Adult congenital anomalies of the coronary arteries described over 31 years of angiographic studies in the Asturias Principality: main angiographic and clinical characteristics

OBJECTIVE: The aim of this study was to determine the incidence of adult congenital anomalies of the coronary arteries over 31 years of angiographic studies, describing their angiographic and clinical characteristics. The results have been compared with the main series published. METHODS: The diagnostic angiographic reports done in the Principado de Asturias from 1968 to 1999 are reviewed. In those in which a congenital anomaly was diagnosed, the clinical report and the angiography were studied. The initial course of the anomaly was defined following angiographic criteria. RESULTS: Thirteen thousand five hundred reports were reviewed describing 75 patients with 75 anomalies (0.5%) including: anomalous origin of the left circumflex coronary artery (n = 24), coronary artery fistulae (n = 21), both coronary arteries arising from the left coronary sinus (n = 15), single coronary arteries (n = 6), both coronary arteries arising from the right coronary sinus (n = 2), separated origin of anterior descending and left circumflex coronary arteries (n = 3), anterior descending artery arising from the right coronary sinus (n = 2), and others (n = 1). Angiographic studies were done because of: angina (59%), dysnea (25%), atypical chest pain (7%), syncope (3%), dizziness (3%) and palpitations (3%). The initial course was retroaortic in all the circumflex arteries, interarterial in the right coronaries, anterior in the anterior descending arteries and retroaortic, septal and combined, in the left coronaries. CONCLUSIONS: Adult congenital anomalies of the coronary arteries are not very common and are usually casual findings of diagnostic angiographic studies. Left circumflex coronary artery anomalies are the most frequently diagnosed.     

Coronary anomalies: Incidence and importance

Most patients with coronary anomalies are asymptomatic. The knowledge of those variations could be important in regard to invasive catheter treatment or bypass surgery. In a retrospective study, the angiographic findings based on 4, 016 patients (1985-1989) were analyzed concerning coronary anomalies and malformations. Of the patients studied, 39 (0.97%) had coronary anomalies, and in 26 of these patients it was an anomalous circumflex branch. In 14 cases, the circumflex branch arose from a separate origin in the left aortic sinus. In 11 patients the origin was from the proximal segment of the right coronary artery. A singular coronary artery was found in five patients, originating from the right aortic sinus in two patients and from the left aortic sinus in three patients. An origin of the left coronary artery from the pulmonary artery, a coronary fistula, or an origin of the left anterior descending coronary artery from the RCA could be found in only one patient. Unexpected findings during invasive procedures would suggest a possibly existing coronary anomaly, especially when main branches cannot be opacified by selective contrast medium injection.     

经皮冠状动脉腔内成形术后再狭窄与介入前病变形态学的关系

目的：探讨国人冠心病经皮冠状动脉腔内成形术（ＰＴＣＡ）后再狭窄与冠状动脉（冠脉）病变类型、部位和病变血管数的关系。　　方法：冠心病介入治疗３５２ 例,选择其中６５ 例（８６个病变）术后３～１６个月造影随访者,按随访结果分为再狭窄组（ｎ＝ ３８）,非再狭窄组（ｎ＝ ２７）,分析再狭窄与病变形态学的关系。　　结果：再狭窄与多个易患因子、多支病变、病变类型和前降支病变呈正相关,与支架置入呈负相关。再狭窄组Ｃ型、钙化病变多见,前降支病变内径丢失比回旋支、右冠脉病变大（Ｐ＜ ０．０５）,半年内一支血管病变再狭窄率（１０．２７％ ）低于二支（４１．１０％ ）和三支（４８．５０％ ）血管病变再狭窄率（Ｐ＜ ０．０００１）。　　结论：再狭窄与冠脉病变类型、部位和血管支数相关,Ｃ型及钙化、多支和前降支病变再狭窄率高     

The primary anomalies of coronary artery origin and course: A coronary angiographic analysis of 16,573 patients

BACKGROUND/OBJECTIVES:

Coronary artery anomalies are present at birth, but relatively few are symptomatic. The majority are discovered incidentally. In the present study, coronary angiograms performed in the authors’ centre (Ondokuz Mayis University Hospital, Samsun, Turkey) were analyzed to determine the prevalence and types of coronary artery origin and course anomalies.

METHODS:

Coronary angiographic data of 16,573 patients were analyzed. Anomalous origins and courses of coronary arteries were assessed.

