|
1
|
Mladenovic Markovic A, Tomic A, Nisevic M, Parapid B, Boskovic N, Vitas M, Jevtovic M, Grujicic S. The Role of MDCT Coronary Angiography in the Detection of Benign Varieties and Anomalies of Coronary Blood Vessels-A Narrative Review. MEDICINA (KAUNAS, LITHUANIA) 2025; 61:765. [PMID: 40283056 PMCID: PMC12028991 DOI: 10.3390/medicina61040765] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 03/19/2025] [Revised: 04/11/2025] [Accepted: 04/17/2025] [Indexed: 04/29/2025]
Abstract
Coronary arteries may vary in quantity, point of origin, or course. These variations fall under the category of anatomical variants/anomalies of the coronary arteries, representing congenital abnormalities of the coronary vascular system. Generally, they are benign, asymptomatic, and identified inadvertently during coronary angiography conducted for alternative indications. However, in some cases, the anomaly's characteristics or its interaction with surrounding structures may cause hemodynamic disturbances. These disturbances can lead to turbulent blood flow, which in turn poses an increased likelihood for the development of atherosclerosis and myocardial ischemia. If symptomatic, potential manifestations include chest pain, arrhythmias, syncope, myocardial infarction, and sudden cardiac death. Given the potential for life-threatening complications in certain cases, the early and accurate diagnosis of coronary artery anomalies is of paramount importance. The most common diagnostic procedures used for the evaluation of coronary vessels are coronary angiography and multi-detector row computed tomography (MDCT) coronary angiography. MDCT angiography is a non-invasive, dependable, safe, and sensitive method for the detailed visualization of coronary anatomy. It offers high-resolution imaging that enables precise assessment of congenital coronary variations, aiding in both clinical decision-making and long-term patient management. We conducted a narrative review to analyze and integrate the body of literature on coronary artery varieties and anomalies. Our objective was to provide a comprehensive, albeit non-exhaustive, overview of essential concepts and findings related to their definition, classification, and detection with MDCT angiography. By integrating current knowledge in MDCT imaging, we seek to contribute to a better understanding of the clinical implications of coronary artery variations and their role in cardiovascular health.
Collapse
Affiliation(s)
- Ana Mladenovic Markovic
- Centre for Radiology and Magnetic Resonance Imaging, University Clinical Centre of Serbia, Pasterova 2, 11000 Belgrade, Serbia; (A.M.M.); (A.T.); (M.N.); (M.J.)
- Faculty of Medicine, University of Belgrade, Dr Subotica Starijeg 8, 11000 Belgrade, Serbia;
| | - Ana Tomic
- Centre for Radiology and Magnetic Resonance Imaging, University Clinical Centre of Serbia, Pasterova 2, 11000 Belgrade, Serbia; (A.M.M.); (A.T.); (M.N.); (M.J.)
| | - Miodrag Nisevic
- Centre for Radiology and Magnetic Resonance Imaging, University Clinical Centre of Serbia, Pasterova 2, 11000 Belgrade, Serbia; (A.M.M.); (A.T.); (M.N.); (M.J.)
| | - Biljana Parapid
- Faculty of Medicine, University of Belgrade, Dr Subotica Starijeg 8, 11000 Belgrade, Serbia;
- Clinic for Cardiology, University Clinical Centre of Serbia, Pasterova 2, 11000 Belgrade, Serbia;
| | - Nikola Boskovic
- Clinic for Cardiology, University Clinical Centre of Serbia, Pasterova 2, 11000 Belgrade, Serbia;
| | - Marina Vitas
- Institute of Mother and Child Health Care of Serbia “Dr Vukan Čupić”, 11000 Belgrade, Serbia;
| | - Miona Jevtovic
- Centre for Radiology and Magnetic Resonance Imaging, University Clinical Centre of Serbia, Pasterova 2, 11000 Belgrade, Serbia; (A.M.M.); (A.T.); (M.N.); (M.J.)
