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Pasaoglu, Lale, Ugur Toprak, Emre Nalbant, and Gokhan Yagiz. "A Rare Coronary Artery Anomaly: Origin of All Three Coronary Arteries from the Right Sinus of Valsalva." Journal of Clinical Imaging Science 5 (April 30, 2015): 25. http://dx.doi.org/10.4103/2156-7514.156137.
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Left anterior descending (LAD) artery and left circumflex (LCx) coronary artery originating separately from the right sinus of valsalva is exceptionally rare and very few cases have been reported in the literature. Congenital coronary artery anomalies are generally incidental, uncommon, and asymptomatic. Some can cause severe potentially life-threatening symptoms such as myocardial ischemia and sudden cardiac death. The aberrant vessels that pass between the aorta and the pulmonary trunk pose a risk of sudden cardiac death, particularly if the vessel supplies the left coronary artery network. The electrocardiographically gated multi-detector computed tomography (MDCT) allows accurate and non-invasive depiction of coronary artery anomalies including origin, course, and termination. We report here a rare case of all three coronary arteries separately originating from the right coronary sinus, which was detected with MDCT.
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Coppi, Francesca, Sara Roversi, Enrico Giuliani, Raffaele Sansone, Giuseppe Massimo Sangiorgi, Maria Grazia Modena, and Alberto Barbieri. "Circumflex Coronary Artery Agenesis Associated With Thoracic Great Vessels Anomalies." Journal of Thoracic Imaging 26, no. 1 (February 2011): W9—W11. http://dx.doi.org/10.1097/rti.0b013e3181e35a9e.
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Çanga, Yiğit, Tolga S. Güvenç, Mehmet B. Karataş, Ali N. Çalık, Tolga Onuk, Veysel O. Tanık, Barış Güngör, and Osman Bolca. "Congenital coronary artery anomalies in adults: review of 111 cases from a single-centre experience." Cardiology in the Young 27, no. 6 (November 15, 2016): 1041–50. http://dx.doi.org/10.1017/s1047951116001906.
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AbstractBackgroundCoronary artery anomalies are a heterogeneous group of congenital disorders presenting with a wide spectrum of symptoms, ranging from vague chest pain to sudden cardiac death. Despite available data, there is no consensus about the classification, nomenclature, and outcomes of coronary anomalies in the normally connected heart. In this study, we aimed to investigate clinical and angiographic characteristics of coronary arterial anomalies, as well as the frequency of atherosclerotic involvement in anomalous coronaries, diagnosed at a tertiary referral centre.MethodsWe retrospectively reviewed coronary angiograms performed between 2011 and 2015 for the presence of a coronary anomaly. A total of 111 patients with a final diagnosis of coronary anomaly were included in the study group. We also recruited 110 age- and sex-matched patients who underwent coronary angiography because of symptomatic coronary artery disease as controls.ResultsAmong 36,893 coronary angiograms, 111 (0.30%) major coronary anomalies were found. Compared with controls, the prevalence of significant atherosclerotic coronary disease was lower in patients with coronary anomalies and stable symptoms (p=0.02); however, the prevalence of significant coronary atherosclerosis was similar among patients admitted with unstable angina or myocardial infarction (p>0.05). Compared with controls, patients with an anomalous left anterior descending coronary artery had significantly less atherosclerotic involvement than those in whom the left anterior descending artery was not anomalous (p=0.005).ConclusionsAlthough coronary artery anomalies are cited as a cause for myocardial ischaemia, atherosclerotic coronary artery disease is also frequent and may offer an alternative explanation to ischaemic symptoms. No predisposition to accelerated atherosclerosis was found, however, and atherosclerotic involvement was less frequent in some anomalous vessels.
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Ono, Yoshikazu, Takaya Hoashi, Kenichi Kurosaki, and Hajime Ichikawa. "Successful Staged Repair for Truncus Arteriosus With Anomalous Arch Vessels and Left Coronary Artery." World Journal for Pediatric and Congenital Heart Surgery 10, no. 5 (September 2019): 643–44. http://dx.doi.org/10.1177/2150135119857705.
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A patient was born with truncus arteriosus type 2, left aortic arch, anomalous origin of arch vessels, left coronary artery from the right common carotid artery, and multiple extracardiac anomalies. Surgery involving translocation of the left coronary artery to the truncal root, division of branch pulmonary arteries from the truncal artery, and right ventricle-to-branch pulmonary arteries conduit placement was performed at the age of four months. Closure of the ventricular septal defect using a one-way fenestrated patch and conduit upsizing was performed successfully when the patient reached four years of age.
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Duarte, S. B. C. P., D. O. Beraldo, L. A. M. Cesar, A. P. Mansur, and J. Y. Takada. "Anomalous Coronary Artery Origin in a Young Patient with Marfan Syndrome." Case Reports in Cardiology 2017 (2017): 1–5. http://dx.doi.org/10.1155/2017/3861923.
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Marfan syndrome is an autosomal dominant genetic disorder that affects connective tissue and is caused by mutations in the fibrillin 1 gene present at chromosome 15. Aortic aneurysm is its main complication, and along the dilation of the aorta root and its descending portion (60–100%), with secondary aortic insufficiency, it increases risk of acute aortic dissection and death. Coronary artery anomalies affect between 0.3% and 1.6% of the general population and are the second leading cause of sudden death in young adults, especially if the anomalous coronary passes through aorta and pulmonary artery. The anomalous origin of the left main coronary artery in the right Valsalva sinus has a prevalence of 0.02%–0.05% and is commonly related to other congenital cardiac anomalies, such as transposition of great vessels, coronary fistulas, bicuspid aortic valve, and tetralogy of Fallot. Its association with Marfan syndrome is not known, and there is no previous report in the literature. We describe here a case of a female with Marfan syndrome diagnosed with symptomatic anomalous origin of the left coronary artery in the right Valsalva sinus.
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Mahla, Rakesh, Himanshu Mahla, Dinesh Choudhary, and Pintu Nahata. "Percutaneous Coronary Intervention in Single Coronary Artery from Right Sinus: Radial Route is Right." Journal of Clinical Imaging Science 5 (November 30, 2015): 65. http://dx.doi.org/10.4103/2156-7514.170735.
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We present percutaneous coronary intervention (PCI) using radial approach in a rare case of single coronary artery originating from the right sinus. Although these anomalies and stenosis of anomalous vessels have been described previously, treatment of atherosclerotic lesions by PCI has rarely been reported. There is a definite procedural risk during PCI in patients with a single ostium because dissection with the guiding catheter would result in a catastrophic event. Additionally, technical difficulties may occur due to the ostial configuration and course of the branch to be stented. The patient suffered an acute coronary syndrome-inferior wall STEMI, and was thrombolysed elsewhere within a window period of 4 h. He had post myocardial infarction (MI) angina and was referred to our center after 3 days of thrombolysis. We present this technically challenging and rare case in which PCI of right coronary artery was performed through the radial route.
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Friedman, Alan H., Mark A. Fogel, Paul Stephens, Jeffrey C. Hellinger, David G. Nykanen, James Tweddell, Timothy F. Feltes, and Jonathan J. Rome. "Identification, imaging, functional assessment and management of congenital coronary arterial abnormalities in children." Cardiology in the Young 17, S4 (September 2007): 56–67. http://dx.doi.org/10.1017/s1047951107001163.
