The single coronary artery arising from the sinus of Valsalva. A rare cause of myocardial ischemia

The presence of a congenital anomaly in coronary arteries can be the cause of a defective coronary flow and ischaemic symptoms. Although they are rare, we must suspect them in the presence of major cardiac events in young people. A single coronary artery is present if the entire coronary system arises from a solitary ostium. Its presence is regarded as having little clinical significance and it is usually a fortuitous finding on coronary angiography. We report the case of a patient with effort anginal symptoms, with a single coronary artery arising from the right sinus of Valsalva without obstructive atherosclerotic lesions.     

The role of transesophageal echocardiography in the detection of coronary anomalies and anatomical variations

OBJECTIVES: The aim of the study is to examine the role of transesophageal echocardiography (TEE) in the diagnosis of anomalies and anatomic variations of the coronary arteries. BACKGROUND: In the past, coronary angiography was the only method for diagnostic confirmation in all cases with coronary anomalies, but even during invasive procedures diagnostic difficulties could and can emerge. The different, varying origin of aberrant coronary arteries can prolong the diagnostic procedure, therefore can increase the irradiation time. So every method which seems to be suitable for diagnosis of suspected coronary anomalies can be helpful. METHODS: The origin and course of anomalous coronary arteries were studied by TEE and coronary angiography during a six-month period. RESULTS: We found 16 patients (2.8%) with coronary anomalies or variations by angiography, the diagnosis of which was technically difficult. Seven of these had TEE examination too. All seven anomalous origins proven angiographically and 2 of the 2 anomalous courses in the relation to the great vessels were diagnosed by TEE. (In two, the left circumflex originated from the right sinus of Valsalva, in two we found anomalous separate origin of left circumflex coronary artery from the left sinus, in another two a common ostium of the left anterior descending and circumflex artery from the left sinus and in one an accessory artery from the non-coronary sinus.) CONCLUSIONS: TEE in a useful test to diagnose the origin of anomalous coronary arteries and confirming their course in relation to the great arteries.     

Angina pectoris caused by coronary microvascular spasm

BACKGROUND: Microvascular angina can occur during exercise and at rest. Reduced vasodilator capacity of the coronary microvessels is implicated as a cause of angina during exercise, but the mechanism of angina at rest is not known. Our aim was to test the hypothesis that primary hyperconstriction (spasm) of coronary microvessels causes myocardial ischaemia at rest. METHODS: Acetylcholine induces coronary artery spasm in patients with variant angina. We tested the effects of intracoronary acetylcholine at graded doses in 117 consecutive patients with chest pain (at rest, during exertion, or both) and no flow-limiting (>50%) organic stenosis in the large epicardial coronary arteries. We also assessed the metabolism of myocardial lactate during acetylcholine administration in 36 of the patients by measurement of lactate in paired blood samples from the coronary artery and coronary sinus vein. FINDINGS: Of the 117 patients, 63 (54%) had large-artery spasm, 29 (25%) had microvascular spasm, and 25 (21%) had atypical chest pain. The 29 patients with microvascular spasm developed angina-like chest pain, ischaemic electrocardiogram (ECG) changes, or both spontaneously (two patients) or after administration of acetylcholine (27 patients) without spasm of the large epicardial coronary arteries. Testing of paired samples of arterial and coronary sinus venous blood showed that lactate was produced during angina attack in nine of 11 patients with microvascular spasm. There was more women (p<0.01) and fewer coronary risk factors (p<0.01) in patients with microvascular spasm than in those with large-artery spasm. INTERPRETATION: Coronary microvascular spasm and resultant myocardial ischaemia may be the cause of chest pain in a subgroup of patients with microvascular angina.     