RESULTS:

Anomalous coronary arteries were detected in 48 (0.29%) of 16,573 patients. The origin of the circumflex (Cx) artery from the right coronary artery (RCA) or right sinus of Valsalva was the most common anomaly (28 patients [58.3%]). An anomalous RCA originating from the left anterior descending artery (LAD) or Cx artery was observed in six patients (12.5%). The left coronary artery originated from the right sinus of Valsalva in five patients, and the LAD originated from the RCA or the right sinus of Valsalva in five patients. The RCA originated from the left sinus of Valsalva in three patients and from an ectopic ostium in the ascending aorta in one patient.

CONCLUSIONS:

The most frequent anomaly observed in the present study was related to the Cx artery, which is consistent with previous reports. Although coronary artery anomalies are rare, they may cause difficulties during coronary interventions or cardiac surgery and may occasionally result in sudden cardiac death. Therefore, the recognition and diagnosis of these anomalies is important and requires specialization in coronary angiographic techniques and other imaging modalities.     

Coronary artery anomalies:prevalence and clinical profile in elderly patients

Although congenital heart diseases are uncommon in the elderly,coronary artery anomalies may beincidentally discovered in old age.We sought to determine the incidence and clinical features of coronary arteryanomalies (CAAs) in patients over 65 years of age.Patients and methods Medical records of patientsundergoing coronary artery angiography in the years 1997-2002 at the Legnago General Hospital were retrospectivelyreviewed.The clinical profiles of all patients with CAAs and CAA subtypes were noted.Comparison between patientsunder and over 65 was performed.Data are given as mean standard deviation and as percentages.Results Sixty-six patients (1.21%,Female/Male 22/44,mean age 65.3±10.6 years) out of the 5450 who underwent coronaryangingraphy in the years 1997-2002 had CAAs.In most cases (63%,41/66 patients),the patients were over 65.CAAs were discovered incidentally in these elderly patients while undergoing coronary artery angiography for dilatedcardiomyopathy, ischemic heart disease,and valvular heart disease in 75% of the cases (30/41 patients).Patientsover 65 had more cardiac comorbidities and a higher incidence of coronary atherosclerosis.Conclusions Theangingraphic incidence of CAAs in elderly patients is increasing as the population ages and this occurrence calls fora wider knowledge of the anatomy and pathophysiology of CAAs among geriatric cardiologists.Elderly patients seemto present with lower risk coronary anomalies (separated origin of left anterior descending coronary artery andcircumflex artery,origin of circumflex artery from the right sinus or the right coronary artery,double coronary artery)but have a higher risk profile compared to younger patients due to the frequency of cardiac coraorbidities andsuperimposed coronary artery atherosclerosis.(J Geriatr Cardiol 2004;1:40-43.)     

冠状动脉起源异常的冠状动脉造影分析

目的探讨冠状动脉起源异常(anomalous origin of coronary artery,AOCA)的冠状动脉造影特征及其临床意义。方法2000年10月~2006年12月在我院行冠状动脉造影的患者1560例,检出有AOCA患者29例,并对其临床资料进行回顾性分析。结果29例AOCA患者检出率为1.9%,其中23例(79.3%)为良性AOCA,包括:前降支和回旋支分别开口于左冠窦15例,回旋支起源于右冠窦3例,右冠状动脉起源于无冠窦1例和高位开口于升主动脉4例;6例(20.7%)为有潜在临床危险AOCA,分别为左主干起源于右冠窦1例,单支左冠状动脉2例,右冠状动脉起源于左冠窦3例。起源异常冠状动脉有明显狭窄患者9例(31.0%),但仅2例为狭窄只累及起源异常动脉。结论AOCA无特征性临床表现,通常在冠状动脉造影时被发现;某些类型有导致严重心脏事件的潜在危险。AOCA与冠状动脉狭窄无相关性。     

A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, with an incidence of <1 in 300 000 live births. We describe a rare case of a 23‐month‐old infant admitted for cough and fever. Echocardiography showed a branch of the left coronary artery originating from the pulmonary artery. The coronary computed tomographic angiography revealed anomalous origin of the left anterior descending (LAD) artery from the pulmonary artery with the left circumflex artery and right coronary artery arising normally from the aorta. The infant successfully underwent surgical reimplantation of the LAD to the ascending aorta. Our case emphasizes that echocardiography and computed tomographic angiography are valuable imaging modalities for making an accurate diagnosis and determining the precise surgical plan.     