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AbstractThe coronary arteries, the vessels through which both substrate and oxygen are provided to the cardiac muscle, normally arise from paired stems, right and left, each arising from a separate and distinct sinus of the aortic valve. The right coronary artery runs through the right atrioventricular groove, terminating in the majority of instances in the inferior interventricular groove. The main stem of the left coronary artery bifurcates into the anterior descending, or interventricular, and the circumflex branches. Origin of the anterior descending and circumflex arteries from separate orifices from the left sinus of Valsalva occurs in about 1% of the population, while it is also frequent to find the infundibular artery arising as a separate branch from the right sinus of Valsalva.Anomalies of the coronary arteries can result from rudimentary persistence of an embryologic coronary arterial structure, failure of normal development or normal atrophy as part of development, or misplacement of connection of a an otherwise normal coronary artery. Anomalies, therefore, can be summarized in terms of abnormal origin or course, abnormal number of coronary arteries, lack of patency of the orifice of coronary artery, or abnormal connections of the arteries.Anomalous origin of the left coronary artery from the pulmonary trunk occurs with an incidence of approximately 1 in 300,000 children. The degree of left ventricular dysfunction produced likely relates to the development of collateral vessels that arise from the right coronary artery, and provide flow into the left system. Anomalous origin of either the right or the left coronary artery from the opposite sinus of Valsalva can be relatively innocuous, but if the anomalous artery takes an interarterial course between the pulmonary trunk and the aorta, this can underlie sudden death, almost invariably during or immediately following strenuous exercise or competitive sporting events. Distal anomalies of the coronary arteries most commonly involve abnormal connections, or fistulas, between the right or left coronary arterial systems and a chamber or vessel.We discuss the current techniques available for imaging these various lesions, along with their functional assessment, concluding with a summary of current strategies for management.
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Aleksandric, Srdjan, Sinisa Stojkovic, Miloje Tomasevic, Jelena Kostic, Marko Banovic, Nemanja Menkovic, and Miodrag Ostojic. "Primary percutaneous coronary intervention in a patient with right internal mammary artery graft originating from arteria lusoria dextra." Srpski arhiv za celokupno lekarstvo 141, no. 3-4 (2013): 223–27. http://dx.doi.org/10.2298/sarh1304223a.
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Introduction. Congenital anomalies of the aortic arch, although numerous and heterogeneous, occur in less than 1% of individuals at autopsies. Left aortic arch with an aberrant right subclavian artery, also called arteria lusoria dextra, is the most common anomaly of the aortic arch, occurring in 0.5-2.5% of individuals. Case Outline. We report the case of a 48-year-old man suffering from acute inferoposterior-wall ST elevation myocardial infarction successfully treated by primary percutaneous coronary intervention. Ten years ago, the patient had undergone coronary artery bypass graft surgery with the implantation of two arterial grafts - left and right internal mammary arteries on both left anterior descending and right coronary artery. After several attempts to canulate truncus brachiocephalicus, angiogram revealed the left aortic arch with the aberrant right subclavian artery. To our knowledge, this is the first described case of primary percutaneous coronary intervention via the aberrant right subclavian artery and right internal mammary artery graft with stent implantation in the infarct related lesion of the distal segment of right coronary artery. Subsequent 64-multidetector computed tomography confirmed the angiographic findings. Conclusion. Early recognition of congenital anomalies of the aortic arch and its great vessels, even before coronary artery bypass graft surgery, could be crucial for the urgent and successful treatment of patients with life-threatening conditions, such as ST segment elevation myocardial infarction.
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Slim, Ahmad, John Thurlow, Jennifer Blevins, Shaun Martinho, and Brian Markelz. "Discovery of a Symptomatic Left Anomalous Coronary Artery from the Opposite Sinus and Postoperative Considerations." Case Reports in Medicine 2009 (2009): 1–3. http://dx.doi.org/10.1155/2009/509064.
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This is the case of an 18 year old active duty soldier with symptoms of exertional chest pressure and syncope who was found to have anomalous origin of the left main coronary artery (LMCA) from the right coronary cusp (RCC) traveling partially between the great vessels before taking a septal approach between the left ventricular outflow tract (LVOT) and the right ventricular outflow tract (RVOT). Anomalous origin of coronary arteries is a rare condition that carries an increased risk of angina, myocardial ischemia, and sudden cardiac death (SCD). Surgical treatment of such anomalies with both high and lower risk features can be challenging, and traditional benefit from surgical correction may not be achieved due to complex anatomy. As evident by our patient, this rare condition even though benign from sudden death standpoint could be debilitating despite best efforts and available resources.
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Jaswal, Vivek, Shyam Kumar Singh Thingnam, Vikas Kumar, Ruchit Patel, Ganesh Kumar Munirathinam, and Dheemta Toshkhani. "A single coronary artery with left circumflex artery crossing right ventricular outflow tract in tetralogy of Fallot with absent left pulmonary artery." Journal of Cardiovascular and Thoracic Research 13, no. 1 (December 23, 2020): 87–89. http://dx.doi.org/10.34172/jcvtr.2020.61.
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Tetralogy of Fallot (TOF) with unilateral absence of pulmonary artery and the anomalous coronary artery is a rare combination. Detailed preoperative evaluation of coronary artery anatomy is must to prevent injury to the major vessels crossing right ventricular outflow tract. We report a rare association of single coronary artery with left circumflex artery crossing right ventricular outflow tract close to the pulmonary annulus in tetralogy of Fallot with absent left pulmonary artery in 11-year-old girl. Though there is a great diversity of coronary anomalies in tetralogy of Fallot, the prepulmonic course of left circumflex artery crossing the right ventricular outflow tract (RVOT) close to the pulmonary annulus has rarely been described in the literature. The patient underwent successful primary single lung intracardiac repair. Right ventricular outflow tract obstruction was treated by handmade valved pericardial autologous conduit and release of the tethering of hypoplastic native unicuspid pulmonary valve leaflet maintaining its integrity.
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Valencia, Damian, Juan Linares, Sulagna Das, Victor Valencia, and Yan Yatsynovich. "Uncommon Coronary Abnormalities in a Patient with Discoid Lupus Erythematosus." International Journal of Angiology 29, no. 04 (September 12, 2019): 260–62. http://dx.doi.org/10.1055/s-0039-1696978.
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AbstractA 43-year-old male presented with shortness of breath and palpitations. Physical examination was significant for skin lichenification, an erythematous maculopapular rash with annular plaques, a fissured tongue, and digital clubbing. Electrocardiogram captured a supraventricular tachycardia and right bundle branch block. Left heart catheterization found several proximal left coronary artery branch aneurysms, a right coronary–pulmonary artery fistula, venous–luminal vessels between the right heart chambers and cardiac veins, and thebesian venous lakes. Functional cardiac capacity was reduced, with left ventricular ejection fraction of 35 to 40%, on ventriculogram. A diagnosis of discoid lupus erythematosus was established after skin biopsies revealed systemic lupus erythematosus-like features but negative anti-nuclear, anti-Smith, anti-ds-deoxyribonucleic acid, anti-Ro, and anti-La antibodies. The case reported is unique, in that while our patient had pathologic electrophysiologic changes typically seen with autoimmune disease, the patient had many anatomical cardiac anomalies without atherosclerotic coronary disease. It is uncertain whether these findings are purely incidental or are related to an underlying genetic disorder.
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Kalinin, R. E., I. A. Suchkov, E. A. Klimentova, and I. N. Shanaev. "RARE VERSION OF TOPOGRAPHY OF DEEP FEMORAL ARTERY." NAUKA MOLODYKH (Eruditio Juvenium) 8, no. 4 (December 30, 2020): 591–98. http://dx.doi.org/10.23888/hmj202084591-598.
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The deep femoral artery is a large branch of the common femoral artery that is of much interest for vascular and endovascular surgeons due to the role it plays in collateral circulation between the vessels of the small pelvis and arteries of the popliteal-tibial segment. In most cases, the deep femoral artery branches off from the posterolateral or posterior surface of the common femoral artery. At the same time, anomalies of development of the deep femoral vessels may become the cause for iatrogenic damages in implementation of the open or endovascular interventions. In the article, a clinical case of a patient referred for a planned ultrasound examination of vessels of the lower limbs before angiographic examination of the vessels of the heart and of the lower limbs, is described that revealed atypical topography of branching of the two trunks of the deep femoral artery from the common femoral artery. The upper trunk of the deep femoral artery branched off from the anteromedial surface of the common femoral artery and in the initial part was positioned above the common femoral vein. The lower trunk of the deep femoral artery sepa-rated from the anterolateral surface of the common femoral artery. Preoperative identification of the variant anatomy of the vessels of the femoral triangle permitted to perform angiographic examination of the coronary vessels through the femoral artery on the contralateral limb without complications.
