Multiple Coronary Artery Fistulae Associated with Bronchiectasis

Abstract

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A 62-year-old woman presented with symptoms of anginal chest pain. Her medical history included hypertension, hypercholesterolemia, and recurrent bronchitis. She had stopped smoking cigarettes 13 years previously. The clinical examination was unremarkable except for elevated blood pressure (148/80 mmHg) and crepitations at the base of the left lung. A 12-lead electrocardiogram was normal, and the results of an exercise tolerance test were equivocal. Left-heart coronary angiography showed minor coronary artery plaque disease and an anomalous circumflex vessel. Angiographic results also suggested the presence of coronary artery fistulous formations that possibly connected to a pulmonary artery (Fig. 1). Magnetic resonance imaging showed an anomalous left pulmonary artery, an anomalous circumflex coronary artery that originated from the proximal right coronary artery and gave rise to multiple coronary artery fistulae (Fig. 2), and bilateral basal bronchiectasis adjacent to the mediastinum.

Fig. 1 Coronary angiography shows an anomalous circumflex coronary artery (Anom Cx) that arises from the proximal segment of the right coronary artery (RCA) and gives rise to multiple coronary artery-to-bronchial artery fistulae (CAF).

Real-time motion image is available at www.texasheart.org/journal.

Fig. 2 Sagittal magnetic resonance imaging (T₁-prepared black blood) shows the anomalous circumflex coronary artery (Anom Cx) arising from the right coronary artery.

Asc Ao = ascending aorta; IVC = inferior vena cava; LA = left atrium; RA = right atrium; RMB = right main bronchus

High-resolution computed tomographic scans (Figs. 3 and 4), which confirmed the magnetic resonance imaging findings, revealed that the bronchiectatic changes were closely related to the fistulae and showed calcification within the bronchi (Fig. 4B). Computed tomography (Fig. 3) also suggested that the coronary artery fistulae were connected to both the pulmonary artery and the bronchial arteries. The patient's symptoms were under control; therefore, evaluation for surgical treatment was postponed.

Fig. 3 Volume-rendered thoracic computed tomography from the right posterior aspect shows coronary artery fistulae.

Ao A = aortic arch; CAF = coronary artery fistulae; LPA = anomalous left pulmonary artery; SVC = superior vena cava

Fig. 4 Axial computed tomography with contrast medium shows A) the anomalous left pulmonary artery (*) and B) the lung windows with bilateral basal medial bronchiectatic changes (arrows).

Asc = ascending aorta; Dsc = descending aorta; SVC = superior vena cava

Comment

Coronary artery fistulae are rare cardiac malformations.^1-3 In most cases, these fistulae have not been associated with other disorders.^1,2 However, imaging studies seem to be unveiling a pattern of associated disease—namely, bronchiectasis—in an increasing number of patients.

A few reports have suggested an association between coronary artery fistulae and bronchiectasis.^3-5 Our case may support this hypothesis, although further evidence is required to clarify the exact origin of bronchiectasis in such situations. This finding may affect clinical management and patient outcome. Occasionally, such cases are misdiagnosed, which can lead to inappropriate and potentially harmful treatment.⁴ The expanding availability of advanced imaging methods and improved imaging resolution greatly increases the likelihood of incidental findings such as the vascular anomalies that were revealed in this patient.