Anatomic variations of the coronary arteries

PURPOSE: To study the coronary arteries and their main branches showing the aspects of source, trajectory and anastomoses of these vessels at the subepicardial level. METHODS: The study was carried out on 110 adult human hearts, of both sexes, fixed in 10% formaldehyde solution. The pericardium was removed to expose the coronary arteries and their branches at the subepicardial level. RESULTS: In 38.18% of the cases the left coronary artery presented a trifurcation into anterior interventricular, circunflex and left marginal branches (35.70%) and into anterior interventricular, circunflex and lateral branches (64.30%). In 60% of the hearts examined, the left coronary artery presented a bifurcation into anterior interventricular and circunflex branches. In 1.82% of the cases these two branches arise directly from the aorta. An anastomosis, at the subepicardial level, between the anterior and posterior interventricular branches was observed in 56.36% of the hearts. In 88.18% the posterior interventricular branch arised from the right coronary artery, whereas in 11.82% this vessel arises from the circunflex branch. Anastomoses between the right coronary artery and the circunflex branch were found in 10% of the hearts (crux cordis). The dominance of the right coronary artery was present in 69.09% of the cases, of the left coronary artery in 11.82% and in 19.09% of the hearts had balanced distribution. CONCLUSION: The coronary arteries and their main branches present a great quantity of variations with regard to source, trajectory and anastomoses. This knowledge is important for the interpretation of coronary angiography and surgical myocardial revascularization.     

Transposition of the great arteries and isolated origin of the sinus node artery

We report the cases of 2 newborns who underwent at 7 days of age an arterial switch operation for transposition of the great arteries with a rare coronary anomaly: the left and right coronary arteries originated with a single ostium from sinus 1 and the sinus node artery had an isolated origin from sinus 2. The sinus node artery was reimplanted into the new aorta in both patients. Both babies were discharged in sinus rhythm. Preserving the vascularization of the sinus node may avoid the occurrence of postoperative atrial rhythm disturbances.     

Sudden unexpected death resulting from an anomalous hypoplastic left coronary artery

We present a case of sudden death in a 24-year-old, healthy white female who was physically active and participated in sports, including soccer. Two weeks prior to her death, an insurance physical examination revealed an abnormal electrocardiogram which demonstrated flipped T waves in the anterior leads. There was no other remarkable medical history. At autopsy, only one coronary ostium was demonstrated and it originated from the right aortic sinus. Approximately 0.8 cm from this right coronary artery (RCA) ostium, a left coronary artery (LCA) branched off the RCA at a 90-degree angle. The LCA had luminal diameter of 0.4 m but the LCA had a luminal diameter of only 0.1 cm. The LCA coursed anterior to the base of the pulmonary artery and down the anterior ventricular septum reflecting the usual course of the left anterior descending (LAD) coronary artery. The LCA and RCA paths appeared to merge or terminate at the anterior left ventricular myocardium which was discolored gray, a process that involved the inner and middle thirds of the myocardium. Based on the autopsy findings, we certified the cause of death as a probable arrhythmia due to myocardial fibrosis and dystrophic calcification resulting from complications of an anomalous hypoplastic left coronary artery. Anomalies of coronary arteries have been classified and studied at autopsy and by clinical angiography. Coronary artery anomalies can be divided into minor and major forms with major anomalies often resulting in cardiac dysfunction that may cause failure and death. Minor anomalies, in general, have no pathophysiological significance and are compatible with life. Minor anomalies include variations in number and location of coronary ostia. A single coronary ostia is exceedingly rare in hearts with no other congenital malformations. The prognostic significance can be unpredictable. A single coronary artery has the potential to be dangerous if obstructed at its main stem, or if it branches at an acute angle. Additionally, hypoplasia of one or more coronary arteries has been found to be associated with sudden death.     

Normal single coronary artery and myocardial infarction

A single left coronary artery was found in an asymptomatic 21-year-old man who initially had electrocardiographic and vectorcardiographic evidence of anterolateral myocardial infarction. The single left coronary artery, which supplied the distribution of both the left and right coronary arteries, was free of disease at catheterization. There has been no previous association of a normal single left coronary artery and anterior myocardial infarction. Patients with the finding of a single coronary artery should be watched closely, as this may represent a potentially fatal condition.     