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Das, Satyaki, Swapan Kumar Ray, Sukanta Bhattacharya, Kripasindhu Chatterjee, Pradyut Kumar Mandal, and Sukanta Sen. "Coronary artery diameter measurement and Z-score regression equation calculation: a comparative study between Indian children between one to five years residing in and around Kolkata with children of South-East Asia and Western population." International Journal of Contemporary Pediatrics 4, no. 2 (February 22, 2017): 403. http://dx.doi.org/10.18203/2349-3291.ijcp20164849.
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Background: Congenital coronary anomalies may be isolated, or they may accompany other congenital heart defects, such as Fallot's tetralogy, transposition of the great arteries or pulmonary atresia. The most common cause of acquired abnormalities of the coronary vessels in children is kawasaki disease (KD). The aim of this study was to find the best model to obtain valid and normally distributed Z-scores for coronary artery (CA) diameters in a large, heterogeneous population of healthy children.Methods: Echocardiography was performed on 300 healthy children. Linear regression models were tested with height, weight, body surface area, and aortic valve diameter. The computed Z scores were tested for normal distribution and stability. Results: CA diameter was best predicted using regression with the body surface area and age in month. The weighted least squares method yielded normally distributed and very stable Z-score estimates for 3 principal CAs.Conclusions: This study showed valid methods to estimate Z scores for CA size in children of all ages. Such Z scores are important for risk stratification in patients with Kawasaki disease.
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Kholkina, Aleksandra A., Yuriy R. Kovalev, Vladimir A. Isakov, and Natal’ya O. Gonchar. "Myocardial bridge and coronary artery fistulas in a patient with angina." Pediatrician (St. Petersburg) 10, no. 2 (June 19, 2019): 137–41. http://dx.doi.org/10.17816/ped102137-141.
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Cardiovascular diseases (CVDs) are the leading cause of mortality among the population. At the core of the progression of the coronary heart disease is the atherosclerosis of the coronary arteries, which is found in majority of patients suffering from angina and in patients with myocardial infarction. However, in some cases, coronary angiography reveals, that patients with the mentioned clinical manifestations have their coronary arteries unchanged. This is treated as syndrome X or microvascular angina. Along with that, development or aggravation of the coronary heart disease may be based on the congenital peculiarities in the coronary arteries location and structure, such as muscular bridges and fistulas of the coronary artery. This is confirmed by a number of studies, which indicate the role of the above mentioned pathologies in the occurrence of angina and myocardial infarction. Nevertheless, there is also the opposite view, which is supported by a number of specialists. According to them, the presence of the mentioned peculiarities in the structure of the coronary channel is deemed as the patient-specific norm. Hence, the issue of the surgical treatment of the patients with the aforementioned coronary arteries anomalies remains controversial. The clinical case report of the patient with the symptoms of angina pectoris, in which the coronary angiography did not reveal the stenosis of the coronaries arteries, but located the myocardial bridge and the coronary fistula. The role of the congenital coronary vessels pathology in the angina pectoris is analyzed. The diagnosis guidelines and the tactics of the conservative and surgical treatment of patients with the above mentioned syndromes are discussed.
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Fofanova, O. V. "Syndrome Hutchinson-Gilford (progeria)." Problems of Endocrinology 41, no. 4 (August 15, 1995): 24–26. http://dx.doi.org/10.14341/probl11459.
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Gatchinson-Guildford syndrome or progeria (senile dwarfism) is an extremely rare genetic disease in children with clinical features of premature aging. The frequency of the disease is 1 in 8 million newborns (De Busk. 1972). To date, about 70 patients with this syndrome have been described in world literature. The etiology of progeria is unclear. The genetic model of inheritance is unknown due to the extreme rarity of the syndrome and the absence of offspring in patients. However, studies by foreign scientists allow us to talk about sporadic dominant mutation as the genetic basis of this syndrome. The clinical picture of progeria is represented by symptoms of progressive premature aging. Characteristic face: exophthalmos, a thin coracoid nose, a large brain and small facial skull, a thin voice, skeletal anomalies. Puberty usually does not occur; external genitalia hypoplasia. Intelligence average or above average. This syndrome is characterized by widespread atherosclerosis with lesions of coronary and mesenteric vessels, aorta, cerebral vessels, with hyperlipidemia. Progeria as a model of premature aging is studied in various aspects: metabolic, hormonal, histological, immunological, molecular.
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Sithamparanathan, Sasi, Simon P. G. Padley, Michael B. Rubens, Michael A. Gatzoulis, Siew Yen Ho, and Edward D. Nicol. "Great Vessel and Coronary Artery Anatomy in Transposition and Other Coronary Anomalies." JACC: Cardiovascular Imaging 6, no. 5 (May 2013): 624–30. http://dx.doi.org/10.1016/j.jcmg.2012.10.027.
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Al-Umairi, Rashid S., Faiza A. Al-Kindi, and Saqar A. Al-Tai. "A New Variant of Dual Left Anterior Descending Artery Anomaly: Type XI." Sultan Qaboos University Medical Journal [SQUMJ] 18, no. 3 (December 19, 2018): 386. http://dx.doi.org/10.18295/squmj.2018.18.03.021.
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A dual left anterior descending (LAD) artery is a rare congenital anomaly which is classified into different types based on the origin, course and termination of the short and long LAD arteries. To date, 10 variants of dual LAD artery anomalies have been described. We report a 44-year-old woman who was referred to the Department of Radiology, Royal Hospital, Muscat, Oman, in 2017. Coronary computed tomography angiography revealed a dual LAD artery anomaly in which the short and long LAD arteries shared a common ostium with the right coronary artery from the right coronary sinus. To the best of the authors’ knowledge, this type of variant has not been previously reported in the literature.Keywords: Coronary Angiography; Congenital Abnormality; Coronary Vessel Anomalies; Case Report; Oman.
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Anantha Narayanan, Mahesh, Christopher DeZorzi, Abhilash Akinapelli, Toufik Mahfood Haddad, Aiman Smer, Janani Baskaran, and William P. Biddle. "Malignant Course of Anomalous Left Coronary Artery Causing Sudden Cardiac Arrest: A Case Report and Review of the Literature." Case Reports in Cardiology 2015 (2015): 1–4. http://dx.doi.org/10.1155/2015/806291.
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Sudden cardiac arrest has been reported to occur in patients with congenital anomalous coronary artery disease. About 80% of the anomalies are benign and incidental findings at the time of catheterization. We present a case of sudden cardiac arrest caused by anomalous left anterior descending artery. 61-year-old African American female was brought to the emergency department after sudden cardiac arrest. Initial EKG showed sinus rhythm with RBBB and LAFB with nonspecific ST-T wave changes. Coronary angiogram revealed no atherosclerotic disease. The left coronary artery was found to originate from the right coronary cusp. Cardiac CAT scan revealed similar findings with interarterial and intramural course. Patient received one-vessel arterial bypass graft to her anomalous coronary vessel along with a defibrillator for secondary prevention. Sudden cardiac arrest secondary to congenital anomalous coronary artery disease is characterized by insufficient coronary flow by the anomalous left coronary artery to meet elevated left ventricular (LV) myocardial demand. High risk defects include those involved with the proximal coronary artery or coursing of the anomalous artery between the aorta and pulmonary trunk. Per guidelines, our patient received one vessel bypass graft to her anomalous vessel. It is important for clinicians to recognize such presentations of anomalous coronary artery.