Echocardiographic prediction of the site of coronary artery obstruction in acute myocardial infarction

In 49 patients with acute myocardial infarction (AMI), the infarctiontopography was assessed by cross-sectional echocardiographyand the location of coronary artery obstruction were correlated.A ventricular segmentation of 5 right and 16 left ventricularsegments was used. The site of coronary obstruction was determinedin 45 patients by coronary angiography and by necropsy in 4patients. The exact location of the obstruction could not befound in 4 patients. The infarct related vessel was the leftmain artery in 1 patient, the left anterior descending artery(LAD) in 19, the left circumflex in 6 and the right coronaryartery in 24.Specific segments were identified for each of the3 coronary arteries: anteroseptal and anterior segments forLAD, right ventricular segments for the right coronary arteryand basal anterolateral segment for the left circumflex. Specificsegments (specificity 100%) were also identified for the principalcoronary branches: basal anterior for the first anterior descendingdiagonal (sensitivity 71%), basal anteroseptal for the firstseptal perforator (83%), middle anterior for the second diagonal(100%), middle anteroseptal for the second septal (89%), basalposteroseptal for a dominant right coronary artery (89%), rightventricular anterolateral segment for the right ventriclar marginalbranch (83%) Echocardiographic identification of the topography of AMI canbe useful in recognizing the infarct-related vessel and identifyingthe site of coronary artery obstruction.     

Primary congenital anomalies of the coronary arteries: a coronary: arteriographic study

Geographic variations in the incidence of different congenital coronary anomalies are well known, but infrequently studied in the Indian population. Among 4,100 adult patients who underwent diagnostic coronary arteriography, 39 (0.95%) patients (34 males, 5 females) had one or more anomalous coronary arteries. Their mean age was 46.4 +/- 8.2 years (range, 26-68 years). Thirty-five (89.74%) had anomalies of origin and distribution, while the remaining four (10.25%) had coronary artery fistulae. Right coronary artery was the commonest anomalous vessel, involved in 19 (48.74%) patients. It was originating from the left sinus of Valsalva in 15 and from the non-facing aortic sinus in four patients. Anomalous left circumflex artery was the second commonest anomaly, seen in 14 (35.89%) patients. Anomalous left anterior descending artery and anomalous left coronary artery from pulmonary artery were seen in one patient each. Among patients with coronary fistulae, two had fistulae between the left anterior descending artery and the main pulmonary artery, one between the conal artery and the right atrium, while the fourth patient had fistulae from the right coronary as well as from the left anterior descending artery to the left atrium. Atherosclerotic plaques in the anomalous arteries were seen in only 13 (33.33%) patients, much less than the overall incidence of coronary artery disease in patients with congenital coronary anomalies in this series (66.66%). In four (10.25%) patients, only the anomalous vessels were involved in coronary artery disease. Thus, in a small subgroup there does not appear to be an increased risk for development of atherosclerotic coronary artery disease in anomalous coronary arteries. Recognition of coronary anomalies is important in patients undergoing coronary arteriography, coronary interventions and cardiac surgery. Variations in the frequency of primary congenital coronary anomalies may possibly have a genetic background.     

Coronary artery anomalies: Anomalous origin of the left coronary artery and circumflex branch in two patients

Coronary anomalies may be isolated defects or accompany congenital malformations of the heart. The determination of these anomalies is important in the treatment approach and the surgical procedure in bypass and valve surgery. The present article reports on clinical and angiographic findings in two patients with coronary artery anomalies -one patient with an anomalous origin of the left coronary artery from the right aortic sinus, and another patient with an anomalous origin of the circumflex branch of the left coronary artery from the right coronary artery.     

Congenital coronary anomalies of origin and distribution in adults: a coronary arteriographic study

BACKGROUND: Coronary anomalies should be recognized to avoid problems during coronary intervention and cardiac surgery. METHODS AND RESULTS: We retrospectively reviewed 7400 coronary angiograms to find out the pattern and incidence of coronary anomalies of origin and distribution. We excluded patients with congenital heart diseases, coronary artery fistulae and patients with separate origin of the conus artery. and found 34 cases (0.46%) (22 males), mean age 50.7 +/- 12 years with coronary anomalies. Six cases underwent angiography prior to valve replacement and the rest were part of the evaluation for atherosclerotic coronary artery disease. The most common anomaly was separate origins of the left anterior descending coronary artery and left circumflex coronary artery [n=12 (35.3%)]. The next most common anomalies were origins of the right coronary artery from the left coronary sinus [n=7 (20.6%)] and left circumflex artery from the right sinus [n=6 (20%)]. A single coronary artery was seen in 3 cases (8.8%) which included one case of postmyocardial infarction ventricular septal rupture with triple-vessel disease, and another with two small coronary fistulae. One case each of the following coronary anomalies was found: (i) double right coronary artery, (ii) left anterior descending coronary artery from the right coronary sinus, (iii) all three coronary arteries originating separately from the right sinus, and (iv) left main coronary artery from the right sinus. Of these 34 patients, 11 (32.4%) had significant atherosclerotic disease in the anomalous vessel. CONCLUSIONS: The incidence of primary coronary anomaly seems to be less than that in earlier reports, but the pattern of anomalies appears to be similar.     

