Congenital Abnormalities Of Coronary Arteries Research Articles

Absent Right Coronary Artery in an Active Young Female

Absent Right Coronary Artery in an Active Young Female

1027 CONGENITAL CORONARY ARTERY ABNORMALITIES: THE DIAGNOSTIC AND PROGNOSTIC VALUE OF CARDIAC COMPUTED TOMOGRAPHY

Abstract Introduction To the best of our knowledge, there are no previous univocal data on how CCT can accurately describe morphological variants and subtypes of congenital coronary artery abnormalities. Taking into consideration the extensive application of CCT in clinical practice in the last decades, it is not uncommon to have a congenital coronary artery anomaly detected at CCT. Thus, the study aim is to evaluate the diagnostic and prognostic impact of specific cardiac CT parameters in subjects with a diagnosis of congenital coronary artery anomalies. Material and Methods This is an intermediate evaluation of a prospective clinical registry on a population of subjects who underwent a cardiac computed tomography imaging evaluation in the period between January 2007 and October 2015 and were diagnosed to have a congenital coronary abnormality. For the present preliminary analysis only coronary anomalies of origin have been considered and 92 patients have been included. Follow-up have been collected either through a structured telephonic interview or through the evaluation of clinical records of subsequent hospitalization or ambulatory visits. Results The population enrolled in the present study has an average age of 63,0 ± 12,8 years (range 34 to 85 years), with a male prevalence of 69,6%. The left main artery is missing in 46 individuals (50%), with separate origin of LAD and LCX. Only two duplications (2.2%), one for the left coronary artery and one for the right coronary artery, have been discovered. Six participants (6.5%) had a non-coronary artery origin abnormalities and pulmonary artery was the site of origin in the majority of cases. 51 subjects (55,4%) have an anomalous origin of the coronary artery from a different coronary sinus and 50 subjects had also an anomalous course with the retro-aortic being the most common (30,4%). 11 participants (12,0%) displayed also an intramural segment and 16 (17,4%) had abnormal ostial morphology. The high take-off of the vessel was uncommon (3,3%). Age appears to be the only relevant characteristic; indeed, it displays a significant correlation both to MACE (OR 95% CI 1.03, 1.01-1.07; p=0.0349) and to all-cause of death (OR 95% CI 1.14, 1.02-1.27; p=0.0151). None of the traditional cardiovascular risk factors were found to be significantly linked with adverse outcomes in this study sample of coronary anomalies. Conclusions The result of this intermediate evaluation is that cardiac CT can be successfully used to define the anatomy and features of CAA. However, it demonstrated that in middle-aged patients, the tomographic finding of an abnormality of coronary origin might not have a meaningful, strong negative prognostic value in terms of major cardiovascular events and all-cause of death. This means that probably, in the majority of the cases, once the diagnosis is performed later on in life, no further systematic assessment is needed but a personalized approach should be suggested.

P48 ALCAPA Presenting With Cardiac Arrest

Congenital coronary artery abnormalities are rare and have an embryogenesis that is not well understood. Anomalous left coronary artery from the pulmonary artery (ALCAPA) accounts for 0.4% of congenital cardiac abnormalities, often presenting with myocardial ischaemia due to coronary mal-perfusion [ [1] Cheezum M.K. Liberthson R.R. Shah N.R. Villines T.C. O’Gara P.T. Landzberg M.J. et al. Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva. J Am Coll Cardiol. 2017; 69: 1592-1608 Crossref PubMed Scopus (107) Google Scholar ].

Echocardiographic diagnosis of congenital coronary artery abnormalities in a continuous series of adolescent football players.

Sudden cardiac death (SCD) in children and adolescents is rare. Several studies have reported a higher risk of SCD during athletic competition. High risk congenital coronary artery abnormalities are the second leading cause of SCD in young athletes in the USA. Echocardiographic assessment of coronary arteries has not been routinely used in screening programmes for junior athletes so far. Prospective cohort study in 1045 consecutive adolescent elite football players. All athletes underwent a standardized cardiovascular screening protocol with a medical history, a physical examination, 12-lead resting electrocardiogram and a complete transthoracic 2D-echocardiography. Two athletes (0.19%) showed a high-risk coronary artery abnormality (CAA) with a right coronary artery originating abnormal from the aorta and coursing inter-arterial. Low-risk CAAs were found in 16 athletes (1.53%). There was an ectasia of the left coronary artery (+3.9z and +4.3z) and a fistula from the left coronary artery in two cases (0.19%), respectively. In 1.05% ( n = 11) we found a high take-off (2.3-6.8 mm) and in one case (0.096%) there was a tangential take-off of the right main coronary artery. Variants of coronary arterial anatomy were identified in 335 of 1045 athletes (32.06%). Basic pre-participation screening tests including 12-lead or exercise electrocardiogram do not safely identify high-risk CAAs. In adolescent athletes an expert cardiologist is able to describe the origin and the proximal course of the coronary arteries and identify major abnormalities in most of the cases by transthoracic 2D-echocardiography.

Iznenadna srčana smrt - mere prevencije i preporuke za bavljenje fizičkom aktivnošću

Physical activity provides many well-documented health benefits, while physical inactivity is a leading risk factor for cardiovascular morbidity and mortality. In order to achieve these benefits, one must expose himself to relatively moderate physical activity. However, many athletes and recreationists go beyond these limits and engage in higher level of physical activity. Occasionally, intense physical activity is associated with sudden death in individuals with concealed heart disease. Despite the media attention provided by such accident, the reputation of physical activity remains intact, as most deaths can be explained by a basic cardiac abnormality where exercise is only the trigger of a fatal arrhythmia, not the actual cause of death. The paradox is that athletes with these abnormalities show identical performance in the form of minute heart volume compared to healthy individuals. In athletes younger than 35, congenital heart disease, especially hypertrophic cardiomyopathy and congenital coronary artery abnormalities, are the leading cause of sudden cardiac death, while most athletes older than 35 die due to atherosclerotic changes in the coronary arteries. Pre-competition screening with apparently healthy individuals is the only way to prevent sudden cardiac death in sports. When diagnosing a specific condition of the cardiovascular system, it is necessary to determine the risk of sudden cardiac death associated with continued physical activity and competitive sports activity, and to define clear disqualification criteria for each athlete individually.

Acquired and congenital coronary artery abnormalities.

Sudden unexpected cardiac deaths in approximately 20% of young athletes are due to acquired or congenital coronary artery abnormalities. Kawasaki disease is the leading cause for acquired coronary artery abnormalities, which can cause late coronary artery sequelae including aneurysms, stenosis, and thrombosis, leading to myocardial ischaemia and ventricular fibrillation. Patients with anomalous left coronary artery from the pulmonary artery can develop adequate collateral circulation from the right coronary artery in the newborn period, which remains asymptomatic only to manifest in adulthood with myocardial ischaemia, ventricular arrhythmias, and sudden death. Anomalous origin of coronary artery from the opposite sinus occurs in 0.7% of the young general population aged between 11 and 15 years. If the anomalous coronary artery courses between the pulmonary artery and the aorta, sudden cardiac death may occur during or shortly after vigorous exercise, especially in patients where the anomalous left coronary artery originates from the right sinus of Valsalva. Symptomatic patients with evidence of ischaemia should have surgical correction. No treatment is needed for asymptomatic patients with an anomalous right coronary artery from the left sinus of Valsalva. At present, there is no consensus regarding how to manage asymptomatic patients with anomalous left coronary artery from the right sinus of Valsalva and interarterial course. Myocardial bridging is commonly observed in cardiac catheterisation and it rarely causes exercise-induced coronary syndrome or cardiac death. In symptomatic patients, refractory or β-blocker treatment and surgical un-bridging may be considered.

Critical care for patients with congenital abnormalities of the coronary arteries.

Congenital abnormalities of the coronary arteries in the absence of structural heart disease account for a small but interesting percentage of cardiac lesions in children. Their presentation may vary from incidental identification to aborted/sudden cardiac death. Patients with aborted sudden death episodes will require significant support if they develop extensive ischaemic myocardial injury. Ultimately, surgical repair should be carried out as soon as haemodynamic stability is attained and the neurological status is evaluated. The aims of this article were to provide a review of congenital abnormalities of the coronary arteries most commonly seen in children in the ICU as well as to review the current critical-care management thereof.

Prevalence of congenital coronary artery abnormalities in south asian patients with pulmonary stenosis and VSD

Background Incidence of anomalous coronary anatomy (ACA) in caucasian and middle eastern patients with ventricular septal defect (VSD) and pulmonary stenosis (PS) is between 29%. Prevalence of ACA in South Asian (SA) patients is unknown. Patients with VSD and PS may have Tetrology of Fallot (TOF) or Double Outlet Right ventricle (DORV) morphology. This study looks at the prevalence of ACA in SA patients with TOF/DORV. Pre-operative assessment of ACA is of paramount importance in these patients as it alters surgical procedure.