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Al-Umairi, Rashid S., Faiza Al-Kindi, and Saqar Al-Tai. "Prevalence and Spectrum of Coronary Anomalies Detected on Coronary Computed Tomography Angiography: A single centre experience in Oman." Sultan Qaboos University Medical Journal [SQUMJ] 19, no. 2 (September 8, 2019): 108. http://dx.doi.org/10.18295/squmj.2019.19.02.005.
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ABSTRACT: Objectives: Coronary artery anomalies (CAAs) are uncommon congenital abnormalities with a prevalence ranging from 0.2–2%. CAAs can be asymptomatic or less commonly present with life-threatening symptoms. This study aimed to investigate the prevalence and spectrum of CAAs in patients who underwent coronary computed tomography angiography (CCTA) in Oman. Methods: This retrospective study was conducted at the National Heart Centre, Muscat, Oman between September 2012 and August 2018. All consecutive patients who had undergone CCTA were included. Results: A total of 4,445 patients were included in this study. Of these, 59 patients (1.3%) were diagnosed with CAAs with a mean age of 52.6 years (range: 12–80 years) and an equal gender distribution. Among the patients with CAAs, the majority (69.5%) had anomalous origins from the opposite or non-coronary sinus. Right coronary artery arising from the left coronary sinus was the most common type (33.9%). Fewer patients (18.6%) had left circumflex arising from the right coronary sinus (RCS). Seven patients (11.9%) had left main arising from the RCS. Other CAAs were in the dual left anterior descending artery (8.5%), high coronary artery take-off (6.8%), single coronary ostia (6.8%) and coronary artery fistula (6.8%). Conclusion: The prevalence of CAAs was 1.3% which is similar to the literature.Keywords: Coronary Vessel Anomalies; Computed Tomography Angiography; Prevalence; Oman.
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Dyamenahalli, Umesh, Brian D. Hanna, and Geoffrey P. Sharratt. "Pulmonary atresia with intact ventricular septum: management of the coronary arterial anomalies." Cardiology in the Young 7, no. 1 (January 1997): 80–87. http://dx.doi.org/10.1017/s1047951100005916.
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AbstractOver a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.
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Wikenheiser, Jamie, Julie A. Wolfram, Madhusudhana Gargesha, Ke Yang, Ganga Karunamuni, David L. Wilson, Gregg L. Semenza, et al. "Altered hypoxia-inducible factor-1 alpha expression levels correlate with coronary vessel anomalies." Developmental Dynamics 238, no. 10 (October 2009): 2688–700. http://dx.doi.org/10.1002/dvdy.22089.
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Chen, Tao, Weihao Xu, Yulun Cai, Qi Wang, Jun Guo, and Yundai Chen. "Safety and Efficacy of Guidezilla Extension Catheter for the Percutaneous Treatment of Complex Coronary Lesions." Heart Surgery Forum 23, no. 2 (March 17, 2020): E147—E150. http://dx.doi.org/10.1532/hsf.2709.
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Background: The GuidezillaTM support extension catheter is designed to provide extra back-up support and efficient device delivery during complex percutaneous coronary interventions (PCIs), such as in treatment of severe calcification, tortuous chronic total occlusions (CTOs), and coronary anomalies. The aim of this study was to describe our initial experience with the GuidezillaTM extension catheter in the treatment of complex coronary artery lesions.
Methods: This study retrospectively analyzed data from 165 PCI cases that used the GuidezillaTM guide extension catheter between March 2015 and August 2017. We collected patient clinical characteristics, target lesion characteristics, and procedural details.
Results: Eighty-six percent of patients had complex Type C lesions, and 13.9% had Type B lesions. Lesion length ranged from 8 mm to 130 mm (≤ 20 mm, 15.4%; 20–40 mm, 35.8%; > 40 mm, 49.1%). The right coronary artery (59.2%) was the most common intervention vessel followed by the left ascending artery (30.6%) and the left circumflex artery (10.2%). CTO accounted for 38% of all lesions, followed by distortions (28%), heavy calcification (24%), proximal stent thrombosis (9%), and coronary artery origin anomalies (1%). A total of 142 patients underwent successful PCI using the GuidezillaTM extension catheter. The success rate was 86%.
Conclusion: The GuidezillaTM guide extension catheter was an effective and safe technique in the transradial treatment of complex coronary lesions. Use of the GuidezillaTM guide extension catheter can shorten the procedure time and ensure overall procedural success with a reduced complication rate in cases where adequate progress using angioplasty devices has not been achieved.
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Stimpel, Bernhard, Jens Wetzl, Christoph Forman, Michaela Schmidt, Andreas Maier, and Mathias Unberath. "Automated Curved and Multiplanar Reformation for Screening of the Proximal Coronary Arteries in MR Angiography." Journal of Imaging 4, no. 11 (October 23, 2018): 124. http://dx.doi.org/10.3390/jimaging4110124.
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Congenital anomalies of the coronary ostia can lead to sudden death. A screening solution would be useful to prevent adverse outcomes for the affected individuals. To be considered for integration into clinical routine, such a procedure must meet strict constraints in terms of invasiveness, time and user interaction. Imaging must be fast and seamlessly integrable into the clinical process. Non-contrast enhanced coronary magnetic resonance angiography (MRA) is well suited for this. Furthermore, planar reformations proved effective to reduce the acquired volumetric datasets to 2D images. These usually require time consuming user interaction, though. To fulfill the aforementioned challenges, we present a fully automated solution for imaging and reformatting of the proximal coronary arteries which enables rapid screening of these. The proposed pipeline consists of: (I) highly accelerated single breath-hold MRA data acquisition, (II) coronary ostia detection and vessel centerline extraction, and (III) curved planar reformation of the proximal coronary arteries, as well as multiplanar reformation of the coronary ostia. The procedure proved robust and effective in ten volunteer data sets. Imaging of the proximal coronary arteries took 24 ± 5 s and was successful within one breath-hold for all patients. The extracted centerlines achieve an overlap of 0.76 ± 0.18 compared to the reference standard and the average distance of the centerline points from the spherical surface for reformation was 1.1 ± 0.51 mm. The promising results encourage further experiments on patient data, particularly in coronary ostia anomaly screening.
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Davis, Deborah A., John A. Tucker, and Pierantonio Russo. "Management of Airway Obstruction in Patients with Congenital Heart Defects." Annals of Otology, Rhinology & Laryngology 102, no. 3 (March 1993): 163–66. http://dx.doi.org/10.1177/000348949310200301.
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Airway obstruction may complicate the course of infants undergoing repair of congenital heart disease. Airway obstruction was encountered in seven patients following surgery for complex congenital heart defects (two with interrupted aortic arch, one with pulmonary atresia and a ventricular septal defect, one with tetralogy of Fallot and an abnormal left anterior descending coronary artery, one with truncus arteriosus, and two with complex univentricular heart). In four patients, a conduit was implanted at the time of surgery. In all cases, bronchoscopy accurately demonstrated the cause of the airway obstruction. Two infants had hypoplasia of the left bronchus, and five had tracheobronchomalacia associated with extrinsic compression of the airway by a great vessel or conduit. All were treated conservatively with therapeutic bronchoscopy, tracheotomy, and/or stenting with prolonged mechanical ventilation. Follow-up ranged from 2 to 40 months. Four infants have been weaned from mechanical ventilation and decannulated; two are awaiting decannulation; and one was decannulated and died awaiting surgical repair. To reduce morbidity in infants undergoing surgery for congenital heart disease, the presence of preoperative airway anomalies should be sought and surgery planned to avoid airway compression. Conservative management using diagnostic and therapeutic bronchoscopy, tracheotomy, and stenting together with prolonged ventilator care is rewarding.
