coronary artery anomalies are rare occurrences , with an incidence ranging from 0.17% in autopsy studies to 1.3% in angiographic series .
these anomalies are generally asymptomatic , but more potentially serious ones may lead to myocardial ischemia , myocardial infarction ( mi ) and/or sudden death . in particular , st - segment elevation mi ( stemi ) is a rare clinical presentation in patients with coronary artery anomalies . as suggested by current guidelines ,
all patients presenting with stemi should receive emerging coronary artery angiography ( caa ) and subsequent primary percutaneous coronary intervention ( pci ) after culprit lesion identification .
unfortunately , in these patients , the identification of the culprit lesion and its treatment may be difficult and complex . from january 2008 to april 2011 , 1015 stemi patients received emergent caa and primary pci in our centre .
of these , 5 ( 0.4% ) patients showed a coronary artery anomaly . in this paper
we reported the clinical management , history and follow - up of two really rare cases , with a very uncommon coronary artery anatomy .
a 61-year - old woman with no past medical history presented to the emergency department with typical chest pain lasting for 45 min .
her electrocardiogram ( ecg ) showed sinus rhythm , right bundle branch block and significant st - segment elevation in v1-v3 leads .
rca courses in all coronary sulcus and gives the posterior descending artery ( encircling the apex ) , two posterolateral branches and three obtuse marginal branches .
these vessels supply all rca and left circumflex artery territory . from the first portion of rca two small anomalous vessels arose ( figure 1 ) .
the first one ( asterisk , figure 1 ) goes into the middle portion of the anterior interventricular sulcus ( ais ) , sprouting diagonal branches .
the other one ( arrow head , figure 1 ) reaches the proximal ais , giving side branches ( figure 1 ) . during the procedure , spontaneous symptom relief and st - segment resolution were observed .
after procedure , no recurrent angina was observed and medical therapy with aspirin , clopidogrel , ramipril , metoprolol and atorvastatin was titrated . a significant increase of myocardial necrosis markers ( ck - mb peak 21.2 ng / ml and troponin i peak 0.91 ng / ml ) above the upper normal range ( 5 ng / ml and 0.15 ng / ml , respectively ) was observed .
four days after , a 256 slices ecg - gated multidetector computerized tomography ( mdct ) study of coronary arteries was done to delineate the anatomy and the relationship with the great vessels .
the mdct study showed that the first anomalous vessel ( asterisk , figure 2 ) hurdles the pulmonary artery ( pa ) and supplies the medium portion of antero - lateral wall . on the contrary , the second anomalous vessel ( arrow head , figure 2 ) courses behind the pa and reaches the basal segment of antero - lateral wall .
curiously , these two anomalous vessels hug the pa and form a singular horseshoe variant of left anterior descending ( lad ) artery ( figure 2c ) .
two days after the mdct study , the patient was discharged and the 1 year follow - up was uneventful .
coronary artery angiography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
coronary artery angiography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
256-slice electrocardiogram -gated computed tomography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
256-slice electrocardiogram -gated computed tomography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
a 71-year - old male with a history of hypertension and hyperlipidemia was admitted to our emergency department with the diagnosis of anterior - lateral stemi .
he complained about chest pain that he had for 2 h. a cca was immediately performed and after a very complex exam the coronary artery anatomy was clarified .
the second coronary artery arises and reaches the mid portion of ais ( figure 3b ) .
this vessel shows a distribution and side branches similar to a normal mid - distal lad and a lesion on proximal segment . finally , only the first portion of a third vessel is visible ( figure 3a , red arrow ) .
it 's distal portion is visible via collateral branches arising from the lad and supplies the territory of circumflex artery and of proximal lad ( asterisk , figure 3b ) . with many difficulties ,
a pci with only balloon was performed ( it was impossible to deliver the stent for the lack of support ) . at the end of the procedure
the final timi flow in the culprit vessel was 2 and a partial resolution of symptoms and of st - segment elevation were observed .
medical therapy with aspirin , perindopril , metoprolol , nitrates and simvastatin was immediately titrated .
cardiac markers confirmed the necrosis ( ck - mb peak 42.8 ng / ml and troponin i peak 1.4 ng / ml ) .
five days after , a mdct study of coronary arteries was done ( figure 4 ) . in particular , mdct permitted to better clarify the relationship between great vessels and coronary arteries .
one of the two anomalous vessels , to reach the mid - distal anterior wall , runs behind the pulmonary artery ( asterisk , figure 4 ) .
contrarily , the other anomalous vessel with the culprit lesion shows a retro - aortic course ( arrow head , figure 4 ) . considering the residual stenosis in the three vessels , the impossibility to obtain a percutaneous revascularization of coronary artery with retro - aortic course , and the relationship between anomalous vessels and aorta and pulmonary artery , surgical revascularization
red arrow : culprit lesion . * collateral branches for distal segments of culprit vessel .
red arrow : culprit lesion . * collateral branches for distal segments of culprit vessel .