| | - Sandra Grujicic
- Faculty of Medicine, University of Belgrade, Dr Subotica Starijeg 8, 11000 Belgrade, Serbia;
- Institute of Epidemiology, Faculty of Medicine, University of Belgrade, Dr Subotica Starijeg 8, 11000 Belgrade, Serbia
| |
Collapse
|
|
2
|
Lau WR, Lee PT, Koh CH. Coronary Artery Anomalies - State of the Art Review. Curr Probl Cardiol 2023; 48:101935. [PMID: 37433414 DOI: 10.1016/j.cpcardiol.2023.101935] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2023] [Accepted: 07/04/2023] [Indexed: 07/13/2023]
Abstract
Coronary artery anomalies (CAAs) comprise a wide spectrum of anatomic entities, with diverse clinical phenotypes. We present a case of an anomalous right coronary artery arising from the left aortic sinus with an interarterial course, a potentially fatal condition that can precipitate ischemia and sudden cardiac death. CAAs are increasingly detected in adults, mostly as incidental findings in the course of cardiac evaluation. This is due to the expanding use of invasive and noninvasive cardiac imaging, usually in the work-up for possible CAD. The prognostic implications of CAAs in this group of patients remain unclear. In AAOCA patients, appropriate work-up with anatomical and functional imaging should be performed for risk stratification. An individualized approach to management should be adopted, considering symptoms, age, sporting activities and the presence of high-risk anatomical features and physiologic consequences (such as ischemia, myocardial fibrosis, or cardiac arrhythmias) detected on multimodality imaging or other functional cardiac investigations. This comprehensive and up to date review seeks to crystallize current data in the recent literature, and proposes a clinical management algorithm for the clinician faced with the conundrum of managing such conditions.
Collapse
Affiliation(s)
- Wei Ren Lau
- Department of Cardiology, National Heart Centre Singapore, Singapore
| | - Phong Teck Lee
- Department of Cardiology, National Heart Centre Singapore, Singapore; Duke-NUS School of Medicine, National University of Singapore, Singapore
| | - Choong Hou Koh
- Department of Cardiology, National Heart Centre Singapore, Singapore; Duke-NUS School of Medicine, National University of Singapore, Singapore; Changi Aviation Medical Centre, Changi General Hospital, Singapore.
| |
Collapse
|
|
3
|
A Rare Report of Hypoplastic Coronary Arteries and Pulmonary Veins: A Case Report and Review of the Literature. Pediatr Cardiol 2020; 41:1231-1237. [PMID: 32361881 DOI: 10.1007/s00246-020-02334-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/09/2020] [Accepted: 02/21/2020] [Indexed: 10/24/2022]
Abstract
Congenital coronary artery anomalies are estimated to affect about 1% of the general population. Hypoplastic coronary artery disease is an uncommon subset associated with significant mortality regardless whether another major cardiac anomaly is present. In this case report, we present an extremely rare case of an infant with hypoplastic coronary artery disease and hypoplastic pulmonary veins. A literature review pertinent to the clinical findings is also contained herein.
Collapse
|
|
4
|
Engineer A, Lim YJ, Lu X, Kim MY, Norozi K, Feng Q. Sapropterin reduces coronary artery malformation in offspring of pregestational diabetes mice. Nitric Oxide 2020; 94:9-18. [PMID: 31600600 DOI: 10.1016/j.niox.2019.10.002] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2019] [Revised: 08/22/2019] [Accepted: 10/03/2019] [Indexed: 11/24/2022]
Abstract
Endothelial nitric oxide synthase (eNOS) and oxidative stress are critical to embryonic coronary artery development. Maternal diabetes increases oxidative stress and reduces eNOS activity in the fetal heart. Sapropterin (Kuvan®) is an orally active, synthetic form of tetrahydrobiopterin (BH4) and a co-factor for eNOS with antioxidant properties. The aim of the present study was to examine the effects of sapropterin on fetal coronary artery development during pregestational diabetes in mice. Diabetes was induced by streptozotocin to adult female C57BL/6 mice. Sapropterin (10 mg/kg/day) was orally administered to pregnant mice from E0.5 to E18.5. Fetal hearts were collected at E18.5 for coronary artery morphological analysis. Sapropterin treatment to diabetic dams reduced the incidence of coronary artery malformation in offspring from 50.0% to 20.6%. Decreases in coronary artery luminal diameter, volume and abundance in fetal hearts from diabetic mothers, were prevented by sapropterin treatment. Maternal diabetes reduced epicardial epithelial-to-mesenchymal transition (EMT) and expression of transcription and growth factors critical to coronary artery development including hypoxia-inducible factor 1a (Hif1a), Snail1, Slug, β-catenin, retinaldehyde dehydrogenase 2 (Aldh1a2), basic fibroblast growth factor (bFGF) and vascular endothelial group factor receptor 2 (Vegfr2) in E12.5 hearts. Additionally, eNOS phosphorylation was lower while oxidative stress was higher in E12.5 hearts from maternal diabetes. Notably, these abnormalities were all restored to normal levels after sapropterin treatment. In conclusion, sapropterin treatment increases eNOS activity, lowers oxidative stress and reduces coronary artery malformation in offspring of pregestational diabetes. Sapropterin may have therapeutic potential in preventing coronary artery malformation in maternal diabetes.