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Gonzalez Bonilla, Hilda M., Nitheesha Ganta, Ahmad A. Awan, and Isaac Opoku Asare. "Abstract 15360: Coronary Artery Dominance, Anatomical Variants and Anomalies in African American Population." Circulation 142, Suppl_3 (November 17, 2020). http://dx.doi.org/10.1161/circ.142.suppl_3.15360.
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Introduction: Coronary arterial dominance is defined by the vessel which gives rise to the posterior descending artery (PDA). The prevalence of right dominance is 80- 85%, left dominance is 8% and codominance is 7% among the general population. The coronary dominance in African Americans is not well known. The aim of this study was to investigate the coronary dominance in African Americans. Previous studies showed that left dominance and codominance are associated with increased post-percutaneous coronary intervention (PCI) in-hospital mortality in patients with acute coronary syndrome (ACS). Methods: A retrospective analysis of coronary angiograms performed in our facility was done. A total of 556 cases were reviewed from 2018 to 2019. All the data was collected from the electronic medical records. Patients were categorized by race, gender, age, and comorbidities. Angiograms were reviewed and coronary dominance was determined by the vessel which supplied the PDA. Results: From the 556 charts reviewed, 430(77%) cases were performed in African Americans. Of the 430 patients, 220(51%) were male and 210(49%) were female. Mean age was 62.6 years. 370(86%) patients had hypertension, 171(39.7%) had diabetes, 28(6.5%) had peripheral vascular disease, 149(34.6%) had heart failure, 33(7.6%) had history of stroke. 28(6.5%) were on dialysis, 201(46.7%) had dyslipidemia, 14(3.2%) had HIV, 43 (10%) had atrial arrhythmias, 237(55.1%) had history of tobacco use and 21(4.8%) had history of cocaine use. In 390(90.7%) patients, left main coronary artery bifurcated into left anterior descending coronary artery (LAD) and left circumflex artery (LCX). In 40(9.3%) patients, left main coronary artery trifurcated into LAD, LCX and ramus intermedius artery. Among these patients, 303(70.4%) had right coronary dominance, 38(8.8%) had left coronary dominance and 89(20.7%) had codominance. 7 patients had ectasia of the coronary vessels and 8 patients had anomalous origin of coronary vessels. Conclusions: Our study showed that African Americans have higher prevalence of codominance compared to general population. It is important to identify it at the time of coronary angiogram since it has been associated with increased post PCI in-hospital mortality in patients with ACS.
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Qasim, Amna, Duraisamy Balaguru, and Ashraf M. Aly. "Post mortem coronary angiography in a preemie heart – a case report." Journal of Congenital Cardiology 3, no. 1 (December 2019). http://dx.doi.org/10.1186/s40949-019-0029-2.
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Abstract Background Postmortem coronary angiography has been used in forensic medicine for several decades but its use has never been documented in neonatal hearts. The objective of this case is to report the use of postmortem coronary angiography as a diagnostic modality for neonates suspected to have complex congenital heart anomalies. Case presentation A 36-week-old female infant required extracorporeal membranous oxygenation for persistent hypotension on day 1 of life. A congenital echocardiogram (ECHO) on day 3 of life revealed multiple anomalous vascular structures within the interventricular septum. The infant passed away on day 4 of life after the parents elected to withdraw support. A consent for autopsy was taken from the parents and a postmortem coronary angiography was performed. The coronary vessels were injected with Iodixanol contrast via a 24 G angiocath under fluoroscopy. The anomalous septal vessels were identified as dilated coronary artery and vein. No other anomalies were identified. Conclusion Postmortem coronary angiography complements other imaging procedures in understanding the nature of some complex congenital heart defects and in determining the cause of death in such neonates.
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Koppel, C., M. R. M. Jongbloed, P. Kies, M. G. Hazekamp, M. J. Schalij, and H. W. Vliegen. "P1797Prevalence of coronary anomalies in tetralogy of Fallot and its clinical implications, a meta-analysis." European Heart Journal 40, Supplement_1 (October 1, 2019). http://dx.doi.org/10.1093/eurheartj/ehz748.0549.
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Abstract Background In literature, anomalous coronary arteries from the opposite sinus of Valsalva or opposite coronary artery (ACAOS) are reported between 2% to 39% of patients with Tetralogy of Fallot (TOF). Knowledge of coronary anatomy prior to corrective surgery is vital to avoid damage to vessels crossing the right ventricular outflow tract (RVOT). The current range of reported prevalences is broad and a general overview comparing current knowledge on anomalous coronary arteries in TOF is lacking to date. Purpose In this meta-analysis, we aim to provide a detailed overview of current knowledge on prevalence of coronary anomalies in TOF and discuss the implications for patient management. Methods PubMed, Embase and Web of Science were searched for articles on TOF and coronary anomalies. Analysis was done using Revman 5.3 (Cochrane Community, London). The primary analysis focused on the origin and proximal course of the right and left coronary arteries. Also, the prevalences of large conus arteries and coronary arteriovenous fistulas were calculated. Results Twenty-nine studies, comprising 6977 patients all together, were included for primary meta-analysis of ACAOS. 6% of TOF patients have an ACAOS. Of these anomalous vessels, 72% crosses the RVOT. 6% of patients have a large conus artery and 4% a coronary arteriovenous fistula. Other incidentally reported coronary anomalies in TOF include a left or right coronary artery originating from the pulmonary artery, an accessory left anterior descending artery, hypoplasia of the entire coronary tree and anastomoses between coronary and bronchial arteries. CT-angiography is the imaging modality of preference because of its high spatial resolution. Transthoracic echocardiography can be used in younger children as well for discerning the coronary anatomy. Most surgical approaches can be adapted to an anomalous coronary artery coursing over the RVOT. Overall prevalence of ACAOS in TOF Conclusions Coronary anomalies have a high prevalence in TOF. An ACAOS occurs in 6%, large conus arteries exist in 6% and coronary arteriovenous fistulas in 4% of cases. A substantial part crosses the RVOT. This has to be taken into account during surgery.
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Singh, Gurkirat, Hemant Khemani, Shakil Shaikh, and Narender Omprakash Bansal. "Successful PCI of Anomalous Left Circumflex Coronary Artery Arising from Right Coronary Sinus – A Case Report." Cardiology and Angiology: An International Journal, April 26, 2019, 1–4. http://dx.doi.org/10.9734/ca/2019/v8i230096.
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Coronary artery anomalies occur in 1.3-5.6% of patients undergoing coronary arteriography. An anomalous origin of LCX from right coronary sinus is the most common congenital variant. It is usually considered “benign” since it is not known to predispose individuals to sudden cardiac death. Such vessels are particularly predisposed to atherosclerotic disease in their proximal portion, due to the acute angulation of its origin from the aorta and its posterior retro aortic course. We present a case of 55 years old female admitted with acute coronary syndrome. Coronary angiogram showed the anomalous origin of the left circumflex artery from right coronary sinus. This artery had a significant lesion which was successfully stented with a drug-eluting stent.
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Wang, Yu, Ying Zhang, and Meilian Wang. "Prenatal diagnosis of an aberrant ductus venosus draining into the coronary sinus using two- and three-dimensional echocardiography: a case report." BMC Pregnancy and Childbirth 21, no. 1 (May 20, 2021). http://dx.doi.org/10.1186/s12884-021-03870-x.
