Coronary artery anomalies affect about 1% of the general population. The definition of the abnormal versus the normal coronary anatomy presents a complex problem. Defect may affect number of ostia, proximal course or termination. Many coronary anomalies don’t cause symptoms and remain undetected. However, they can be associated with chest pain, sudden death, cardiomyopathy, arrythmias and myocardial infarction.
We report a case of a 61-year-old Caucasian man referred to our Clinic. He had a history of coronary artery disease, arterial hypertension, diabetes mellitus type 2, lipid abnormalities and obesity (BMI- 30,2 kg/m2). In January 2012 he was hospitalized due to acute myocardial infarction with ST-segment elevation, treated with primary PCI RCA and BMS implantation. Furthermore, coronarography revealed fistula between the right coronary artery and the main pulmonary artery. On current admission he was hemodynamically stable with no signs of peripheral oedema. He reported exercise chest pain, he was in CCS class II. Blood laboratory analysis revealed increased level of glucose and slight anaemia. ECG showed sinus rhythm 60 bpm, low R wave in II, III, aVF, negative T wave in III. The transthoracic echocardiogram (TTE) showed enlargement of the left atrium (26 cm2), preserved left ventricular ejection fraction (EF 64 %), hypokinesia of cardiac apex, additional vessel 2.5 mm in diameter, terminated into the main pulmonary artery, 2 cm from annulus of the pulmonary trunk. Coronary angiography and IVUS revealed multivessel coronary artery disease: significant stenosis of LAD, Cx and Mg II. Myocardial perfusion scintigraphy showed decrease of exercise myocardial perfusion mainly in lateral and inferior wall. Post exercise ischemic area assessed as 25 %. Right heart catheterization excluded pulmonary hypertension. We performed CT heart scan which confirmed the presence of the fistula. The conduit had variable diameter about 4–5mm, originated 3-4 mm from the RCA ostium, terminated into the main pulmonary artery, 15 mm from annulus of the pulmonary trunk.
ESC Guidelines on myocardial revascularization (European Heart Journal 2010)
→ Indications for revascularization in stable angina:
- Any proximal LAD >50% – Class IA
- Proven large area of ischaemia (>10% LV) – Class IB
- Any stenosis >50% with limiting angina or angina equivalent, unresponsive to optimal medical therapy – Class IA
→ Indications for coronary artery bypass grafting vs. percutaneous coronary intervention in stable patients with lesions suitable for both procedures and low predicted surgical mortality:
- 3-vessel disease complex lesions, incomplete revascularization achievable with PCI, SYNTAX score >22 – Favours CABG – Class IA
1. Angelini P. Congenital Heart Disease for the Adult Cadiologist. Coronary artery anomalies. Circulation. 2007; 115: 1296-1305.
2. ESC Guidelines on myocardial revascularization. European Heart Journal. (2010) 31; 2511-2514.
Expert’s comments:(Written authorization required from each expert)
1. Krzysztof Bederski MD
Because of significant ischemia in the area of left coronary artery supply in heart scintigraphy CABG is recommended. Closing of coronaro-pulmonary fistula is a simple procedure and may eliminate ischemia in the field of RCA supplay.
Alternative option: Percutaneous closing of coronaro-pulmonary fistula and reassessment of myocardial perfusion in cardiac scintigraphy. After that re-evaluation of indications for
2. Henryk Siniawski MD, PhD
Ischemia in inferior wall observed in scintigraphy may be caused by coronaro-pulmonary fistula.
3. Prof. Egle Ereminiene MD, PhD
The patient is with chest pain (stable angina pectoris class II) after inferior MI with ST elevation – treated with PCA of RCA and BMS implantation several months ago. Coronary angiogram revealed multivessel coronary artery disease and fistula between RCA and the main PA. Myocardial perfusion decreased in inferolateral segments of the LV with exercise ( zone of Cx artery). As the patient is stable with extensive CAD and with diabetes mellitus, according to the guidelines, myocardial revascularization (CABG) is indicated in order to improve major adverse events free survival. Closure of coronaro-pulmonary fistula could be performed simultaneously during the procedure.
Patient was qualified for CABG (LIMA-LAD, SVG-Mg II) and closing of coronaro-pulmonary fistula. After three months control cardiac scintigraphy should be performed.
Justyna Błaut-Jurkowska MD1, Leszek Drabik MD1, Piotr Musiałek MD, PhD1
Lidia Tomkiewicz-Pająk MD, PhD1, Bartosz Laskowicz MD1, Prof. Maria Olszowska MD, PhD1, Prof. Piotr Podolec MD, PhD1, Prof. Tadeusz Przewłocki MD, PhD1
Krzysztof Bederski MD2, Henryk Siniawski MD, PhD3, Prof. Egle Ereminiene MD, PhD.4
1Department of Heart and Vascular Disease, John Paul II Hospital, Kraków, Poland
2Department of Thoracic Surgery in John Paul II Hospital, Krakow
3Deutsches Herzzentrum Berlin, Germany
4Department of Cardiology. Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania