Abnormal Origin Of Coronary Artery Research Articles

462 Accidental detection of an anomalous origin of the coronary arteries in a patient with STEMI: a case report

Abstract Coronary artery anomalies and variants are relatively uncommon congenital disorders of the coronary artery anatomy and constitute the second most common cause of sudden cardiac death in young competitive athletes. Normally there are two main coronary arteries, which stem from the sinuses of Valsalva and descend towards the cardiac apex. The most frequent anomalous origin of the coronary arteries associated with sudden cardiac death is the anomalous origin of a coronary artery from the contralateral sinus, particularly if the anomalous coronary artery has a course between the aorta and the pulmonary artery. The diagnosis of a coronary artery anomaly is insidious since patients are usually asymptomatic. Indeed, in most of the cases, coronary anomalies are discovered incidentally during coronary angiography or on autopsy following sudden cardiac death. However, in some cases, symptoms like angina, syncope, heart failure, and myocardial infarction may occur. We want to describe the case of a 54 years old man who went to another hospital of our district with chest pain. The EKG showed an infero-lateral STEMI. The patient underwent systemic thrombolysis complicated by arrhythmic storm, treated with effective DC shock, then he was urgently transferred to our Hospital to perform a PCI rescue. The coronary angiography showed a critical stenosis of the right coronary artery, treated with the implantation of a zotarolimus-eluting coronary stent system. Surprisingly, an abnormal origin of the anterior interventricular artery and circumflex artery from seperated hosts in the right coronary sinus was detected. A computed tomography showed the separated origin of the right coronary artery and the left main from the right coronary sinus; moreover, the left main presented a retroaortic course between the bulb and the left atrium; the left anterior intraventricular artery run anterior to the aortic root. During hospitalization, a diagnosis of diabetes was made with consequent setting of adequate hypoglycemic therapy. Serial echocardiograms showed an improval in the ejection fraction (from 35–40% to 50%). The patient was discharged in well clinical conditions and 1 month later at the follow-up, during an outpatient visit, he was fully asymptomatic. The dynamic EKG according to Holter showed rare isolated ventricular beats; laboratory tests were normal. The anomalous origin of a coronary ostium from the contralateral sinus is the most frequently anomaly which is associated with sudden cardiac death, in particular when the left coronary artery origins from the right sinus. The rapid advancement of the imaging techniques, including CT, CMR, intravascular ultrasound, and optical coherence tomography, have provided us with a wealth of new information on this subject. Coronary artery CT offers the best performance in terms of spatial resolution, acquisition time, and image contrast but the use is limited due the dose of ionizing radiation and the use of contrast agents, in particular taking into account that most patients are young. CMR is capable of visualizing the origin of the coronary arteries non-invasively, without use of ionizing radiation and contrast agents. Currently however, due to spatial resolution, the capability of CMR to visualize smaller coronary branches is still a limiting factor preventing full assessment of coronary arteries using this modality. Developments and more widespread access to advanced cardiac imaging will undoubtedly lead to earlier diagnoses. The taxonomies of the anomalous origin of coronary arteries are inconsistent and complex and so we want to make our contribution to the register of cases of abnormal origin of coronary arteries for the risk of sudden cardiac death and ischaemic events related to.

Corrected Transposition of the Great Arteries with Ebstein’s Anomaly, Dysplasia of the Mitral Leaflets and Persistence of Left Superior Vena Cava in an Adult

Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult population, especially if it is associated with other abnormalities such as Ebstein’s anomaly and abnormal origin of coronary arteries. Case presentation: Twenty-two-year-old woman admitted to intensive care unit with acute decompensated heart failure. The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein’s anomaly. The computed tomography corroborated these findings and additionally showed a left superior vena cava and an abnormal origin of the coronary arteries. The patient is in NYHA functional class II, receiving treatment with diuretic, digitalis, beta-blockers and angiotensin-converting enzyme (ACE) inhibitor. Conclusion: This is a very interesting case of an adult in the third decade of life with cc-TGA associated with abnormalities not described in the literature that had reached adulthood. Noninvasive images play an important role in its diagnosis.

