Chest Pain Secondary to Recurrent Coronary-Pulmonary Artery Fistula

Case

A 40-year-old female with history of hypertension, dyslipidemia, and palpitations presented initially for evaluation in 2005. Work up for dyspnea at that time confirmed congenital heart disease with a fistula between the proximal left anterior descending (LAD) and proximal right coronary (RCA) arteries to the main pulmonary artery (PA) and the patient subsequently underwent surgical pulmonary artery fistula ligation in November of 2005.  She presented again in January 2011 with intermittent exertional angina and dyspnea on a medication regimen that included aspirin and metoprolol.  Physical examination was unremarkable, with a regular heart rate of 61 beats per minute, blood pressure of 116/57 mm Hg, respiratory rate of 18 breaths per minute, and an oxygen saturation of 96% on room air.

Echocardiogram was unremarkable with preserved left ventricular function and no obvious shunting into the PA. Right heart catheterization revealed elevated right-sided pressures with a modest step-up in the left PA consistent with significant left-to-right shunting. Diagnostic angiography showed two branch vessels of the RCA (conus and sinus atrial node branches) emptying into the left PA via a large plexus of abnormal collateral vessels (Figure 1a); two branches of the left anterior descending artery (LAD) communicating with the left PA (Figure 1b); and large, serpentine left internal mammary artery (LIMA) with extensive collateral network and brisk emptying into the left PA (Figure 1c).

Additional imaging with computed tomography (CT) angiogram was performed to further delineate the anatomy and confirmed the findings of arteriovenous malformations (AVM) between the coronaries and PA. CT head did not show any intracranial AVM concerning for potential intracerebral hemorrhage with anticoagulation.

The patient was subsequently scheduled for percutaneous coil embolization of anomalous coronary PA fistulas. A 6 French JR-4 guiding catheter was used to engage the RCA and a PT2 guide wire (Boston Scientific) advanced selectively into the conus and sinus atrial node branches. A Renegade microcatheter (Boston Scientific) was used to deploy 8 Interlock coils (Boston Scientific) in the conus branch and 5 in the sinus atrial node branch (Figure 2a). Next, an extra back-up (EBU)-3.5 guiding catheter was used to access the LAD and a total of 5 Interlock coils deployed to occlude the two branches of the LMCA. Finally, an IM guiding catheter was used to engage the LIMA graft and 11 coils deployed to demonstrate complete cessation of flow through the graft.

The patient was discharged home the following day after excellent angiographic obliteration of the AVM. She was seen in follow-up at 1 month, with resolution of symptoms on a medication regimen of aspirin, clopidogrel, and metoprolol.

Discussion

Coronary artery fistulas are believed to be embryological remnants of sinusdidal connections between the lumens of the primitive tubular heart, first described by Maude Abbott in 1908.¹ A coronary artery fistula involves a sizable communication that bypasses the myocardial capillary bed and enters either a chamber of the heart (coronary-cameral fistula), or any segment of the systemic or pulmonary circulation (coronary arteriovenous fistula).² Most small fistulas do not cause any hemodynamic compromise, but large fistulas can result in coronary artery steal phenomenon, especially during activity.³ Adults with significant shunts may present with congestive heart failure, syncope, arrhythmias, fatigue, dyspnea on exertion or angina.⁴ On clinical exam, a continuous diastolic murmur in the lower sternal border may be ausculated.⁵ The diagnosis is usually made nonivasively with echocardiography, computed tomography angiography, or magnetic reasonance angiography; however, coronary angiography remains the imaging standard by defining the anatomy, course, and nature of the insertion.⁶

Lifestyle modifications, including a low-cholesterol diet and smoking cessation, are recommended in all patients with chest pain due to coronary artery disease, in addition to optimized medical therapy for hyperlipidemia, hypertension, and antiplatelet therapy.⁷ However, for patients with coronary fistulas that result in angina⁸, definitive surgical or percutaneous treatment is recommended for refractory symptoms. While the surgical approach requires open ligation of the fistula⁹, the percutaneous strategy utilizes endovascular coil embolization.⁵ The shorter recovery period of the percutaneous approach makes this an attractive option, especially for patients who have previously undergone surgery. On the other hand, the need for multiple, staged interventions may render the percutaneous route more challenging and increase the risk of complications, including spasm, arrhythmia, perforation and iatrogenic catheter-induced dissection.⁶ Our case, herein, illustrates a unique case of congenital coronary-pulmonary fistula refractory to surgical therapy and treated successfully via percutaneous coil embolization.

The authors may be contacted via Dr. Jon George at georgej@deborah.org.

References

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Disclosure: Dr. George reports he is a consultant for Boston Scientific. Dr. Patel and Dr. Kovach report no conflicts of interest regarding the content herein.