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Abstract Background Ductus venosus (DV) abnormalities may be associated with intracardiac or extracardiac deformities, chromosomal anomalies, and/or congestive heart failure. Aberrant DV connecting with the coronary sinus (CS) is rare and the prenatal diagnosis presents challenges for most examiners. Case presentation A 35-year-old pregnant woman, gravida 2, para 1, was referred to our center at 27 gestational weeks for a full evaluation of fetal cardiac anomalies. Transverse scans indicated normal cardiac anatomy except for a dilated CS; we then scanned sagittal planes to clarify the reasons for the CS dilatation. High-definition flow imaging (HDFI) together with radiant flow (R-flow) imaging was used to delineate the aberrant DV returning to the CS, enabling the diagnosis. Three-dimensional (3D) technology was also used to obtain color-rendered images showing the spatial relationships of the vessels involved, thus confirming the two-dimensional (2D) diagnosis. Chromosomal analysis revealed a normal karyotype. The neonate appeared healthy and the echocardiogram showed a normal cardiac anatomy except for a dilated CS with the DV closed and imperceptible. Conclusions The aberrant course of the DV returning to the CS was clearly demonstrable by traditional 2D echocardiography using HDFI and the R-flow technique. We deem it helpful to trace the inflow of the dilated CS to make the differential diagnosis. The 3D modality might also provide additional spatial information on the associated vessels and thereby assist in prenatal diagnosis.
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Soleimantabar, Hussein, Sofia Sabouri, Leila Khedmat, Samira Salajeghe, Behzad Memari, and Bahareh Heshmat Ghahderijani. "Assessment of CT angiographic findings in comparison with echocardiography findings of chest among patients with aortic arch anomalies." Monaldi Archives for Chest Disease 89, no. 3 (October 1, 2019). http://dx.doi.org/10.4081/monaldi.2019.1120.
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The objective of this study was to examine the findings of computed tomographic (CT) angiography in patients with aortic arch anomalies in comparison with transthoracic echocardiography findings who referred to a private imaging center in Tehran during 2009-2012. The cases included 203 patients with clinical symptoms or echocardiogram of congenital heart disease to assess the presence of aortic arch anomalies among patients referred to imaging center. This study is a retrospective study based on the CT angiographic findings in comparison with transthoracic echocardiography findings of chest among patients with aortic arch anomalies. In this study, 203 patients with congenital anomalies were enrolled in the study, among those, 107 patients were men and 96 were female. The most common anomaly of the aortic arch was found to be coarctation (19.7%), followed by right sided arch with mirror image branching (19.2%). Furthermore, the most common cardiac anomalies associated with aortic arch anomalies were VSD, PA and PDA. The sensitivity and specificity of transthoracic echocardiography in the diagnosis of aortic arch anomalies was 59% and 100% in comparison with CT angiography. In addition, the agreement between the two methods (kappa) in the diagnosis of aortic arch anomalies was 0.72. But, transthoracic echocardiography is the first diagnostic method for patients with congenital heart disease. In some patients, the ability of this method was limited to the detection of coronary artery anomalies and thoracic vessels. Therefore, CT is used for morphological evaluation of congenital heart disease (CHD) due to its main advantages including fast acquisition time, large anatomical coverage, high speed, and great spatial resolution. Moreover, CT is essential for proper evaluation of CHD regarding its high spatial and temporal resolution.
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Elshimy, Ahmed, Rasha Tolba Khattab, and Hend Galal Eldeen Mohamed Ali Hassan. "The role of MDCT in the assessment of cardiac and extra-cardiac vascular defects among Egyptian children with tetralogy of Fallot and its surgical implementation." Egyptian Journal of Radiology and Nuclear Medicine 52, no. 1 (February 8, 2021). http://dx.doi.org/10.1186/s43055-021-00426-z.
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Abstract Background Tetralogy of Fallot (TOF) is considered the most common form of cyanotic congenital heart diseases (CHD), accounting for about 10% of cases. It includes four main cardiac defects, in addition to various extra-cardiac anomalies. Aim This study aimed to evaluate cardiac and extra-cardiac vascular defects associated with TOF among Egyptian children, regarding frequency and types with assessment of multi-slice or multi-detector computed tomography (MDCT) role in their diagnosis. Definitely, full detection of these vascular anomalies has utmost importance when evaluating such patients particularly before surgical intervention. Methods This study included 60 pediatric patients diagnosed as TOF, who underwent MDCT examination in our institute during period of 6 months from (March to September 2020), to confirm their trans-thoracic echocardiography (TTE) findings and detect other vascular abnormalities which cannot be precisely detected with TTE before their surgical interventions. Results The incidence of different extra-cardiac vascular defects diagnosed by MDCT among our patients was 85% which was significantly higher than that detected by TTE (55%). Moreover, MDCT was superior to TTE assessment as regards its diagnostic accuracy (96.6% vs. 80%), sensitivity (98% vs. 76.9%), and specificity (88.9% vs. 85.7%), in addition to both positive and negative predictive values. The most common anomalies detected were affecting the pulmonary artery (80%), followed by aorto-pulmonary vessels (45%), then aortic artery (40%), coronary arteries (20%), and lastly vena cava connection (6.7%). Patients’ demographic characteristics and clinical presentations were also presented. Conclusion This study confirmed that many extra-cardiac vascular defects are commonly associating cardiac lesions in TOF and emphasizing the great value of MDCT in their diagnosis. Certainly, proper detection of these anomalies will help decision-making during preoperative evaluation, corrective interventions, and further management of these cases.
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Bigler, Marius Reto, Afreed Ashraf, Christian Seiler, Fabien Praz, Yasushi Ueki, Stephan Windecker, Alexander Kadner, Lorenz Räber, and Christoph Gräni. "Hemodynamic Relevance of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva-In Search of the Evidence." Frontiers in Cardiovascular Medicine 7 (January 21, 2021). http://dx.doi.org/10.3389/fcvm.2020.591326.
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Coronary artery anomalies (CAA) represent a heterogeneous group of congenital disorders of the arterial coronary circulation, defined by an anomalous origin of the coronary ostium and/or vessel course. Of particular interest are anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). The interarterial variants (with the anomalous vessel situated between the great arteries) are historically called “malignant,” based on an anticipated higher risk for myocardial ischemia and sudden cardiac death (SCD), especially affecting young patients during strenuous physical activity. However, the interarterial course itself may not be the predominant cause of ischemia, but rather represents a surrogate for other ischemia-associated anatomical high-risk features. As the exact pathophysiology of ACAOS is not well-understood, there is a lack of evidence-based guidelines addressing optimal diagnostic work-up, downstream testing, sports counseling, and therapeutic options in patients with ACAOS. Therefore, treating physicians are often left with uncertainty regarding the clinical management of affected patients. This review focuses on the pathophysiologic consequences of ACAOS on myocardial ischemia and discusses the concept of the interplay between fixed and dynamic coronary stenosis. Further, we discuss the advantages and limitations of the different diagnostic modalities and give an outlook by highlighting the gaps of knowledge in the assessment of such anomalies.
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Nadadur, Srinivas, Justice Oranefo, Muhammad Adeel, Mansour Khaddr, and Wassim Mosleh. "Abstract 16067: Unique Challenge of Acs - Anomalous Left Main Coronary Artery From Right Coronary Cusp." Circulation 142, Suppl_3 (November 17, 2020). http://dx.doi.org/10.1161/circ.142.suppl_3.16067.
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Introduction: Anomalous aortic origin of coronary arteries (AAOCA) is uncommon congenital anomalies of the coronary circulation. Anomalous left main coronary artery (LMCA) originating from the right cusp is one of the rarer anomalies (incidence ~ 0.03% of patients undergoing coronary angiography). Mostly asymptomatic, however, this may become clinically significant with symptoms ranging from atypical chest discomfort to sudden cardiac death. We present a case of anomalous origin LMCA from the right coronary cusp. Case presentation: A 47-year-old female presented to the ED with three days of substernal chest pressure at rest. BP 106/71 mmHg, pulse 72 bpm, normal regular heart sounds, and clear lungs. The ECG showed new anterolateral T-wave inversions. Troponin-I was 0.31 ng/ mL. Echocardiogram showed normal LVEF without focal wall motion abnormalities. Coronary angiography revealed a dominant patent RCA without any disease. LMCA originated from the right coronary cusp with focal 90% ostial LAD stenosis. The rest of the coronary tree was free of disease. CABG with LIMA to LAD and SVG to OM1 was performed. Discussion: AAOCA presents a unique challenge to diagnosis and management. CT coronary angiogram or magnetic resonance angiography is recommended for more accurate delineation of the course of the coronary vessel. The diagnostic challenge to effectively engage the anomalous artery and to obtain coronary angiogram to delineate anatomy is critical in further management. As seen in our case, this is easy to overlook and in the acute event could lead to unnecessary delay.
