Background
Coronary artery anomalies (CAAs) are a diverse group of congenital malformations, which clinical manifestations and pathology are highly variable . Being rare, they are recognized cause of myocardial ischemia and occasionally sudden cardiac death . The frequency of anomalous aortic origin of the coronary arteries is noted in 0,6 to 1,2 % of patients (based on coronary angiography), with significant higher incidence in young victims of sudden cardiac death in comparison to adults (4-15% versus 1% respectively). There is no racial or sexual predisposition. The initial pathology of prolonged myocardial ischemia, may result in typical or atypical angina, syncope, arrhythmias, and infractions .
Case presentation
We report the case of 28-year-old Caucasian male, with no remarkable family history who was referred to the Local Hospital due to non specific chest symptoms which occurred on relation to the New Year celebrations. His baseline ECG showed ST-T elevations in leads I, aVL, deep negative T waves in III, aVR. His troponins were grossly elevated. Therefore, suspecting acute myocardial infarction, coronarography was conducted, which did not reveal any significant stenosis in coronary arteries. However, a muscular bridge narrowing left anterior descending artery (LAD) to 50% during the systole was observed and the anomalous origin of the right coronary artery was seen. In a view of patient’s symptoms complete withdrawal, as well patient’s specific request, he was discharged home. One year later he was admitted to our Centre due to unspecific chest discomfort and palpitations. At presentation he was 70 kg weight and 178 cm height. Physical examination revealed clear, correctly accented heart sounds, alveolar murmur on lungs auscultation, 60/min heart rate, blood pressure110/70 mm Hg, oxygen saturation 96%. Biochemical parameters such as hemoglobin, amount of erythrocytes, liver functioning enzymes (ASpat, Alat), renal parameters (eGFR) and lipids profile were normal. 12–lead ECG showed sinus rhythm, 70/min, left deviated axis, ST segment elevation (up to 1-2 mm) in leads I, aVL and deep negative T waves in III, aVR. Echocardiography showed normal size of heart chambers and preserved systolic function of left ventricle. Also coronarography was conducted which confirmed the anomalous origin of the right coronary artery (RCA) from the anterior part of the ascending aorta. Likewise, no significant stenosis was observed and surprisingly no myocardial bridge over the LAD was noted. To broaden the diagnosis, the angio-CT was scheduled, which confirmed the anomalous origin of RCA from the left Valsalva sinus, just above the origin of the left coronary artery. Moreover, the atypical course of the vessel between aorta and pulmonary artery was noted. As those two great vessels may have compressed the meandering coronary artery and thus be a precipitating factor of symptoms, the perfusion scyntygraphy was performed. The result of the test was negative, meaning that there is not a compression on the RCA either at rest or during the exercise. As a result the decision has been made to manage the patient conservatively and he was prescribed with bisoprolol 2,5 mg per day. Since that time he has been symptom-free and remains in occasional follow-up.
References
1. Coronary artery anomalies- Paulo Angelini – Circulation 2007
2. Alexander RW, Griffith GC. Anomalies of the coronary arteries and their clinical significance. Circulation 1956;14(5):800–5.
3. Loukas M, Sharma A, Blaak C, Sorenson E, Mian ,J Cardiovasc Transl Res. 2013 Feb 20. The clinical anatomy of coronary arteries
4. Angelini P, Velasco JA, Flamm S. Coronary anomalies: incidence, pathophysiology, and clinical relevance. Circulation 2002;105(20):2449–54.
Expert’s comments:(Written authorization required from each expert)
1. Prof. Piotr Podolec MD, Ph.D
The patient presented ECG changes and had highly elevated troponins level which clearly suggested an incident of ischemia of the heart and determined the reasons to conduct the coronarography.
2. Prof. Piotr Pieniążek MD, Ph.D
Based on clinical manifestation and the results of obtain imaging there is no evidence of underwent infarction of the heart. Anomalies of coronary arteries are rare and mostly asymptomatic. Patient should be under observation.
3. Prof. Tadeusz Przewłocki MD, Ph.D
Anomalies of coronary arteries generally are asymptomatic findings during the standard x-ray coronarography. There are no particular guidelines to managing with patients. They should remain under observation.
4. Grzegorz Kopeć MD, Ph.D
As there is no evidence of underwent heart infarction and there is no hemodynamic relevant of anomalous origin and course of right coronary artery patient should be mange conservatively.
5. Magdalena Kostkiewicz MD, Ph.D
Based on perfusion scyntygraphy both: at rest and during the exercise the ischemia of myocardium has been unambiguously excluded. Patient should be manage conservatively and follow up occasionaly.
6. Jakub Podolec MD, PhD
However some anomalies of the coronary circulation may present with symptoms, most of then are asymptomatic or not recognized. Among all coronary anomalies, the subgroup with origination of coronary arteries from the opposite sinus have the most potential for adverse clinical presentation, especially sudden cardiac death in young people. Angelini et al. suggests that it is very important to establish diagnostic screening protocols to prevent adverse events especially in people undergoing extreme excertion. Uebleis et al. found no correlation between the anatomical variants of coronary anomalies and presence of myocardial ischemia while using hybrid imaging evaluation of patients with symptomatic abnormal origin of coronary arteries with multi detector – CT for coronary CT angiography and stress-rest myocardial perfusion SPECT. Therefore this young patient should be treated conservatively at this point and evaluated with one of the imaging methods at least once every year, because symptoms, even not typical for angina occurred and the presence of myocardial bridge over the LAD was previously diagnosed. It is probable that during the first coronaroangiography Prinzmetal angina has been diagnosed. That could explain symptomatic patient presentation. Avoiding extreme exercise should be also recommended to this patient.
Expert’s conclusions:
Anomalies of coronary arteries are quite rare and mostly they are asymptomatic findings during the standard x-ray coronarography. There are no particular guidelines. As the reported patient had no evidence of underwent myocardial infarction and the incidence of ischemia – related to abnormal origin and course of right coronary artery – was definitely excluded, he should be manage conservatively and remain follow up occasionally.
Authors:
Sylwia Wiśniowka-Śmiałek MD1, Agata Leśniak-Sobelga MD1, Paweł Rubiś MD, Ph.D1, Maciej Krupiński MD.1
Experts:
Prof. Piotr Podolec MD, Ph.D1, Prof. Piotr Pieniążek MD, Ph.D1, Prof. Tadeusz Przewłocki MD, Ph.D1, Magdalena Kostkiewicz MD, Ph.D1, Grzegorz Kopeć MD, Ph.D1, Jakub Podolec MD2.
1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
2Department of Hemodynamics and Angiocardiography, Cardiology Institute, Collegium Medicum, Jagiellonian University, Krakow, Poland; Center for Interventional Cardiac and Vascular Diseases, John Paul II Hospital, Kraków, Poland
