Anomalous origin of the right coronary artery from the pulmonary artery and simultaneous coronary-bronchial artery fistula.

A 63-year-old male presented to cardiology clinic with complaints of dyspnea and chest pain during exercise since 2 months. The patient’s medical history and family history were unremarkable. He was an ex-smoker and has a history of 10 pack-years of smoking. His cardiac examination was normal, except for tachycardia. His extremities were free of edema. Atrial fibrillation was detected in the patient’s electrocardiogram, and laboratory results showed slightly elevated troponin level (0.91 ug/L; normal range, <0.1 ug/L). Complete blood count and other laboratory test results were within normal limits. Transthoracic echocardiogram revealed depressed left ventricular function with an estimated ejection fraction of 35%, global hypokinesia in the left ventricular walls, and mild pericardial effusion. The patient underwent coronary angiography with a preliminary diagnosis of non-ST segment elevation myocardial infarction. The left main coronary artery and its branches were found to be normal. However, the right coronary artery (RCA) ostium was unidentifiable, and there appeared to be collaterals with retrograde filling of the RCA (Fig. 1). The patient underwent coronary computed tomography angiography (CTA) for further investigation with suspicion of anomalous origin of the RCA. Coronary CTA demonstrated an anomalous RCA from the pulmonary artery (ARCAPA) (Fig. 2). Also, there were hypertrophied bronchial arteries in mediastinum, and bronchial-coronary artery fistula between the bronchial artery and the proximal part of the ARCAPA was observed (Fig. 3). With a diagnosis of simultaneous ARCAPA and coronary-bronchial artery fistulae, surgical treatment was recommended to the patient but he refused the surgery.

Figure 1

Coronary angiography image shows retrograde filling of the right coronary artery

Figure 2

Coronary computed tomography angiography with volume rendering image demonstrates an anomalous right coronary artery from the pulmonary artery

Figure 3

Coronary computed tomography angiography with a multiplanar reconstruction image shows a bronchial-coronary artery fistula between the hypertrophied bronchial artery and the proximal part of the anomalous right coronary artery from the pulmonary artery

Both coronary-bronchial artery fistula and ARCAPA are very rare congenital anomalies. The estimated rate of ARCAPA and coronary-bronchial artery fistulae was in 0.002% and 0.18% of the population, respectively (1, 2). To the best of our knowledge, this is the first case of ARCAPA and simultaneous bronchial artery-ARCAPA fistula. Most of the cases with ARCAPA or coronary-bronchial artery fistula are asymptomatic during infancy and early childhood. Therefore, those are more frequently detected in late adolescents and young adults (1-3). These anomalies have been rarely reported in the elderly, as in our case.

Coronary CTA is currently the best method for the detection and characterization of coronary artery anomalies due to the ability to define the origin and course of the coronary arteries (4). In line with the literature, both of these two rare anomalies (ARCAPA and coronary-bronchial artery fistulae) have been clearly demonstrated with coronary CTA. In conclusion, this case highlights the imaging findings of simultaneous ARCAPA and coronary-bronchial artery fistulae that are important for guidance during surgery.

Informed consent: Informed consent was obtained from the patient.