Mid-term outcomes after the surgical correction of anomalous connection of the coronary artery to the pulmonary artery in infants

Objective Abnormal origin of coronary artery from the pulmonary artery (ACAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. Immediate surgical correction can provide excellent results. This study aimed to determine the surgical outcome of ACAPA. Methods From 1993 to 2016, 31 consecutive patients underwent coronary re-implantation. Concomitant mitral valvuloplasty was performed in two. The study cohort was divided into two groups according to age (group 1: ≤ 6 months and group 2: > 6 months). Data from demographic characteristics, electrocardiography, echocardiography, surgery, intensive care unit stay and follow-up were evaluated. Results The study included 29 patients with anomalous left coronary artery from the pulmonary artery and 2 with anomalous right coronary artery from the pulmonary artery. The median age at repair was 4.75 [2.3–16.3] months and the median weight was 6.2 [4.3–9.4] kg. Preoperative echocardiography showed severe LV dysfunction in 56.7% of patients. The median preoperative LVEF was 33.5% [20–55]. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Requirement to extracorporeal membrane oxygenation (ECMO) support systems was necessary in 2 cases (6.4%) before surgery and 5 cases (16.1%) after correction. Postoperative mortality rate was 9.7%. Younger age ( Conclusions Young age is significantly associated with a more severe presentation, more ECMO requirement and mortality. Lesser development of coronary collateral circulation can explain these findings. After establishment of a two-coronary circulation both ventricular function and MR tend to be normalized over time. This ‘recovery’ usually occurs within 6 months but may require more time (maximum of thirty months in our population).

Exercise Echocardiography in Children

Exercise echocardiography has been widely used in adult cardiology, and is mainly used for assessing regional myocardial function in patients with known or suspected coronary artery disease. In addition, it is also useful in the evaluation of the haemodynamic significance of valve disease, such as aortic stenosis and mitral regurgitation. In children, exercise echocardiography has found its place in the detection of ischaemia in patients with coronary artery abnormalities such as Kawasaki disease, and abnormal origin of coronary arteries. Other indications include assessment of haemodynamic and myocardial response in patients with different types of congenital heart disease, the early detection of myocardial dysfunction in specific populations such as patients after chemotherapy exposure. The incorporation of tissue Doppler and strain imaging with exercise echocardiography has allowed a more quantitative analysis of regional and global systolic and diastolic function.

964Experience in the management of abnormal origin of coronary artery from the opposite sinus at childhood age

964Experience in the management of abnormal origin of coronary artery from the opposite sinus at childhood age

A cadaveric study on the anatomical variations of origin of the coronary arteries in Gujarat state

Background: A thorough knowledge of the anatomy of coronary arteries, the crown of the heart does a self-evident prerequisite for more intelligent planning of surgery or a complete understand of the coronary artery disease. Abnormal origin of coronary arteries may cause potentially dangerous symptoms and even sudden death during strenuous activity. A cadaveric study in an unsuspected population provides a basis for acknowledgment of normal variants, which may facilitate determination of prevalence and evaluation of the value of screening for such anomalies. Objectives: This study was conducted with an aim to describe the anatomical variation in the origin of right and left coronary arteries (RCA and LCA). Materials and Methods: This study based on the examination of 100 cadaveric hearts from the anatomy dissection room and obtained at an autopsy department. By using conventional dissecting techniques, heart specimens were dissected and studied for number of ostia, and their position within respective sinuses. The heights of the ostia from the bottom of sinuses were measured with the help of Vernier caliper. Results: There was no opening found in pulmonary artery and non-coronary sinus coronary arteries. Single, double and triple ostia were present in 78%, 20%, 2% of cases, respectively. The height of coronary ostia from the bottom of the respective aortic cusp which was measured by digital Vernier caliper depicts that RCA ostium was higher than that of the LCA ostium. Conclusion: The findings in this study may provide a basis for understanding the normal variants, for determining the incidence of anomalies, and for evaluating the value of screening for such anomalies.

Stress echocardiography in paediatric cardiology.

Stress echocardiography is a well-established technique in adult cardiology and is mainly used for assessing regional myocardial function in patients with known or suspected coronary artery disease (CAD). Apart from detecting ischaemia, stress echocardiography has found its place in the assessment of the haemodynamic significance of valve disease, particularly in selected patients with aortic stenosis and mitral regurgitation. Also in the paediatric population, stress imaging is most commonly used for the detection of ischaemia in patients with CADs such as post heart transplantation, Kawasaki Disease, and abnormal origin of coronary arteries. Other paediatric indications include the haemodynamic and myocardial response in patients with different types of congenital heart disease, the early detection of myocardial dysfunction in specific populations such as patients after anthracycline exposure, and the evaluation of pulmonary artery pressures and the right ventricular functional response. Techniques have evolved over time and in different paediatric echocardiographic laboratories, exercise stress echocardiography is replacing dobutamine stress echocardiography in older children. Moreover, integrating tissue Doppler and strain technology with stress imaging allows a more quantitative analysis of regional and global systolic and diastolic function. Current clinical applications mainly include patients after transplant, suspected CAD, and hypertrophic cardiomyopathy.