Working Group Report 2: In situ caries models, saliva, microbiology, and statistical considerations

We present a case of a single coronary artery where the right coronary artery (RCA) arose from its proximal part. This rare anomaly was detected during elective coronary angiography in a patient with suspected coronary artery disease. The single coronary artery originated from the left sinus of valsalva, giving rise to RCA proximally and distally dividing into left anterior descending (LAD), ramus intermedius and left circumflex (LCX) arteries. The anginal symptoms in this patient was attributed to a significant stenosis at the proximal LAD which was subsequently dilated by coronary angioplasty. To the best of our knowledge, this is the first reported case of angioplasty of LAD in an anomalous single coronary artery.     

Pelvic floor exercises versus vaginal weight cones in genuine stress incontinence

BACKGROUND: Anomalous origin of coronary arteries (AOCA) is a rare congenital disease. Although it may have a benign course, it has been identified as a frequent cause of sports-related sudden death. Unfortunately, in vivo detection of AOCA is not easy, as individuals with this anomaly often are asymptomatic and show no signs of myocardial ischemia. Presently, transthoracic two-dimensional echocardiography (TTE) is the only noninvasive, widely available tool to visualize the ostia and first tracts of coronary arteries. OBJECTIVE: To assess the efficacy of TTE in the screening of AOCA in a large athletic population. STUDY DESIGN: In a prospective study, we assessed the ostia and first tracts of coronary arteries in 3,650 subjects (mean age, 30+/-12 years) practicing different sports at various competitive levels. Subjects underwent a TTE examination in our laboratory for scientific or diagnostic purposes. RESULTS: Technically satisfactory echocardiograms were obtained in 3,504 subjects (96%); a clear visualization of the ostia and first tracts of both coronary arteries was obtained in 3,150 cases (90%). Three asymptomatic athletes (0.09%) were suspected to have an AOCA; two with a right coronary artery origin from the left sinus, and one with a left coronary artery origin from the right sinus. Diagnosis was confirmed by coronary angiography. CONCLUSIONS: Our study indicated that AOCA is rare in asymptomatic athletes. Systematic and accurate exploration of coronary anatomy in athletes referred for a diagnostic TTE examination may be useful in identifying those with AOCA.     

Transatrial-transpulmonary tetralogy of Fallot repair is effective in the presence of anomalous coronary arteries

OBJECTIVES: The study's object was to analyze the outcomes of transatrial-transpulmonary repair in children with tetralogy of Fallot and anomalous coronary artery crossing the right ventricular outflow tract. METHODS: The transatrial-transpulmonary approach was used in 611 consecutive repairs, 36 (5.9%) of which were associated with a surgically relevant coronary artery anomaly. The median age and weight of the patients at repair were 23 months (2.8-170 months) and 9.9 kg (5.2-41 kg), respectively. Anomalies included left anterior descending coronary artery from right coronary artery or single right coronary artery (n = 22), right coronary artery from left coronary artery or left anterior descending coronary artery (n = 8), and large right coronary artery conal branch (n = 6). Diagnosis was established before the operation in 25 of 36 cases by angiography (n = 24) or echocardiography (n = 1). The approach was successful in 34 cases, in 25 of which placement of a limited transannular patch was necessary. Two patients had a right ventricle-pulmonary artery conduit as a result of proximity of the coronary branch to the pulmonary arterial anulus and inability to adequately relieve the right ventricular outflow tract obstruction. RESULTS: There have been no early or late deaths. Mean right ventricle-pulmonary artery gradient at last follow-up was 19 mm Hg (95% confidence interval 14.5-24 mm Hg), compared with 15 mm Hg (95% confidence interval 12.5-17.5 mm Hg) for patients with normal coronary arteries (P = .3). Actuarial freedom from reoperation at 120 months was 96.5% (95% confidence interval 79.8%-99.5%) and was also similar between patients with and without coronary artery abnormalities (P = .92). CONCLUSIONS: Surgically important coronary anomalies in tetralogy of Fallot can be dealt with through the transatrial-transpulmonary approach in most cases without major alterations in technique. Outcomes are similar to those of other patients with tetralogy of Fallot. The presence of anomalous coronary arteries does not impart incremental risk after this surgical strategy.     