Collapse
Affiliation(s)
- Anish Engineer
- Department of Physiology and Pharmacology, London, Ontario, Canada
| | - Yong Jin Lim
- Department of Physiology and Pharmacology, London, Ontario, Canada
| | - Xiangru Lu
- Department of Physiology and Pharmacology, London, Ontario, Canada
| | - Mella Y Kim
- Department of Physiology and Pharmacology, London, Ontario, Canada
| | - Kambiz Norozi
- Children's Health Research Institute, London, Ontario, Canada; Department of Paediatrics, Western University, London, Ontario, Canada; Department of Paediatric Cardiology and Intensive Care Medicine, Medical School Hannover, Germany; Department of Paediatric Cardiology and Intensive Care Medicine, University of Goettingen, Germany
| | - Qingping Feng
- Department of Physiology and Pharmacology, London, Ontario, Canada; Department of Medicine, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada; Children's Health Research Institute, London, Ontario, Canada.
| |
Collapse
|
|
5
|
Sayit AT, Celenk C. Hypoplastic Left Circumflex Coronary Artery: Imaging Findings with Coronary Computed Tomography Angiography - A Case Report. Curr Med Imaging 2019; 15:427-429. [DOI: 10.2174/1573405614666180111151623] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2017] [Revised: 12/25/2017] [Accepted: 01/01/2018] [Indexed: 11/22/2022]
Abstract
<P>Background: Hypoplastic coronary artery disease is a rare congenital coronary artery anomaly. It is often detected incidentally, and its true incidence in the general population is not known. </P><P> Discussion: Symptoms of HCAD are syncope, palpitations, dyspnea, and chest pain. Also, arrhythmia and myocardial infarction can be seen; these can cause sudden death, especially in athletes and young people. Diagnosis is often made at autopsy.Conclusion:Here, we present the case of a 39-year-old male with isolated hypoplasia of the left circumflex artery detected by coronary Computed Tomography (CT) angiography who complained of palpitation.</P>
Collapse
Affiliation(s)
| | - Cetin Celenk
- Department of Radiology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey
| |
Collapse
|
|
6
|
Kastellanos S, Aznaouridis K, Vlachopoulos C, Tsiamis E, Oikonomou E, Tousoulis D. Overview of coronary artery variants, aberrations and anomalies. World J Cardiol 2018; 10:127-140. [PMID: 30386490 PMCID: PMC6205847 DOI: 10.4330/wjc.v10.i10.127] [Citation(s) in RCA: 52] [Impact Index Per Article: 7.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2018] [Revised: 08/12/2018] [Accepted: 08/31/2018] [Indexed: 02/06/2023] Open
Abstract
Coronary artery anomalies and variants are relatively uncommon congenital disorders of the coronary artery anatomy and constitute the second most common cause of sudden cardiac death in young competitive athletes. The rapid advancement of imaging techniques, including computed tomography, magnetic resonance imaging, intravascular ultrasound and optical coherence tomography, have provided us with a wealth of new information on the subject. Anomalous origin of a coronary artery from the contralateral sinus is the anomaly most frequently associated with sudden cardiac death, in particular if the anomalous coronary artery has a course between the aorta and the pulmonary artery. However, other coronary anomalies, like anomalous origin of the left coronary artery from the pulmonary artery, atresia of the left main stem and coronary fistulae, have also been implicated in cases of sudden cardiac death. Patients are usually asymptomatic, and in most of the cases, coronary anomalies are discovered incidentally during coronary angiography or on autopsy following sudden cardiac death. However, in some cases, symptoms like angina, syncope, heart failure and myocardial infarction may occur. The aims of this article are to present a brief overview of the diverse coronary variants and anomalies, focusing especially on anatomical features, clinical manifestations, risk of sudden cardiac death and pathophysiologic mechanism of symptoms, as well as to provide valuable information regarding diagnostic workup, follow-up, therapeutic choices and timing of surgical treatment.