256-slice electrocardiogram -gated computed tomography showing the relationship between great vessels and coronary arteries and their distribution to myocardial tissue .
256-slice electrocardiogram -gated computed tomography showing the relationship between great vessels and coronary arteries and their distribution to myocardial tissue .
a 61-year - old woman with no past medical history presented to the emergency department with typical chest pain lasting for 45 min .
her electrocardiogram ( ecg ) showed sinus rhythm , right bundle branch block and significant st - segment elevation in v1-v3 leads .
rca courses in all coronary sulcus and gives the posterior descending artery ( encircling the apex ) , two posterolateral branches and three obtuse marginal branches .
these vessels supply all rca and left circumflex artery territory . from the first portion of rca two small anomalous vessels arose ( figure 1 ) .
the first one ( asterisk , figure 1 ) goes into the middle portion of the anterior interventricular sulcus ( ais ) , sprouting diagonal branches .
the other one ( arrow head , figure 1 ) reaches the proximal ais , giving side branches ( figure 1 ) . during the procedure , spontaneous symptom relief and st - segment resolution were observed .
after procedure , no recurrent angina was observed and medical therapy with aspirin , clopidogrel , ramipril , metoprolol and atorvastatin was titrated . a significant increase of myocardial necrosis markers ( ck - mb peak 21.2 ng / ml and troponin i peak 0.91 ng / ml ) above the upper normal range ( 5 ng / ml and 0.15 ng / ml , respectively ) was observed .
four days after , a 256 slices ecg - gated multidetector computerized tomography ( mdct ) study of coronary arteries was done to delineate the anatomy and the relationship with the great vessels .
the mdct study showed that the first anomalous vessel ( asterisk , figure 2 ) hurdles the pulmonary artery ( pa ) and supplies the medium portion of antero - lateral wall . on the contrary , the second anomalous vessel ( arrow head , figure 2 ) courses behind the pa and reaches the basal segment of antero - lateral wall .
curiously , these two anomalous vessels hug the pa and form a singular horseshoe variant of left anterior descending ( lad ) artery ( figure 2c ) .
two days after the mdct study , the patient was discharged and the 1 year follow - up was uneventful .
coronary artery angiography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
coronary artery angiography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
256-slice electrocardiogram -gated computed tomography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
256-slice electrocardiogram -gated computed tomography of single coronary artery . * anomalous vessel for the middle portion of the anterior interventricular sulcus .
white arrow head : anomalous vessel for the proximal portion of the anterior interventricular sulcus .
a 71-year - old male with a history of hypertension and hyperlipidemia was admitted to our emergency department with the diagnosis of anterior - lateral stemi .
he complained about chest pain that he had for 2 h. a cca was immediately performed and after a very complex exam the coronary artery anatomy was clarified .
the second coronary artery arises and reaches the mid portion of ais ( figure 3b ) .
this vessel shows a distribution and side branches similar to a normal mid - distal lad and a lesion on proximal segment .
finally , only the first portion of a third vessel is visible ( figure 3a , red arrow ) .
it 's distal portion is visible via collateral branches arising from the lad and supplies the territory of circumflex artery and of proximal lad ( asterisk , figure 3b ) . with many difficulties ,
a pci with only balloon was performed ( it was impossible to deliver the stent for the lack of support ) . at the end of the procedure
the final timi flow in the culprit vessel was 2 and a partial resolution of symptoms and of st - segment elevation were observed .
medical therapy with aspirin , perindopril , metoprolol , nitrates and simvastatin was immediately titrated .
cardiac markers confirmed the necrosis ( ck - mb peak 42.8 ng / ml and troponin
five days after , a mdct study of coronary arteries was done ( figure 4 ) . in particular , mdct permitted to better clarify the relationship between great vessels and coronary arteries .
one of the two anomalous vessels , to reach the mid - distal anterior wall , runs behind the pulmonary artery ( asterisk , figure 4 ) .
contrarily , the other anomalous vessel with the culprit lesion shows a retro - aortic course ( arrow head , figure 4 ) . considering the residual stenosis in the three vessels , the impossibility to obtain a percutaneous revascularization of coronary artery with retro - aortic course , and the relationship between anomalous vessels and aorta and pulmonary artery , surgical revascularization
red arrow : culprit lesion . * collateral branches for distal segments of culprit vessel .