Complete occlusion of all three main coronary arteries, with survival dependent on two septal arteries

A 68-year-old Asian gentleman presented with limiting angina pectoris following myocardial infarction. Coronary angiography demonstrated complete occlusion of the left anterior descending artery after the first septal, the proximal right coronary artery and also the proximal part of the circumflex. The myocardial blood supply was wholly dependent on two septal arteries.     

pH sensitive properties of Tc(V)-DMS: analytical and in vitro cellular studies

Coronary arteries anomalies may be part of complex congenital malformations of the heart or be an isolated defect. In our anatomic collection of congenital heart disease, an isolated anomalous origin of coronary arteries was observed in 27 of 1,200 specimens (2.2%): left coronary artery from pulmonary trunk in five, origin from the wrong aortic sinus in 12 (both right and left coronary artery from the right sinus in four and from the left sinus in seven, left coronary artery from the posterior sinus in one), left circumflex branch from right aortic sinus or from very proximal right coronary artery in three, high takeoff of right coronary artery in three, stenosis of the coronary ostia attributable to valvelike ridge in four. In 16 (59%) patients (12 males and 4 females, age ranging from 2 months to 53 years; median, 14), the final outcome was sudden death; it occurred in all cases of left coronary artery origin from right aortic sinus, in 43% of right coronary artery origin from left aortic sinus, and in 40% of the left coronary artery from the pulmonary trunk. Sudden death was precipitated by effort in eight (50%) and was the first manifestation of the disease in eight (50%); previous symptoms consisted of recurrent syncope in four, palpitations in three, and chest pain in one. Five patients who died suddenly during effort were athletes. In conclusion, (1) more than half of our postmortem cases with anomalous origin of coronary arteries died suddenly, (2) all but two patients with sudden death had anomalous coronary artery origin from the aorta itself, (3) the fatal event was frequently precipitated by effort, (4) palpitations, syncope, and ventricular arrhythmias were the only prodromic symptoms and signs. Recognition during life of these coronary anomalies, by the use of noninvasive procedures, is mandatory to prevent the risk of sudden death and to plan surgical correction if clinically indicated.     

Nitrergic innervation and relaxant response of rectal circular smooth muscle

We report a case of an anomalous right coronary artery arising from the morphological left sinus of Valsalva in a patient with Kartagener's syndrome. Literature review has revealed only a small number of cases of anomalous coronary arteries in patients with dextrocardia and none previously reported in Kartagener's syndrome.     

Cardiopathy and ocular abnormalities in Noonan syndrome

When the left anterior descending coronary artery follows an anomalous course between the aorta and pulmonary artery it can cause myocardial ischaemia or sudden death during exercise in young people. Coronary arteriography in a 27 year old man with angina pectoris at rest showed a left anterior descending coronary artery arising from a common right trunk and running from the aorta to the pulmonary artery. Follow up after revascularisation was uneventful.     

Pathogenesis of acute coronary syndromes

Coronary artery diseases may categorized into asymptomatic disease, angina pectoris, myocardial infarction, chronic heart failure, and sudden coronary death. Unstable angina, acute myocardial infarction, and sudden cardiac death are known as the acute coronary syndromes. Coronary atheroma is unstable in the patients with acute coronary syndromes. Stable plaques will be unstable when dynamic alterations occur. The alterations are plaque rupture, plaque hemorrhage, coronary thrombosis and vasospasm. They act each other. We analysed the histopathology of coronary arteries who died of acute myocardial infarction in 85 cases. It showed that the risk factors of plaque rupture are clusters of form cells, eccentric plaque with soft lipid rich core, and thinning of fibrous cap in atheroma. Most of these cases ruptured at edge of the atheroma.     

Right coronary artery cirsoid with fistulous connection to the coronary sinus

A 61-yr-old woman was referred to our hospital for evaluation of a suspected right atrial myxoma. The transesophageal echocardiogram suggested the presence of an anomalous right coronary artery with fistulous connection to the coronary sinus. At cardiac catheterization, an oxygen step-up in the right atrium indicated a 1.3:1.0 left-to-right shunt. Aortic root angiography showed a large and calcified right coronary artery cirsoid draining to the coronary sinus, which appeared remarkably dilated. In this rare anomaly, cardiac catheterization is necessary, not only to quantify the magnitude of the left-to-right shunt, which is an important requirement for the indication to surgical treatment, but also to confirm the echocardiographic diagnosis.     