Collapse
Affiliation(s)
- Stylianos Kastellanos
- Cardiology Department, Castle Hill Hospital, Hull and East Yorkshire NHS Trust, Cottingham HU16 5JQ, United Kingdom
- Peripheral Vessels Unit and EKKAN (Unit for the athletes and for hereditary cardiovascular diseases), 1 Department of Cardiology, Hippokration Hospital, Medical School of National and Kapodistrian University of Athens, Athens 11527, Greece
| | - Konstantinos Aznaouridis
- Cardiology Department, Castle Hill Hospital, Hull and East Yorkshire NHS Trust, Cottingham HU16 5JQ, United Kingdom
- Peripheral Vessels Unit and EKKAN (Unit for the athletes and for hereditary cardiovascular diseases), 1 Department of Cardiology, Hippokration Hospital, Medical School of National and Kapodistrian University of Athens, Athens 11527, Greece.
| | - Charalambos Vlachopoulos
- Peripheral Vessels Unit and EKKAN (Unit for the athletes and for hereditary cardiovascular diseases), 1 Department of Cardiology, Hippokration Hospital, Medical School of National and Kapodistrian University of Athens, Athens 11527, Greece
| | - Eleftherios Tsiamis
- Peripheral Vessels Unit and EKKAN (Unit for the athletes and for hereditary cardiovascular diseases), 1 Department of Cardiology, Hippokration Hospital, Medical School of National and Kapodistrian University of Athens, Athens 11527, Greece
| | - Evangelos Oikonomou
- Peripheral Vessels Unit and EKKAN (Unit for the athletes and for hereditary cardiovascular diseases), 1 Department of Cardiology, Hippokration Hospital, Medical School of National and Kapodistrian University of Athens, Athens 11527, Greece
| | - Dimitris Tousoulis
- Peripheral Vessels Unit and EKKAN (Unit for the athletes and for hereditary cardiovascular diseases), 1 Department of Cardiology, Hippokration Hospital, Medical School of National and Kapodistrian University of Athens, Athens 11527, Greece
| |
Collapse
|
|
7
|
Villa ADM, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery anomalies overview: The normal and the abnormal. World J Radiol 2016; 8:537-555. [PMID: 27358682 PMCID: PMC4919754 DOI: 10.4329/wjr.v8.i6.537] [Citation(s) in RCA: 202] [Impact Index Per Article: 22.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/26/2015] [Revised: 01/21/2016] [Accepted: 03/16/2016] [Indexed: 02/06/2023] Open
Abstract
The aim of this review is to give a comprehensive and concise overview of coronary embryology and normal coronary anatomy, describe common variants of normal and summarize typical patterns of anomalous coronary artery anatomy. Extensive iconography supports the text, with particular attention to images obtained in vivo using non-invasive imaging. We have divided this article into three groups, according to their frequency in the general population: Normal, normal variant and anomaly. Although congenital coronary artery anomalies are relatively uncommon, they are the second most common cause of sudden cardiac death among young athletes and therefore warrant detailed review. Based on the functional relevance of each abnormality, coronary artery anomalies can be classified as anomalies with obligatory ischemia, without ischemia or with exceptional ischemia. The clinical symptoms may include chest pain, dyspnea, palpitations, syncope, cardiomyopathy, arrhythmia, myocardial infarction and sudden cardiac death. Moreover, it is important to also identify variants and anomalies without clinical relevance in their own right as complications during surgery or angioplasty can occur.
Collapse
|
|
8
|
Riede FN, Bulla S, Grundmann S, Werner M, Riede UN, Otto C. Isolated hypoplastic circumflex coronary artery: a rare cause of haemorrhagic myocardial infarction in a young athlete. Diagn Pathol 2013; 8:91. [PMID: 23742172 PMCID: PMC3682927 DOI: 10.1186/1746-1596-8-91] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2013] [Accepted: 05/15/2013] [Indexed: 12/13/2022] Open
Abstract
Hypoplastic coronary artery disease is a rare condition that may lead to myocardial infarction and sudden death. Here we describe for the first time an isolated hypoplasia of the left circumflex artery (LCX). An otherwise healthy and athletically active 16-year-old boy was admitted to the intensive care unit (ICU) after out-of-hospital cardiac arrest. He died 12 hours after the initial event. Autopsy revealed an isolated hypoplastic LCX and acute haemorrhagic infarction in the posterolateral myocardium. The existence of isolated hypoplasia of the LCX challenges our understanding of coronary artery development. Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1558483061962648.