Hypertrophic obstructive cardiomyopathy and anomalous origin of the right coronary artery. A rare coexistence

We present the case of a 65 year-old patient with a history of hypertension and hypertrophic obstructive cardiomyopathy (HOCM) in whom anomalous origin of the right coronary artery (RCA) was diagnosed. Echocardiography measured left ventricular outflow tract (LVOT) gradient ranged between 80 and 123 mm Hg. Systolic anterior motion (SAM) of the mitral valve was visualised. The angiography showed favorable coronary anatomy for alcohol septal ablation (Fig. 1). Additionally, anomalous RCA origin from the left coronary sinus (CS) in sub-selective contrast injection was diagnosed. Cardiac magnetic resonance (CMR) showed asymmetrical left ventricular hypertrophy up to 22 mm of the base anterior segment, base and mid segments of the interventricular septum and inferior segment. Stress CMR revealed reversible, dipyridamole induced ischaemia in the base segment of the lateral wall, apex segments of the inferior and septal wall, and within papillary muscles. Computed tomography (CT) scan revealed RCA ostium angulation and moderate compression at rest of the proximal RCA segment (Fig. 2). Corrective surgery for HOCM was performed. Based on intraoperative assessment of the anomalous RCA course, no surgical correction was done. The anatomical course of a coronary artery that passes between the aorta and pulmonary artery in a young person is a major risk factor for adverse events, even without symptoms. The co-existence of HOCM with marked LVOT obstruction at rest may potentially increase the sudden cardiac death risk. Recently Moza et al. (Am J Med Sci, 2011; 342: 341–342) reported a similar case, but in a much younger patient, who presented with a two-week history of exertional chest pain. Additionally, the combination of HOCM with left coronary artery originating from the right CS was described. Invasive treatment for asymptomatic patients remains controversial, with growing advocacy for patients with ‘left from right’ as opposed to ‘right from left’ origins. CMR provides relevant information on the extent and functional relevance of ischaemia in HOCM patients, mainly attributed to microvascular dysfunction. The value of CT in assessing anomalous coronary arteries has been established. Miller et al. (Int J Cadiovasc Imag, 2012; 28: 1525–1532) presented a series of 15 patients with abnormal origin of coronary arteries, who were examined by CT. This modality proved to be very accurate in showing the anatomical details, confirmed later during corrective surgery. Accurate depiction of the RCA course can be helpful in planning the operative strategy. Interventional treatment of the LVOT obstruction and SAM is discussed elsewhere. Surgical correction of anomalous course of coronary artery is recommended for all adult patients who are symptomatic or who have clear evidence of exercise-induced myocardial ischaemia and the mid-term results are encouraging.

Focus on Echocardiographic and Doppler Analysis of Coronary Artery Abnormal Origin from the Pulmonary Trunk with Mild Myocardial Dysfunction

Late presentation of abnormal origin of coronary artery from the pulmonary artery (ACAPA) is uncommon compared with early presentation, which usually induces extended myocardial necrosis and severe heart failure. The late presentation is characterized by abundant development of intercoronary collaterals resulting in mild and rare symptoms, but nevertheless can cause sudden cardiac death. Our objective was to describe presentation, cardiovascular imaging methods for diagnosis and outcomes of patients with late presentation of ACAP. The study is a retrospective review of a single-center database to identify all patients diagnosed with ACAPA beyond the first year of life. From 1976 to 2011, 10 patients were identified with ACAPA at the age of 1.1-64 years: 6 with left coronary artery from the pulmonary artery (ALCAPA) and 4 with right coronary artery from the pulmonary artery (ARCAPA). Echocardiography and Doppler imaging evidenced: (1) direct signs: the abnormal coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow and (2) indirect signs: abundant intercoronary septal collaterals with anterograde flow (ARCAPA) or retrograde flow (ALCAPA) and dilatation of the controlateral normally originated coronary artery. Nine patients underwent surgical implantation of the ACAPA into the ascending aorta. After 7.9 years mean follow-up, all were asymptomatic except one who required a second surgery. Noninvasive cardiovascular imaging, namely transthoracic echocardiography and Doppler specific parameters, can reach diagnosis of late presentation of ACAPA. Direct aortic implantation is a reliable and effective to establish dual coronary artery circulation and prevent risks due to myocardial ischemia.