Whole-heart coronary magnetic resonance angiography of double left anterior descending artery

Double left anterior descending artery arising from the left and right coronary arteries is an extremely rare congenital coronary anomaly. We describe, for the first time, three-dimensional, whole-heart coronary magnetic resonance angiographic findings of double left anterior descending artery.     

Spontaneous Termination of Ventricular Fibrillation in a Patient with Congenital Coronary Anomaly

Sudden death is common in patients with congenital coronary artery anomalies mainly when the left main coronary artery originates from the right coronary sinus. Ventricular fibrillation in these patients is irreversible unless defibrillation can be rapidly performed. We describe a 57‐year‐old male with an anomalous origin of circumflex and the left anterior descending coronary arteries from the right coronary sinus. He developed two episodes of ventricular fibrillation that terminated spontaneously, 10 hours after percutaneous revascularization of the circumflex coronary artery. Computed tomography angiography, in addition to confirming the anomalous origin of the coronary arteries, showed a muscle bridge over the midportion of the left anterior descending coronary artery. This is the first report of spontaneous termination of ventricular fibrillation in a patient with congenital anomaly of the coronary arteries.     

Single coronary artery from the right aortic sinus of Valsalva with anomalous prepulmonic course of the left coronary artery

Cardiac computed tomography allows for improved, noninvasive and accurate visualization of coronary artery anomalies. The case of a single coronary artery with origin from a single ostium in the right sinus of Valsalva with an anomalous course of the left coronary artery anterior to the pulmonary trunk is presented. The unusual distal reconstitution of a normal anatomical course at the junction of the mid and distal left anterior descending artery with occlusion of the proximal circumflex artery has not, to the authors’ knowledge, been previously described.     

Percutaneous revascularization in a patient with anomalous origin of left main coronary artery

Anomalous origin of the coronary artery from opposite coronary sinus is infrequently observed during coronary angiography. Percutaneous coronary intervention (PCI) of anomalous coronary artery is technically difficult and challenging. It requires appropriate selection of guide catheters for adequate stability, coaxial alignment and backup support during the intervention. We hereby report a rare case of anomalous origin of left main coronary artery (LM) from the right coronary sinus, having a retro-aortic course to the left side before its bifurcation into left anterior descending (LAD) and circumflex artery. The 59-year-old man had successful PCI of atherosclerotic LAD lesions. A 64-slice Multi-Detector Computed Tomography (MDCT) performed at 4 years of follow-up demonstrated patency of coronary stents and also delineated the origin and course of the anomalous LM. The case illustrates the rarity of anomalous LM, and describes technical issues during PCI and the role of MDCT in coronary anomaly imaging.     

Origin of all three major coronary arteries from the right sinus of Valsalva: clinical, angiographic, and magnetic resonance imaging findings and incidence in a select referral population.

OBJECTIVE: We sought to determine the incidence and imaging features by coronary angiography and cardiac magnetic resonance imaging (MRI) of anomalies in which the right, circumflex, and left anterior descending coronary arteries arise separately from the right sinus of Valsalva. BACKGROUND: The anomalous origin of all major coronary arteries from separate ostia in the right sinus of Valsalva has been reported as exceedingly rare, with mainly isolated cases reported. A knowledge of the origin and proximal courses of aberrant arteries is critical for patient management. METHODS: 42 consecutive patients without other congenital heart disease referred to our institution for MRI evaluation of anomalous coronary artery over a six year period were evaluated. Analysis of angiograms and MRI was done to determine the anatomic origin and proximal pathway of coronary arteries (determined by conventional angiography and MRI) and degree of any stenosis (by angiography). RESULTS: Seven of the 42 patients (17%) in this referral population had the described anatomy. Both conventional angiography and MRI depicted the origin and proximal courses of these arteries. In all patients, the circumflex passed behind the aorta. In three, the left anterior descending passed through the ventricular septum; in four, it passed anterior to the pulmonary trunk. CONCLUSIONS: This series is the largest ever reported on this complex anatomical variant and the first to give a systematic analysis of the anatomy by angiography and MRI. This constellation of multiple anomalous coronary arterial origins and proximal courses may not be as rare as previously reported.     