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M Kurniawan, Isyana, Elen Elen, Celly A Atmadikoesoemah, and Manoefris Kasim. "Right Anomalous Coronary Artery Origin: The Role of Multislice CT Angiography." Indonesian Journal of Cardiology, May 2, 2016, 274–7. http://dx.doi.org/10.30701/ijc.v35i4.495.
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Introduction: The anomalous origin of the right coronary artery (RCA) from the left coronary sinus coursing between the aorta and the pulmonary trunk is rare, but may cause myocardial ischemia and sudden death. Multislice CT coronaryangiography offers the possibility to visualize anomalous coronary artery origin non-invasively in details.Case Illustration: A 54-year-old man with a history of arterial hypertension, and hypercholesterolemia began to present with typical chest pain. After some non-invasive examination, he had coronary angiographythat revealed 70% stenosis at mid intermediate artery, normalLMCA, LAD and LCX. Ostium of RCA was found near the left valsava sinus afterrepeated cannulation attempts, no stenosis was found at RCA. After successful revascularization at intermediate artery, patient still had typical chest pain with positive ischemic response in treadmill test. Multislice CT coronary angiography was performedto evaluate the etiology of chest pain. The scan showed patent stent at intermediate artery and anomalous RCA origin from the left coronary sinus withacute angle take-off, luminal narrowing of the osteal-proximal part, as well as luminal compression between the ascending aorta and the pulmonary trunk (an interarterial course) while RCA appeared as dominant vessel. These features were considered as malignant coronary anomaly that could lead to the recommendation of surgical correction.Discussion: Accurate recognition and documentation of coronary artery anomalies are essential to determine the significance of such findings and to avoid furtherclinical complications. Multislice CT coronary angiography is a non-invasive imaging modality that can easily and precisely depict the origin and course of coronary artery anomalies as well as its relationship with adjacent structures.
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Elagha, Abdalla, Waleed Khaled, Sahar Gamal, Mohamed Helmy, and Ayman Kaddah. "Coronary computed tomography versus coronary angiography for preoperative coronary assessment before valve surgery." Egyptian Heart Journal 73, no. 1 (July 5, 2021). http://dx.doi.org/10.1186/s43044-021-00180-7.
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Abstract Background Conventional coronary angiography (CAG) is currently the gold standard technique for the assessment of coronary arteries prior to cardiac valve surgery. Although CAG is a relatively safe procedure, however, it is still an invasive procedure, and it has potential hazards and complications. Coronary computed tomography angiography (CCTA) is a non-invasive technique that has emerged robustly as an excellent and attractive tool for delineating coronary anatomy. Therefore, we sought to evaluate the accuracy of CCTA when compared with the gold standard CAG in the evaluation of coronary arteries before valve surgery. We screened 111 consecutive patients with VHD undergoing a routine cardiac catheterization for preoperative evaluation of CAD. Fifty patients were eligible and underwent both CAG and CCTA. Significant coronary stenosis was defined as a luminal diameter decrease of ≥ 50%. Additionally, ectasia, calcifications, and congenital coronary anomalies were analyzed. Also, we compared both techniques regarding radiation dose, contrast volume, and complications. Non-evaluable segments were excluded from all levels of analysis. Sixty-one patients were excluded from the study due to various reasons. Results Among the 50 patients of the study population, 27 (54%) were males. The prevalence of significant CAD in the study population was 19.6% according to the patient-based analysis, and CAG could have been avoided in 80.4% of patients with a true-negative CCTA result. Diagnostic accuracy of CCTA for detection of significant stenosis was evaluated regarding sensitivity and specificity, positive predictive value, negative predictive value, and overall accuracy of CCTA, which was 87.5%, 99.6%,87.5%, 99.6%, and 99.2%, respectively, for segmental-based analysis; 86%, 100%, 100%, 99%, and 99%, respectively, for vessel-based analysis; and 77.8%,100%,100%, 94.9%, and 95.7%, respectively, for patient-based analysis. Fewer rates of complications were encountered with CCTA. Additional information obtained like calcifications and congenital anomalies was diagnosed better with CCTA than CAG. Conclusion Owing mainly to its high negative predictive value, a well-performed CCTA exam is an excellent method to rule out coronary artery disease, specially in patients who are not at high risk of atherosclerosis.
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Mehlman, Andrew, Jaymin Patel, Christopher Bitetzakis, and Michael Berlowitz. "Multi-vessel coronary artery aneurysms in a patient with Parry Romberg syndrome: a case report." European Heart Journal - Case Reports 3, no. 3 (July 4, 2019). http://dx.doi.org/10.1093/ehjcr/ytz103.
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Abstract Background Coronary artery aneurysms (CAAs) are a very rare finding on coronary angiograms with multiple known aetiologies. Parry Romberg syndrome (PRS) is also a very rare disease, and the underlying aetiology remains unknown. We present a rare case of CAAs in a patient with PRS, and discuss possible implications regarding the primary pathophysiological cause for both of these diseases. Case summary A 48-year-old woman with a history of PRS presented with atypical and non-exertional chest pain. Initial evaluation demonstrated a rising troponin without associated electrocardiogram changes, and as such she was taken for left heart catheterization. Left heart catheterization demonstrated diffuse aneurysmal and ectatic disease of multiple coronary arteries. Further evaluation with magnetic resonance angiogram and autoantibody panel did not demonstrate other vascular anomalies or rheumatologic disease, respectively. She was treated with dual anti-platelet therapy and statin, and at 1 year follow-up, she had resolution of her symptoms. Discussion It has been postulated that the underlying mechanism causing CAA is intravascular inflammation. Parry Romberg syndrome is theorized to be a neurovasculopathy, as evidenced by cases of associated intracranial aneurysms. Intravascular inflammation may play a key pathological role in CAA, and an association between CAA and PRS may exist.
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Bhargav, Anish, Parminder S. Otaal, and Manphool K. Singhal. "Type X dual left anterior descending (LAD) artery masquerading as type 1 LAD — a case report." Egyptian Journal of Internal Medicine 33, no. 1 (July 22, 2021). http://dx.doi.org/10.1186/s43162-021-00053-0.
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Abstract Background Dual left anterior descending (LAD) coronary artery is a rare congenital anomaly. To date, eleven variants of dual LAD have been described with three published reports of type X dual LAD. Here, we describe a new variant of type X dual LAD with a short LAD artery masquerading as type 1 LAD. Case presentation A 42-year hypertensive female presented with recent onset angina with a treadmill test positive for inducible ischemia. Coronary angiography showed a normal right coronary artery (RCA). The left main coronary artery (LMCA) originated from the left sinus of Valsalva (SOV), giving rise to a LAD and the left circumflex artery (LCX). Appearing a normal angiogram with type 1 LAD based on its length, the presence of a large bare area in LAD territory (especially at the apex) and lack of septal branches prompted a search for an additional vessel. Right SOV injection showed a vessel originating separately from RCA, which was confirmed to be a long LAD on selective injection, with a pre-pulmonic course and giving rise to septal branches exclusively before wrapping around the apex. Computed tomography coronary angiography (CTCA) confirmed the pre-pulmonic course of long LAD, defined its entry to the distal interventricular septum to the right of short LAD, and ruled out other coronary artery anomalies. In the absence of a stenotic lesion in the epicardial coronaries, angina in our case was presumed to be due to microvascular dysfunction. She was discharged on beta-blocker therapy for co-existing hypertension and is asymptomatic on follow-up at one year. Conclusions A short LAD artery of type X Dual LAD could be potentially misdiagnosed as type 1 LAD based on its length. However, an active search for a long LAD could properly diagnose the case as a variant of type X dual LAD, which has important clinical implications. Its awareness is critical for cardiologists and cardiac surgeons to correctly interpret the coronary angiogram and plan proper management.