Connexion ectopique de l’artère coronaire gauche dans le sinus controlatéral avec ou sans passage intramural : comment et pourquoi les différencier ?

Among the wide spectrum of congenital abnormalities of coronary arteries, a left coronary artery connected with the contralateral sinus is considered as an anatomical high-risk while associated with a proximal preaortic course. The ectopic connection may be associated with or without intramural pathway regarding the aortic wall. An intramural pathway is a characteristic to be considered because it is able to be involved in myocardial ischemia-related adverse events of which the sudden death. Therefore, making an identification of an intramural pathway is essential for the ectopic connections of the left coronary artery associated with a preaortic course.

Ostium coronaire unique : artère coronaire unique ou artère coronaire ectopique connectée à l’artère controlatérale. Comment et pourquoi les différencier ?

Among the wide spectrum of congenital abnormalities of coronary arteries, a single coronary artery is often confused with an ectopic coronary artery connected with the contralateral coronary artery. Both abnormalities are characterized by a single coronary ostium, but they differ by the lack or not of an initial ectopic course. The prognosis of anomalous connections of coronary arteries depends mainly on the type of the initial course in relation to other cardiac structures. Therefore, the distinction between a single coronary artery and an ectopic coronary artery connected with the contralateral artery is of importance.

Early neonatal death and congenital left coronary abnormalities: Ostial atresia, stenosis and anomalous aortic origin

Congenital left coronary artery abnormalities such as ostial stenosis or atresia are extremely rare. Diagnosis in the neonate has not been reported. To describe five neonates with left coronary artery orifice abnormalities and discuss pathophysiology, diagnosis and treatment options, with a focus on the importance of autopsy in unexpected neonatal death. Retrospective assessment of medical files of neonates with left coronary abnormalities seen during a 12-year period (2000-2012). Three neonates with anatomical (n=2) and functional (n=1) left coronary stenosis and two neonates with ostial atresia were identified. The three infants with coronary stenosis died within minutes to days after birth because of cardiac failure refractory to intensive care treatment; at autopsy, left coronary ostial stenosis (n=2) and high take-off with acute angle origin and tangential vertical course (n=1) were diagnosed. The fourth neonate was in cardiac failure due to critical aortic stenosis; left coronary ostial atresia was diagnosed during an emergency catheter procedure and the infant died after aortic valve dilatation. The fifth infant had a cardiac arrest on the third day of life; she was diagnosed with left coronary ostial atresia by coronary angiography and died during attempted revascularization surgery at 2 weeks of life. Congenital coronary ostial abnormalities can lead to severe heart failure and unexpected neonatal death. Systematic examination of the coronary arteries should be part of any neonatal autopsy. Coronary angiography remains the diagnostic method of choice despite advances in non-invasive imaging. Revascularization surgery seems indicated in symptomatic children based on small patient series.

The Role of Computed Tomography Coronary Angiography in the Management of Coronary Anomalies

Congenital abnormalities of coronary arteries are an uncommon cause of sudden cardiac death and are difficult to detect at coronary angiography. We describe two patients with acute coronary syndrome and non-occlusive coronary artery disease in which a 64-multidetector computed tomography (MDCT) coronary angiography showed the presence of a malignant coronary anomaly. Sixty-four-MDCT with the possibility of 3D reconstructions allows for easier diagnosis of coronary anomalies and provides essential details necessary for operative intervention.

Congenital Coronary Artery Abnormalities Predisposing to Sudden Cardiac Death

Isolated congenital coronary artery anomalies (CCAAs) are a rare but well-described cause of sudden cardiac death (SCD). Anomalous origin of a coronary artery from the opposite sinus has been most frequently associated with myocardial ischemia, ventricular arrhythmias, and sudden death, particularly when the anomalous coronary courses between the great arteries. Importantly, symptoms occur in only about 50% of people with CCAA who subsequently have SCD. Echocardiography is effective in prospectively identifying these coronary artery abnormalities but requires sophisticated echo equipment and a laboratory examination with targeted, high-quality imaging. Although surgical intervention for some forms of CCAA is feasible with good early results and is clearly indicated for symptomatic patients, no clear best practice has been established for the management of those identified serendipitously without symptoms.

Congenital Coronary Artery Abnormalities in Children.