Collapse
Affiliation(s)
- Florian-Nikolaus Riede
- Department of Cardiology, University Hospital Basel, Petersgraben 3, CH-4031 Basel, Switzerland
| | - Stefan Bulla
- Department of Diagnostic Radiology, University Hospital Freiburg, i. Br., Hugstetterstr. 55, D-79106 Freiburg i. Br., Germany
| | - Sebastian Grundmann
- Department of Internal Medicine, University Hospital Freiburg, i. Br., Hugstetterstr. 55, D-79106 Freiburg i. Br., Germany
| | - Martin Werner
- Department of Pathology, University Hospital Freiburg, i. Br., Breisacherstr. 115a, D-79106 Freiburg i. Br., Germany
| | - Urs-Nikolaus Riede
- Department of Pathology, University Hospital Freiburg, i. Br., Breisacherstr. 115a, D-79106 Freiburg i. Br., Germany
| | - Claudia Otto
- Department of Pathology, University Hospital Freiburg, i. Br., Breisacherstr. 115a, D-79106 Freiburg i. Br., Germany
| |
Collapse
|
|
9
|
Liu Y, Lu X, Xiang FL, Poelmann RE, Gittenberger-de Groot AC, Robbins J, Feng Q. Nitric oxide synthase-3 deficiency results in hypoplastic coronary arteries and postnatal myocardial infarction. Eur Heart J 2012; 35:920-31. [PMID: 23048191 DOI: 10.1093/eurheartj/ehs306] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
AIMS Hypoplastic coronary artery disease is a rare congenital abnormality that is associated with sudden cardiac death. However, molecular mechanisms responsible for this disease are not clear. The aim of the present study was to assess the role of nitric oxide synthase-3 (NOS3) in the pathogenesis of hypoplastic coronary arteries. METHODS AND RESULTS Wild-type (WT), NOS3(-/-), and a novel cardiac-specific NOS3 overexpression mouse model were employed. Deficiency in NOS3 resulted in coronary artery hypoplasia in foetal mice and spontaneous myocardial infarction in postnatal hearts. Coronary artery diameters, vessel density, and volume were significantly decreased in NOS3(-/-) mice at postnatal day 0. In addition, NOS3(-/-) mice showed a significant increase in the ventricular wall thickness, myocardial volume, and cardiomyocyte cell size compared with WT mice. Lack of NOS3 also down-regulated the expression of Gata4, Wilms tumour-1, vascular endothelial growth factor, basic fibroblast growth factor and erythropoietin, and inhibited migration of epicardial cells. These abnormalities and hypoplastic coronary arteries in the NOS3(-/-) mice were completely rescued by the cardiac-specific overexpression of NOS3. CONCLUSION Nitric oxide synthase-3 is required for coronary artery development and deficiency in NOS3 leads to hypoplastic coronary arteries.
Collapse
Affiliation(s)
- Yin Liu
- Department of Physiology and Pharmacology, Western University, London, Ontario, Canada N6A 5C1
| | | | | | | | | | | | | |
Collapse
|
|
10
|
Sudden death due to coronary tree hypoplasia. Am J Forensic Med Pathol 2011; 32:227-31. [PMID: 21512381 DOI: 10.1097/paf.0b013e318219c8e6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
A 20-year-old woman complained for several weeks before her death of unspecific epigastric pains. Such episodes were labeled as "dyspeptic episode." A month later, once again she started to complain about stomach pains, then she collapsed unconscious. She was rushed to a hospital, where she was admitted in cardiorespiratory arrest. An electrocardiogram revealed a ventricular fibrillation. All resuscitation efforts were unsuccessful. The autopsy revealed nonspecific general changes (subpleural petechiae and blood pooling). Dissection of the coronary arteries revealed a normal topography of coronary arteries but a marked hypoplasia of all the subepicardial coronary arteries (maximum diameter of left anterior descending coronary artery = 1.9 mm, maximum diameter of right coronary artery = 1.43 mm). Furthermore, the left anterior descending coronary artery was slightly compressed by a fibrous bridge at about 1.5 cm of its origin; a fibrinoleukocytic thrombus has formed immediately above this bottleneck, obstructing the entire lumen of the artery. Microscopic examination revealed an area of recent myocardial necrosis; diffuse subendocardial fibrosis, with extension into the subjacent myocardium, disorganizing the myocardial fibers; and variable thickening of the vascular walls with periarteriolar fibrosis. Weigert stain has shown marked intimal hyperplasia, either focal or diffuse, in different segments of vascular wall, accompanied by disruption of the internal elastic lamina due to subintimal cellular proliferation, in the medium branches of coronary arteries. The micrometric measurements showed that intimal thickening was at least the size of the media (∼358 vs 356.8 μm) and sometimes double.