256-slice electrocardiogram -gated computed tomography showing the relationship between great vessels and coronary arteries and their distribution to myocardial tissue .
256-slice electrocardiogram -gated computed tomography showing the relationship between great vessels and coronary arteries and their distribution to myocardial tissue .
coronary artery anomalies are the result of changes during the third week of fetal development .
the coronary artery anomalies are classified into anomalies : i ) of origin ii ) of courses iii ) of termination .
although their incidence is low , the potentially fatal consequences of coronary artery anomalies mean that it should always be taken into consideration by clinicians .
as this is the second most common cause of sudden death in young adults , after hypertrophic cardiomyopathy . the diagnosis can be challenging because patients are often asymptomatic and have a normal physical examination .
most patients are asymptomatic for a large portion of their lives , and an atypical chest - pain syndrome is the most common reason for which they are referred to for medical attention .
the milder cases are more likely to be identified fortuitously ( because of a falsely positive stress test and/or coincidental atherosclerotic disease ) .
coronary artery anomaly does not appear to be associated with an increased risk for the development of coronary atherosclerosis .
some authors reported an increase in stenosis of the anomalous lcx , but survival was not adversely affected within seven years .
conventional caa is regarded as the gold standard to diagnose coronary artery disease . however ,
it is particularly difficult to be selective in the ostium of anomalous vessel , and for the lack of 3d information , the relationship between coronary arteries and great vessels remain unclear . in the emergency setting all these difficulties are enhanced . during primary pci it is frequently difficult to identify the culprit lesion .
when the course of anomalous vessel and the culprit lesion are clear , often the revascularization procedure hides several pitfalls .
frequently the lack of support by guiding catheter limits the possibility to cross culprit lesion with guide - wire and/or to deliver balloon and stent .
analysing the last four years of emergent activity of our cath - lab , we found five ( 0.4% ) patients with stemi and coronary artery anomalies .
this data confirms that coronary artery anomalies are really rare and infrequent in daily clinical practice . even in a high - volume centre with skilled interventional cardiologists ( > 60 primary pci by year for operator ) , coronary artery anomalies presenting with stemi are a complex occurrence . of our five cases ,
two were really complex and in one patient ( case # 2 ) the reperfusion obtained was partial , whereas in the other patient ( case # 1 ) was luckily , spontaneous .
however , considering the coronary anatomy of this last patient , it was very difficult to predict a successful mechanical intervention .
in the first patient ( case # 2 ) we observed a 3-vessel disease with separate origins of three coronary arteries originating from the right sinus of valsalva .
as far as we know this anomaly is very uncommon and there are only few reports previously published .
additionally , the course of these anomalous vessels is peculiar , being one retro - aortic and the other between pulmonary artery and aorta . the second patient ( case # 1 ) presents a single coronary artery .
a single coronary artery is the rarest coronary anomaly and is an isolated finding that occurs in approximately 0.030.09% of the population , with a prevalence reported to be < 3% of all major coronary anomalies .
currently , the ideal imaging tool for the diagnosis and delineation of coronary artery anomalies is angiography supported by mdct .
mdct is particularly essential to clarify the relationship between coronary arteries and great vessels and the correct position of coronary arteries ostia .
these informations are crucial to select the correct revascularization procedure ( surgical vs. percutaneous ) and to correctly plan the intervention , if pci is preferred . in the primary pci setting
, the aim of each operator should be the reperfusion , also using only balloon angioplasty or thromboaspiration .
thereafter a mdct study may be planned and the final strategy of revascularization may be chosen . in conclusion ,
coronary artery anomalies presenting with stemi are really uncommon , but often are a challenge .
the integration between traditional caa and mdct is crucial to optimize the management of these patients .
 st - segment elevation mi ( stemi ) is a rare presentation in patients with coronary artery anomalies . in these patients , 
 the identification of the culprit lesion and its treatment may be difficult , particularly in the emergency setting of primary percutaneous coronary intervention ( pci ) . from january 2008 to april 2011 , 1015 stemi patients received coronary artery angiography and primary pci in our centre . 
 of these , 5 ( 0.4% ) patients showed a coronary artery anomaly . in this paper 
 we reported two rare cases : i ) the first is a single coronary artery originating from right sinus of valsalva ; ii ) the second is a separate origin of 3 coronary arteries originating from the right sinus of valsalva . in conclusion , 
 coronary artery anomalies presenting with stemi are really uncommon , but often are a challenge . 
 the integration between traditional coronary artery angiography and multidetector computerized tomography is crucial to optimize the interventional and medical management of these patients . 
Introduction
Case Reports
Case #1
Case #2
Discussion