After a significant coronary artery abnormality is recognized in a pediatric patient, surgery or appropriate transcatheter intervention should be performed. The risk of fatality from a congenital coronary abnormality far outweighs the small risks of surgical or transcatheter intervention. Angiography, although considered the state-of-the-art method of diagnosis, has significant spatial limitations and is not always diagnostic of aberrant coronary origins from the contralateral aortic sinus. In the hands of an experienced coronary imager, color flow Doppler echocardiography is one of the best diagnostic tools for congenital coronary abnormalities. Symptoms of a coronary abnormality vary from none to a sudden coronary event that may result in death. Awareness of subtle as well as obvious symptoms is essential for a timely intervention. Surgical or transcatheter intervention in an asymptomatic child with a coronary abnormality is controversial, but it is becoming more acceptable due to a better understanding of the risks involved in unrepaired congenital coronary abnormalities. Surgical reimplantation is the treatment of choice for a patients with a pulmonary origin of a coronary artery. Surgical unroofing of the intramural segment is preferable in an aberrant coronary origin from the contralateral aortic sinus. Surgical enlargement of a stenotic ostium is recommended for ostial stenosis. Transcatheter coil embolization is becoming the treatment of choice of large coronary artery fistula.

Congenital coronary artery abnormalities

Congenital coronary artery abnormalities are rare and account for approximately 0.1 to 2% of congenital heart defects. They may pose significant risk of mortality or morbidity to the patient. The pediatrician and the pediatric cardiologist should be aware of their subtle but very serious presentations and diagnostic steps to be undertaken to pinpoint the diagnosis. Prevention of serious complications from these abnormalities can be achieved by making the appropriate diagnosis and performing timely surgical intervention. This review will discuss the most common congenital coronary artery abnormalities and their management.

Diagnosis of Congenital Coronary Artery Abnormalities by Echoca rdiography

Congenitai abnormalities of the coronary arteries arerarbut may resu t in imprtant morta ity and morb idtyfy not Vrecgnzed. This case report describes the clinicala presentaton, physiology, and ecocardiographic features of two types of congenital coronar artery abnormalities.

Association of Right Coronary Artery Hypoplasia with Sudden Death in an Eleven-Year-Old Child

Congenital coronary artery abnormalities are a rare but well-documented cause of sudden and unexpected death in the pediatric age group. Most reported cases involve both an aberrant origin and course of the abnormal vessel. A case of unexpected death occurring in an otherwise healthy eleven-year-old boy, who had been previously investigated for sudden collapse, is described. The major finding at autopsy was marked disparity in diameter between the coronary arteries due to diffuse hypoplasia of the right coronary artery. This case demonstrates the importance of meticulous examination of the coronary artery system in cases of sudden death in childhood so that significant reduction in luminal cross section will be adequately documented. The possible role played by this finding in the etiology of sudden death is discussed.

Sudden death and the competitive athlete: Perspectives on preparticipation screening studies

Sudden death in healthy athletes is uncommon but, when it occurs, the primary mechanism is cardiovascular. The major cause of sudden death in the young athlete is hypertrophic cardiomyopathy or related conditions characterized by left ventricular hypertrophy, aortic rupture due to cystic medial necrosis and congenital coronary artery abnormalities. In the middle-aged or older athlete, coronary artery disease is the most significant cause of sudden death. Noninvasive screening procedures are currently available that can detect most subjects at risk of sudden death. However, although some potentially lethal diseases can be excluded by a relatively simple screening program, other diseases require expensive procedures, such as echocardiography and exercise electrocardiographic stress testing. This means that the sensitivity of detecting diseases leading to sudden death increases in proportion to the financial resources that can be applied to the screening program. Thus, when a screening program designed to identify all cardiac diseases that have the potential to cause sudden death is planned by a community, school or nonprofessional athletic team, the costs will almost undoubtedly be considered prohibitive. The practicality of applying a community- or school-initiated screening program can be questioned because of the very low incidence of sudden unexpected death in young healthy individuals. It is therefore likely that comprehensive screening programs will be confined to individuals or organizations with adequate financial resources. Less expensive, limited screening can be undertaken by individuals or groups to identify some subjects at risk of sudden death during athletic competition.

Hypoplastic Coronary Arteries and High Takeoff Position of the Right Coronary Ostium: A Fatal Combination of Congenital Coronary Artery Anomalies in an Amateur Athlete

This report describes a previously unrecognized combination of congenital coronary artery abnormalities in the heart of a 30-year-old amateur athlete who died suddenly during a basketball game. Both right and left circumflex coronary arteries were half of their normal length (hypoplastic) decreasing posterior ventricular myocardial perfusion. In addition, the right coronary ostium rose 5 mm above the sinotubular junction (high takeoff position), which also contributed to decreased right coronary artery perfusion. This combination of congenital coronary arterial lesions should be added to the list of structural cardiac defects associated with exercise-related sudden death.