Collapse
|
|
11
|
De Giorgio F, Abbate A, Stigliano E, Capelli A, Arena V. Hypoplastic coronary artery disease causing sudden death. Report of two cases and review of the literature. Cardiovasc Pathol 2009; 19:e107-11. [PMID: 19616973 DOI: 10.1016/j.carpath.2009.05.002] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/21/2008] [Revised: 05/06/2009] [Accepted: 05/19/2009] [Indexed: 01/25/2023] Open
Abstract
Congenital coronary anomalies represent a condition often unrecognized in the living and in the dead. Investigating this condition is relevant for both clinicians and pathologists in order to identify potentially unrecognized coronary causes of sudden death. Hypoplastic coronary artery disease (HCAD) is a rare congenital abnormality reported to be associated with sudden death. We report two additional cases of previously apparently healthy people who died suddenly and were found to have HCAD at postmortem evaluation. The clinicopathologic findings are discussed along with a review of the literature.
Collapse
Affiliation(s)
- Fabio De Giorgio
- Institute of Legal Medicine, Catholic University of the Sacred Heart, School of Medicine, Rome, Italy
| | | | | | | | | |
Collapse
|
|
12
|
Durán AC, Arqué JM, Fernández B, Fernández MC, Fernández-Gallego T, Rodríguez C, Sans-Coma V. Rudimentary coronary artery in Syrian hamsters (Mesocricetus auratus). Anat Histol Embryol 2009; 38:270-4. [PMID: 19426435 DOI: 10.1111/j.1439-0264.2009.00935.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
Congenital underdevelopment of one or more main branches of the coronary arteries has been reported in man, but not in non-human mammals. In man, this defective coronary artery arrangement may cause myocardial ischaemia and even sudden death. The main goal of this study was to describe the coronary artery distribution patterns associated with the presence of a markedly underdeveloped (rudimentary) coronary artery in Syrian hamsters. Moreover, an attempt was made to explain the morphogenesis of these patterns, according to current knowledge on coronary artery development. Eleven affected hamsters belonging to a laboratory inbred family were examined by means of internal casts of the heart, great arterial trunks and coronary arteries. The aortic valve was tricuspid (normal) in seven hamsters and bicuspid in the other four. A rudimentary coronary artery arose from the right side of the aortic valve in four specimens, from the left side of the aortic valve in a further three, and from the dorsal aortic sinus in the remaining four. In all cases, a second, well-developed coronary artery provided for all the coronary blood flow. Except for the existence of a rudimentary coronary artery, the present anomalous coronary artery distribution patterns are similar to coronary artery patterns reported in Syrian hamsters, dogs and humans in association with a solitary coronary ostium in aorta. We suggest that an unusual prolonged time interval in the development of the embryonic coronary stems might be a key factor in the formation of coronary arteries displaying significantly dissimilar developmental degrees.
Collapse
Affiliation(s)
- A C Durán
- Department of Animal Biology, Faculty of Science, University of Málaga, Málaga, Spain.
| | | | | | | | | | | | | |
Collapse
|
|
13
|
Sim DS, Jeong MH, Choi S, Yoon NS, Yoon HJ, Moon JY, Hong YJ, Kim KH, Park HW, Kim JH, Ahn Y, Cho JG, Park JC, Kang JC. Myocardial Infarction in a Young Man due to a Hypoplastic Coronary Artery. Korean Circ J 2009; 39:163-7. [PMID: 19949606 PMCID: PMC2771812 DOI: 10.4070/kcj.2009.39.4.163] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/23/2008] [Revised: 11/04/2008] [Accepted: 11/12/2008] [Indexed: 11/13/2022] Open
Abstract
Hypoplastic coronary artery disease (HCAD) is a rare condition that may lead to myocardial infarction (MI) and sudden death. We discovered HCAD in a young man who developed chest pain after heavy drinking and who was found to have suffered an MI. His ECG showed ST-segment elevation with Q waves in the anterior leads, and echocardiography revealed apical dyskinesia with moderate left ventricular (LV) dysfunction. Coronary angiography showed hypoplasia of the left anterior descending (LAD) artery. 99mTc-tetrofosmin-gated myocardial perfusion scintigraphy showed a large, fixed perfusion defect in the anteroseptal and apical segments. Sixty-four-slice cardiac CT and cardiac MR imaging demonstrated thinning of the apical wall with calcification and delayed enhancement, supporting the diagnosis of long-standing MI. The patient was discharged symptom-free on medication for ischemic heart failure two weeks after admission. Although HCAD is very uncommon, it should be considered in children and young adults who suffer MI or sudden cardiac death.