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Doll, Cassandra F., Natalia J. Pereira, Mustafa S. Hashimi, Tabor J. Grindrod, Fariz F. Alkassis, Lawrence X. Cai, Una Milovanovic, Adriana I. Sandino, and Hideko Kasahara. "Gestational intermittent hyperoxia rescues murine genetic congenital heart disease in part." Scientific Reports 11, no. 1 (March 23, 2021). http://dx.doi.org/10.1038/s41598-021-85569-9.
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AbstractCardiac development is a dynamic process, temporally and spatially. When disturbed, it leads to congenital cardiac anomalies that affect approximately 1% of live births. Genetic variants in several loci lead to anomalies, with the transcription factor NKX2-5 being one of the largest. However, there are also non-genetic factors that influence cardiac malformations. We examined the hypothesis that hyperoxia may be beneficial and can rescue genetic cardiac anomalies induced by an Nkx2-5 mutation. Intermittent mild hyperoxia (40% PO2) was applied for 10 h per day to normal wild-type female mice mated with heterozygous Nkx2-5 mutant males from gestational day 8.5 to birth. Hyperoxia therapy reduced excessive trabeculation in Nkx2-5 mutant mice compared to normoxic conditions (ratio of trabecular layer relative to compact layer area, normoxia 1.84 ± 0.07 vs. hyperoxia 1.51 ± 0.04) and frequency of muscular ventricular septal defects per heart (1.53 ± 0.32 vs. 0.68 ± 0.15); however, the incidence of membranous ventricular septal defects in Nkx2-5 mutant hearts was not changed. Nkx2-5 mutant embryonic hearts showed defective coronary vessel organization, which was improved by intermittent mild hyperoxia. The results of our study showed that mild gestational hyperoxia therapy rescued genetic cardiac malformation induced by Nkx2-5 mutation in part.
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Cardoso Torres, S., M. Vasconcelos, C. X. Resende, P. Diogo, R. Pinto, T. Proenca, J. M. Carvalho, et al. "Coronary artery fistulas: a single-center case series." European Heart Journal 41, Supplement_2 (November 1, 2020). http://dx.doi.org/10.1093/ehjci/ehaa946.1526.
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Abstract Introduction Coronary artery fistulas (CAFs) are rare anomalous connections between a coronary artery and a major vessel or cardiac chamber. Currently they are being increasingly encountered due to the more widespread use of various imaging modalities and coronary angiography. Although the vast majority of CAFs are incidentally diagnosed and have no clinical relevance, they can cause significant morbidity such as myocardial infarction, congestive heart failure and endocarditis. Methods A consecutive series of 55867 coronary arteriograms performed in our Cardiology Department from 2007 to 2019 was retrospectively investigated for the presence of coronary artery fistulas. Patients clinical, angiographic and therapeutic data up to november 2019 were analyzed. Data were obtained from medical records of hospital stay and subsequent consultations. Results We identified 50 patients who were diagnosed with one or more CAFs, with ages between 5 and 85 years (mean 59 years). 62% (n=31) were males. The great majority of patients had a single fistula (n=34, 68%), 11 patients had two fistulas (22%), 1 patient had 3 fistulas (2%) and 4 patients had multiple fistulas (8%). CAFs arose more frequently from the left anterior descending artery (n=27), followed by the right coronary (n=18), left circumflex (n=15), left main (n=5) and intermediate artery (n=2). The most frequent drainage site was the pulmonary artery (n=38). The majority of CAFs were incidentally found (n=32; 64%) and thought to have no significance for the patients' clinical status. As for the rest of the patients, CAFs were diagnosed during evaluation of: a heart murmur (n=7); exertional chest pain with no associated significant atherosclerotic coronary artery disease (n=7); exertional dyspnea (n=2); positive exercise stress test (n=1); NSTEMI and cardiac arrest (n=1). Regarding treatment, watchful waiting was the main approach (n=40; 80%). 3 patients had their CAFs closed during surgery for another heart condition (CABG/aortic valve replacement). In 1 patient, heart surgery was specifically conducted for fistula closure. 6 patients (12%) underwent fistula transcatheter closure. Conclusion CAFs are rare coronary anomalies and the majority has no clinical relevance, so watchful waiting is the commonest approach. When they are hemodynamically significant or symptoms/complications arise, surgical or transcatheter closure should be considered. This study describes the angiographic, clinical and therapeutic data of CAFs detected along the last 12 years in a single tertiary care center catheterization laboratory. Funding Acknowledgement Type of funding source: None
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Fady, G., A. M. Komaranchath, F. B. Al Bakshy, and M. G. Gibreel. "P243 A rare association of isolated persistent left superior vena cava(PLSVC) with anomalous right coronary artery (RCA) in a symptomatic patient. Role of saline contrast echo (SCE) with multimodal imaging." European Heart Journal - Cardiovascular Imaging 21, Supplement_1 (January 1, 2020). http://dx.doi.org/10.1093/ehjci/jez319.106.
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Abstract Funding Acknowledgements Nil Introduction PLSVC with absent right superior vena cava (RSVC) is an extremely rare venous anomaly. Most of the patients with this anomaly have other associated cardiac anomalies. However, presence of an anomalous RCA has not been one of these anomalies. Case report A 36-year-old Asian healthy female with no significant medical history presented with over 3 years symptoms of chest heaviness and shortness of breath on walking two blocks. Examination was unremarkable. 2D echocardiogram revealed a 36mm dilated coronary sinus (CS), grade II tricuspid regurgitation (TR) with estimated right ventricular systolic pressure (RVSP) of 40 mmhg. Normal right and left side of the heart. SCE using 50:50 agitated saline and Gelofusine injected intravenously through the left arm showed opacification of the dilated CS first before opacifying the right atrium (RA). Right arm injection depicted the same pattern of contrast opacification which denotes presence of PLSVC with congenital absence of RSVC. No detected intracardiac shunts by SCE. No partial anomalous pulmonary venous connection (PAPVC). However, findings did not justify patient’s symptoms. Seven days loop recorder ruled out any arrhythmias that might be associated with isolated PLSVC. An exercise stress ECG was equivocal with chest discomfort but no ECG changes. We opted for CT coronary angiography (CTCA) to rule out obstructive coronary artery disease (CAD). CTCA confirmed our diagnosis of isolated PLSVC with absence of RSVC with huge CS. In addition, there was an anomalous origin of RCA from sinotubular junction above the commissure between left and right aortic CS and not from LM CS proper, taking a malignant interarterial course of a slit origin RCA with first part of it showing intramural course within the aortic wall. In view of symptoms we as a heart team explained the present risk of sudden cardiac death and possible indication for corrective surgery. A month later patient again presented with chest discomfort. We decided to do Coronary angiography to delineate actual size and dominance of RCA. RCA was a non-dominant small 1.75 mm vessel. There was a left anterior descending artery myocardial bridge. We started a beta blocker (BB) for the patient after which her symptoms improved. Patient followed up for two years later with no symptoms. We attributed her chest pain to the myocardial bridge since she improved on BB. We found there was no solid role for surgery in view of a very small non dominant RCA with no further chest pain. Conclusions Isolated PLSVC is a very rare condition. It is usually asymptomatic. But since our patient had symptoms, a search for another diagnosis was convenient. A dilated CS should always alert us to search for PLSVC. SCE is a simple, inexpensive and reproducible diagnostic tool that might help in solving a challenging diagnosis. Multimodal imaging has a leading role in both proper diagnosis and in management of this condition Abstract P243 Figure. Anomls. RCA - PLSVC to CS -Contrast echo