285: Adult-type anomalous origin of coronary artery from pulmonary artery: Echocardiographic and Doppler diagnosis and outcomes

Adult-type abnormal origin of coronary artery from pulmonary artery (ACAPA) is uncommon compared to infant-form which usually induces extended myocardial necrosis and severe heart failure. The adult-type form results in mild and rare symptoms, but it can cause myocardial ischemia and sudden cardiac death at childhood and adult age. The objective of this study was to describe the mode of presentation, cardiovascular imaging methods for diagnosis and outcomes of patients diagnosed with adult-type ACAPA. The Patient Congenital Heart Disease database was reviewed to identify all patients diagnosed with ACAPA and/or who had surgical repair beyond the first year of life. From March 1976 to July 2011, 13 patients were identified with adult-type ACAPA, at the age of 6 months to 64 years: 9 with left coronary artery from pulmonary artery (ALCAPA) and 4 with right coronary artery from pulmonary artery (ARCAPA). Cardiac symptoms initiated investigation in 9 patients and a murmur in 4. Nine were diagnosed by transthoracic echocardiography, 4 by coronary artery angiography and 1 by cardiac computed tomography. Myocardial ischemia was detected preoperatively in 6 patients with ALCAPA (66.7%) and 1 with ARCAPA (25%). Ten patients underwent surgical implantation of the ACAPA into the ascending aorta. All were asymptomatic after repair except one who required a second surgery at 7.9 years follow-up. Adult-type ACAPA can be assessed by non invasive cardiovascular imaging. Direct aortic implantation is a reliable and effective to establish dual coronary artery circulation and prevent risks due to myocardial ischemia.

Hypoplastic left heart syndrome with an anomalous origin of the left coronary artery

Abnormal origin of the coronary artery in children with hypoplastic left heart syndrome is an extremely rare defect. We describe a newborn with concomitant hypoplastic left heart syndrome and abnormal origin of the left coronary artery arising from the right pulmonary artery. A Norwood procedure and direct reimplantation of the left coronary artery to the ascending aorta was performed.

Angiographic Diagnosis of an Anomalous Right Coronary Artery Arising from the Pulmonary Artery

Abnormal origin of the coronary arteries from the pulmonary artery can occur in four different ways. Either the left or the right coronary artery, both coronary arteries, or an accessory coronary artery may arise from the pulmonary artery. The most frequent of these malformations is the abnormal origin of the left coronary artery. Many publications have dealt with the clinical, physiological, and anatomical picture of this malformation (7). The majority of patients are symptomatic in early life because of marked anoxia of the anterolateral and apical portions of the left ventricle, the result of insufficient intercoronary anastomoses. Some patients, however, may become symptomatic later in life, and in these abundant anastomoses are found between both coronary arteries. From catheterization and angiographic investigations, there is conclusive evidence that the blood flow is directed from the right coronary artery through intercoronary anastomoses into the distal branches of the left coronary artery and from there in retrograde fashion into the pulmonary artery (1, 7, 13, 16). The other three forms of abnormal origin of the coronary artery from the pulmonary artery are extremely rare. An accessory coronary artery arising from the pulmonary artery has been described in only a few cases and represents an incidental finding (8). Origin of both coronary arteries from the pulmonary artery is not compatible with life, and infants with such a malformation die within the first two weeks after birth (19). The origin of the right coronary artery from the pulmonary artery has been described by several authors (2–4, 9, 14, 15, 17) . No publication of such a malformation diagnosed clinically could be found, and the following observation is reported. A Negro girl of six and a half years was admitted to The University of Chicago, with moderate cyanosis and clubbing of fingers and toes apparent on physical examination. A mild right ventricular tap was palpable and barely visible. A grade 4/6 harsh systolic murmur, starting after S1 and ending distinctly before S1, was heard best along the lower left sternal border, radiating faintly to the apex and the left back, but was well audible along the upper left sternal border. A grade 2/6 soft diastolic murmur (early mid-diastolic but not proto-diastolic) was present at the apex, as well as at the anterior axillary line in the fifth intercostal space. In conjunction with the systolic murmur, this one had a distinct to-andfro character, the diastolic component being louder than the (? transmitted) systolic component. No other murmurs were discovered. The remainder of the physical examination was within normal limits. Laboratory Data With the exception of a hematocrit of 48 per cent and a hemoglobin of 14.7 g per 100 cc, this patient's blood count, blood electrolytes, blood-urea-nitrogen, clotting and bleeding time, as well as findings on urinalysis, were within normal limits.