Collapse
Affiliation(s)
- Doo Sun Sim
- The Heart Center of Chonnam National University Hospital, Cardiovascular Research Institute of Chonnam National University, Gwangju, Korea
| | | | | | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
14
|
Wick R, Otto S, Byard RW. Is Right Coronary Artery Hypoplasia and Sudden Death an Underdiagnosed Association? Am J Forensic Med Pathol 2007; 28:128-30. [PMID: 17525562 DOI: 10.1097/paf.0b013e31805c93fd] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
Determining whether hypoplasia of a coronary artery has caused or contributed to death is often complicated by an absence of histologic evidence of myocardial ischemia in the area of the heart supplied by the affected artery and also by the lack of data for assessing coronary artery size at autopsy. A 45-year-old woman is reported who collapsed and died and who was found at autopsy to have a dominant, small-caliber, right coronary artery, with acute and chronic ischemic changes in the posterior interventricular septum supplied by the diminutive vessel. This case provides evidence that small-caliber coronary arteries may be associated with a lethal outcome. Given the difficulties that may occur in determining whether there is a causal link between small coronary artery caliber and death, it is possible that this may be an underdiagnosed cause of sudden cardiac death, rather than a coincidental finding of minimal significance.
Collapse
Affiliation(s)
- Regula Wick
- Forensic Science SA, University of Adelaide, Adelaide, Australia
| | | | | |
Collapse
|
|
15
|
Celik T, Iyisoy A, Kursaklioglu H, Kose S, Kilic S, Amasyali B, Isik E. Does Coronary Artery Size Really Matter? Echocardiography 2005; 22:479-86. [PMID: 15966932 DOI: 10.1111/j.1540-8175.2005.40042.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022] Open
Abstract
OBJECTIVE To evaluate ischemia in right ventricle (RV) in patients with small caliber of right coronary artery (RCA). PATIENTS AND METHODS The study population consisted of 60 consecutive patients undergoing coronary angiography within 3 months. The patients were divided into three different groups according to lumen diameter of RCA and coronary dominance. Group 1, 2, and 3 consisted of patients with small diameter of RCA, dominant RCA, and dominant circumflex artery, respectively. RV ischemia was assessed by using pulse-wave tissue Doppler sampling obtained from RV free wall close to the lateral tricuspid annulus at the apical four-chamber view during dobutamine stress echocardiography (DSE). RESULTS When the mean systolic velocity percentages of increase from low to peak dose dobutamine in patients with small RCA were compared to those of other groups, statistically significant difference was found between group 1 and the other groups (P = 0.007 for group 1 vs group 2; P = 0.01 for group 1 vs group 3). The mean systolic velocity at peak dobutamine dose of patients with small caliber of right coronary artery was statistically lower than the other groups (P = 0.001 for group 1 vs group 2, P < 0.001 group 1 vs group 3). CONCLUSIONS There are a group of patients with small diameter of RCA causing probable ischemia in RV and small caliber of RCA can really matter in these patients.
Collapse
Affiliation(s)
- Turgay Celik
- Department of Cardiology, Gulhane Military Medical Academy, School of Medicine, Etlik, Ankara, Turkey.
| | | | | | | | | | | | | |
Collapse
|
|
16
|
Abstract
Hypoplastic coronary artery disease (HCAD) is a rare abnormality with a high rate of sudden death and poor outcome. HCAD was revealed by myocardial infarction in a teenager with objective evidence of silent ischaemia on myocardial scintigraphy. After four years of follow up, he suddenly collapsed during exercise and subsequently died. Although HCAD is very uncommon, it should be actively excluded in children and young adults who experience sudden cardiac death. Aggressive treatment such as implantable cardioverter-defibrillator or heart transplantation may be indicated for this rare coronary abnormality.