The distribution and severity of coronary artery disease and left ventricular dysfunction among patients with single coronary artery disease and angina pectoris

The distribution and severity of coronary artery disease and left ventricular dysfunction were evaluated in 103 patients with single coronary artery disease and angina pectoris. The coronary arterial patterns were separated into right (76), mixed (18), and left (9) systems depending upon the blood supply to the inferior surface of the left ventricle. The following relationships were noted: (1) In patients with single coronary artery disease and angina pectoris, coronary arterial lesions are similar among right, mixed, and left systems. (2) In order of frequency, single coronary artery disease occurred most commonly in the left anterior descending coronary artery (55), next most commonly in the right coronary artery (36), and least commonly in the circumflex/obtuse marginal coronary artery (12). (3) Left ventricular wall motion abnormalities were seen in descending order of frequency: patients with right coronary artery (50%), left anterior descending coronary artery (36%), and circumflex/obtuse marginal coronary artery (25%) disease.     

Congenital anomalies of coronary artery origin: a diagnostic challenge

From a series of 4,313 consecutive patients who underwent a diagnostic coronary angiogram, 16 (0.37%) presented a congenital anomalous origin of the coronary arteries. None of these patients had other congenital cardiac anomalies associated. Age was 57 +/- 9 years and 13 (81%) were male. The diagnostic catheterization was performed for unstable angina in 8 patients (50%), for stable angina in five (32%), for dyspnea in two and for atypical chest pain in the remaining patient. A previous myocardial infarction was present in 6 patients (37%) whereas one patient had apical hypertrophic cardiomyopathy. We observed absence of coronary lesions in 4 patients and severe coronary stenosis lesions in 12 patients (75%), five of those with lesions located in the anomalous vessel. The most frequent abnormality found was an anomalous origin of left circumflex coronary artery in 8 cases (50%), followed by an abnormal origin of the right coronary artery in 5 cases (31%), and an abnormal origin of the left coronary tree in 3 cases (19%) (left anterior descending coronary artery arising from the right coronary artery, a single coronary artery which originated in the left coronary sinus, and a left main coronary artery which originated in the noncoronary sinus). The relationship of the anomalous coronary artery to the great vessels was the following: A retro-aortic course in 11 patient (69%), by the anterior free wall in two (12.5%), interarterial in two (12.5%), and septal in one (6%). Finally, as an index of the difficulty to visualize the anomalous coronary artery, an unusual catheter was needed in six (37%) of the diagnostic procedures to reach the target vessel.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anomalous origin of the circumflex coronary artery--two case reports.

As shown in many series, congenital coronary artery anomalies are found in 0.6 to 1.5% of patients undergoing coronary angiography. Various types of coronary anomalies have been described, many involving the circumflex artery. The second most common anomaly is of the circumflex arising from the right sinus of Valsalva, while origin in the right coronary artery is also frequent. The most common anomaly is absence of the left main coronary artery, the anterior descending and circumflex arteries originating separately in the left coronary sinus. Such anomalies are usually benign, although earlier and more aggressive atherosclerosis is more likely than in normal coronaries and myocardial ischemia can result. Although rare, this can manifest as sudden death. Conventional coronary angiography may be unable to determine the three-dimensional course of the anomalous vessel. The development of multislice computed tomography and its application to cardiac imaging mean that it is now possible to visualize the coronary arteries non-invasively and to obtain more accurate information on their proximal course. We present two cases of congenital anomaly of the circumflex coronary artery diagnosed with the aid of multislice computed tomography.     

Single coronary artery with anomalous origin of the right coronary artery as a branch from the left anterior descending artery: a very rare coronary anomaly

Anomalous origin of the right coronary artery arising from the left anterior descending artery (LAD) is a very rare coronary anomaly. It has previously been reported in only six adult cases. In this report, we present a patient with an anomalous origin of the right coronary artery from the LAD. The patient had anginal symptoms with exercise. Myocardial perfusion imaging with thallium-201 revealed a reversible inferior perfusion defect. We suggest that this could cause myocardial ischemia. Received: November 5, 2001 / Accepted: December 7, 2001