Coronary atherosclerosis with dual coronary artery fistulas

Coronary artery fistula (CAF) is an uncommon congenital anomaly. Bilateral CAFs, arising from both right and left coronary arteries are rare. Myocardial infarction is by far a less frequent complication of CAF than angina pectoris. Ligation of CAF and coronary artery bypass graft were performed in the treatment of coronary artery stenosis with CAF.     

Anomalous origin of the right coronary artery from the left anterior descending coronary artery

A 77-year-old male presented with a recent posterior myocardial infarction for coronary angiography. This angiogram revealed a rare, previously unreported anomalous origin of the right coronary artery from the proximal left anterior descending coronary artery distal to the first major diagonal branch.     

High-grade coronary stenosis due to a bronchogenic cyst

A 32-year-old woman without preceding illness or significant cardiovascular risk factors was admitted to hospital because of a syncope after physical exertion. Transoesophageal echocardiography, performed to exclude aortic dissection, showed as an incidental finding a cystic space-occupying mass, 5 x 6 cm in diameter, dorsal to the aortic root and main pulmonary artery. Exercise ECG had S-T segment depressions of about 0.3 mV, associated with anginal symptoms, so that inadequate coronary artery flow on exertion was suspected. Subsequent coronary angiography demonstrated a funnel-shaped 80% stenosis at the origin of the main left coronary artery, while all other coronary arteries were normal and smooth-walled. A causal connection between the two findings was assumed and quickly undertaken surgical intervention revealed an intrapericardial bronchogenic cyst which, presumably as a result of its size and location, had pressed on the main stem of the left coronary artery and caused the stenosis of its ostium. The cyst was completely resected and angioplasty of the coronary artery ostium was performed to ensure its patency. 3 months postoperatively the patient underwent an exercise test of up to 125 Watt without signs of ischaemia. This case presents a previously unreported complication of a bronchogenic cyst.     

Three-dimensional respiratory-gated MR angiography of coronary arteries: comparison with conventional coronary angiography

OBJECTIVE: MR coronary angiography is most often performed using two-dimensional techniques. Although three-dimensional (3D) acquisitions do have important advantages, they take too long for a single breath-hold and are thus susceptible to respiratory motion artifacts. The purpose of this study was to investigate the accuracy of a unique respiratory-gated 3D MR angiographic technique in identifying the proximal coronary arteries in patients suspected of having coronary artery disease. In addition, we investigated the capability of this technique to detect proximal stenoses. SUBJECTS AND METHODS: We performed a prospective blinded study in 20 patients who were referred for conventional coronary angiography. A cardiac-gated 3D gradient-echo sequence with fat suppression was used. Retrospective respiratory gating was performed using navigator echoes of the diaphragm position. Using multiplanar reformatting, two independent readers blindly analyzed the data sets for visualization of major coronary arteries, lengths of imaged segments, and detection of significant stenoses (> 50% occlusion of the luminal diameter by conventional angiography). RESULTS: Seventy-seven of 80 (96%) coronary arteries were positively identified. In one patient, an anomalous coronary anatomy was readily identified and confirmed by conventional angiography. The average lengths of the imaged segments of the right, left main, left anterior descending, and left circumflex coronary arteries were 58 +/- 13 mm, 9 +/- 5 mm, 59 +/- 16 mm, and 24 +/- 10 mm, respectively. Overall sensitivity for the detection of stenoses was low (38%), with a specificity of 95%. Interobserver agreement was 0.92, with a kappa value of 0.65. CONCLUSION: Respiratory-gated 3D MR angiography allows accurate identification of proximal coronary arteries and may be valuable for 3D imaging of coronary anomalies. Further technical improvements are required to enhance the value of the technique in detecting stenoses.