Collapse
Affiliation(s)
- N Amabile
- Département de cardiologie, Hôpital de la Timone, 264 rue St Pierre, 13385 Marseille Cedex 05, France
| | | | | |
Collapse
|
|
17
|
Hashimoto N, Nagashima J, Miyazu O, Akashi Y, Kawasaki K, Imai Y, Tsuchiya K, Ozawa A, So T, Musha H, Sakakibara M, Miyake F. Congenital Absence of the Left Circumflex Coronary Artery Associated With Acute Myocardial Infarction-A Case Report-. Circ J 2004; 68:91-3. [PMID: 14695474 DOI: 10.1253/circj.68.91] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Among the congenital anomalies of the coronary arteries, a left circumflex artery (LCX) defect is extremely rare. A 49-year-old man who developed an acute anterior infarction underwent coronary angiography, which revealed complete occlusion of the left main trunk, but the territory usually supplied by the LCX had been perfused by the superdominant right coronary artery. Treatment of the left main trunk by percutaneous coronary intervention produced a favorable result. Accurate evaluation of the principal vessels and the extent of compensatory perfusion is important when diagnosing ischemic heart disease accompanied by anomalous coronary arteries and for choosing the best treatment modality.
Collapse
Affiliation(s)
- Nobuyuki Hashimoto
- Department of Cardiology, St Marianna University Yokohama City Seibu Hospital and St. Marianna University School of Medicine, Kanagawa, Japan
| | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
18
|
Maron BJ, Carney KP, Lever HM, Lewis JF, Barac I, Casey SA, Sherrid MV. Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003; 41:974-80. [PMID: 12651044 DOI: 10.1016/s0735-1097(02)02976-5] [Citation(s) in RCA: 193] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
Abstract
OBJECTIVES The goal of this study was to determine the impact of race on identification of hypertrophic cardiomyopathy (HCM). BACKGROUND Sudden death in young competitive athletes is due to a variety of cardiovascular diseases (CVDs) and, most commonly, HCM. These catastrophes have become an important issue for African Americans, although HCM has been previously regarded as rare in this segment of the U.S. population. METHODS We studied the relationship of race to the prevalence of CVDs causing sudden death in our national athlete registry, and compared these findings with a representative multicenter hospital-based cohort of patients with HCM. RESULTS Of 584 athlete deaths, 286 were documented to be due to CVD at ages 17 +/- 3 years; 156 (55%) were white, and 120 (42%) were African American. Most were male (90%), and 67% participated in basketball and football. Among the 286 cardiovascular deaths, most were due to HCM (n = 102; 36%) or anomalous coronary artery of wrong sinus origin (n = 37; 13%). Of the athletes who died of HCM, 42 (41%) were white, but 56 (55%) were African American. In contrast, of 1,986 clinically identified HCM patients, only 158 (8%) were African American (p < 0.001). CONCLUSIONS In this autopsy series, HCM represented a common cause of sudden death in young and previously undiagnosed African American male athletes, in sharp contrast with the infrequent clinical identification of HCM in a hospital-based population (i.e., by seven-fold). This discrepancy suggests that many HCM cases go unrecognized in the African American community, underscoring the need for enhanced clinical recognition of HCM to create the opportunity for preventive measures to be employed in high-risk patients with this complex disease.
Collapse
Affiliation(s)
- Barry J Maron
- Minneapolis Heart Institute Foundation, 920 East 28th Street, Suite 60, Minneapolis, MN 55407, USA.
| | | | | | | | | | | | | |
Collapse
|
|
19
|
Affiliation(s)
- Paolo Angelini
- Department of Adult Cardiology, Texas Heart Institute at St Luke's Episcopal Hospital, Houston 77030, USA.
| | | | | |
Collapse
|
|
20
|
Fraisse A, Quilici J, Canavy I, Savin B, Aubert F, Bory M. Images in cardiovascular medicine. Myocardial infarction in children with hypoplastic coronary arteries. Circulation 2000; 101:1219-22. [PMID: 10715271 DOI: 10.1161/01.cir.101.10.1219] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Affiliation(s)
- A Fraisse
- Service de Cardiologie A, Hôpital de la Timone, and the Service de Chirurgie thoracique et cardiovasculaire, Hôpital d'enfants de la Timone, Marseille, France.
| | | | | | | | | | | |
Collapse
|