the afferent branch not seen on the diagnostic study and that originates proximal to the coils recently implanted. It was decided to complete the embolization with a 3 mm x 2 cm fiber coil (AZUR, Terumo, United States). Ten minutes later the angiography performed reveals the total closure of the fistula (Figure 3). After completing the procedure, the patient remained in the coronary unit for 24 hours. At the 1-month follow-up the patient’s symptoms improved with no signs of dyspnea while performing activities of daily living.

DISCUSSION

Coronary fistulas are rare anomalies that amount to 0.3% of all congenital heart disease (1-3). To this date, the incidence rate of CPF has increased thanks to the widespread use of cardiac computed tomography and represents 15% to 30% of all coronary fistulas (4). Although former studies have reported that the most common origin of CPFs is the right coronary artery, a recent systematic review confirmed that the most common origin of CPFs is the left main coronary artery (84% of the cases), most of them draining into the pulmonary artery trunk (89%) (7). Two different types of CPF have been reported, one of them is a large sized single fistula; the other a small sized fistula multiple connections (7). It is more likely that large sized single fistulas are associated with the generation of hemodynamic changes and symptom onset. The therapeutic approach should be based on the size of the fistula, the presence of symptoms, the anatomy of the fistula, age of the patient, and presence of associated heart diseases (6). Asymptomatic patients with small sized fistulas are often treated with antiplatelet therapy and antibiotic prophylaxis. and disease progression monitoring. According to the clinical practice guidelines published by the American College of Cardiology—regardless of symptoms—the corrective treatment of large sized CPFs and small and medium sized fistulas in symptomatic patients (including myocardial ischemia, arrythmias, ventricular dysfunction, and endarteritis) has a class IC indication.5 Treatment options are surgical ligation and endovascular treatment. The most adequate therapeutic option will depend on the characteristics of each particular case (Table 1) (7).

CONCLUSION

The scientific literature available on coronary-pulmonary fistulas is scarce. We presented the case of an asymptomatic APF successfully treated with endovascular treatment.

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7. Gabin Yun, MD Tae Hyun Nam, MS Eun Ju Chun, MD, PhD. Coronary Artery Fistulas: Pathophysiology, Imaging Findings, and Management. RadioGraphics 2018;38:688-703.

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10. Gowda ST, Forbes TJ, Singh H, et al. Remodeling and thrombosis following closure of coronary artery fistula with review of management: large distal coronary artery fistula—to close or not to close? Catheter Cardiovasc Interv 2013;82(1):132-42.

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