Unexpected sudden death resulting from anomalous origin of the right coronary artery from the left sinus of Valsalva: a case report involving identical twins

An anomalous origin of the coronary arteries had been implicated as a cause of sudden cardiac death in a case involving a 16-year-old student who suddenly collapsed while running track at school. An autopsy revealed that the right coronary artery arose from the aorta in the left sinus of Valsalva. In order to determine whether the same anomaly was inherent in his brother--an identical twin--a complete cardiac medical examination was conducted. Multi-detector-row computed tomography (MDCT) coronary angiography showed no anomaly at the sinus of Valsalva, which indicates that the anomaly, in this case, was not hereditary. This case exemplifies instances where forensic medicine may intervene to prevent sudden deaths in surviving family members.     

Coronary anomalies: Cardiac CT evaluation of the symptomatic adult athlete

Cardiac CT has become widely available as a diagnostic test. It is noninvasive, low risk (for contrast allergies), quick, and highly sensitive for identifying the coronary origins and course. Two coronary anomalies, the left main artery from the right sinus of Valsalva, and the right coronary artery originating from the left sinus, have been associated both with myocardial ischemia and sudden cardiac death when they course between the aorta and pulmonary arteries. These anomalies can go undetected into adulthood. The yield of noninvasive exercise testing is low. In the past, the ability to demonstrate these anomalies depended on a high index of suspicion and an aggressive invasive work-up with cardiac catheterization. Now cardiac CT can demonstrate these anomalies with ease and facilitate appropriate treatment.     

Anomalous origin of left main coronary artery: the value of myocardial scintigraphic and spiral computed tomography scans

Anomalous origin of the left main coronary artery (LMCA) from the right sinus of the Valsalva or the proximal right coronary artery (RCA) is one of the most clinically important anomalies of coronary circulation. We report the case of a patient with chest pain and abnormal thallium myocardial perfusion scan in whom the anomaly was first detected on invasive coronary arteriography. The exact anatomic course of anomalous LMCA was confirmed using contrast enhanced computed tomography.     

Sudden cardiac death related to left coronary artery anomalies including hypoplasia and anomalous origin with retro-aortic course

Congenital anomalies of the coronary arteries are a rare condition with an incidence of 0.3–1.3% in the general population. Clinically, sometimes these anomalies increase the risk of myocardial ischemia, which can present with a wide spectrum of symptoms, from angina to sudden cardiac death (SCD). This case report is about the SCD of an 8-year-old male, in apparent good health, during a football training. Although basic life support maneuvers were performed timely from bystanders and medical staff, the automated external defibrillator (AED) was not used. Autopsy revealed multiple left coronary artery (LCA) anomalies: origin from a separate ostium in the right sinus of Valsalva, slit-like shape of the ostium, acute angle take-off of the LCA from the aorta, retro-aortic course and focal coronary hypoplasia of some branches of the LCA. Microscopic examination revealed diffuse ischemic consequences at a different stage of tissue repair and mild multifocal lymphocytic infiltration. No other significant elements were detected at post-mortem examination. We discuss the forensic evaluation about the cause and the manner of death, considering also the modality of the resuscitation attempts and the claimed malpractice, as often occurs in case of sudden unexpected death in young athletes.     

Single coronary artery: a fatal R-I type

Coronary artery anomalies are found in approximately 1.3 % of symptomatic adult patients undergoing coronary arteriography and encompass a wide variety of anatomic patterns. Single coronary arteries are found in 0.024–0.066 % of this group. The particular type of a single coronary artery with an anatomically correct course of either artery (so-called L/R-I type according to the Lipton and Yamanaka/Hobbs classification) is an especially rare phenomenon and has been described for the left coronary artery and for the right coronary artery in adults. In these anomalies an isolated coronary artery ensuring the blood supply of the entire heart displayed a compensatory widening of the lumen. The case of a 6-year-old boy who collapsed during exercise and died subsequently of acute cardiac death is presented. At autopsy a single right coronary artery with an anatomically correct course (R-I type) arising from the right sinus of Valsalva was found. On microscopic examination myocardial calcifications and scars were found in the papillary muscles of the mitral valve. Common ion channel disorders were excluded by DNA analysis. Sudden cardiac death on the basis of chronic ischemic heart disease was ascertained as the cause of death. Autopsy and microscopic findings, as well as aspects of the underlying pathophysiology, are presented and discussed.     

Evaluation of unroofing procedure of anomalous origin of right coronary artery from left sinus of valsalva between aorta and pulmonary trunk by multidetector computed tomography

An anomalous origin of a coronary artery is a rare congenital anomaly. Conventionally, the diagnosis of coronary artery anomalies is performed by coronary angiography. Currently developed multidetector computed tomography has permitted better definition of the coronary arteries, however. Multidetector computed tomography could be used effectively in the diagnosis and evaluation of the postoperative status of a patient with an anomalous origin of the right coronary artery from the left sinus of Valsalva between the aorta and pulmonary trunk.     

Remarkable anatomic anomalies of coronary arteries and their clinical importance: a multidetector computed tomography angiographic study

OBJECTIVE: An anomalous origin and course of the coronary arteries can be benign or life threatening. Recently, because of new advances in computed tomography technology, radiologists have begun to interpret the diseases of coronary arteries. We aimed to demonstrate some remarkable anomalies of coronary arteries, some of which were not shown by multidetector computed tomography (MDCT) coronary angiography previously, and to discuss the clinical importance of these anomalies. MATERIALS AND METHODS: Seven hundred twenty-five consequent patients referred to Florence Nightingale Hospital and Atatürk University Hospital for MDCT coronary angiography were included in this study. The patients were between the ages of 33 and 78 years (mean +/- SD, 59 +/- 13.86 years). Four hundred ninety-seven patients (68.6%) were men, and 228 (31.4%) were women. All the examinations were evaluated by both a radiologist and a cardiologist. RESULTS: The incidence of anomalous anatomical origin and course of the coronaries found in our study group was 5.79% (n = 42). The anomalies found in our study are absence of the right coronary artery (RCA; n = 1, 0.13%), ectopic origin of RCA from the left anterior descending (LAD) artery (n = 1, 0.13%), absence of the left main coronary artery (n = 4, 0.52%), ectopic origin of the left main coronary artery from the right sinus of Valsalva (n = 1, 0.13%), double LAD and ectopic origin of LAD from RCA (n = 1, 0.13%), ectopic origin of the left circumflex artery from the right sinus of Valsalva (n = 3, 0.39%), ectopic origin of the left circumflex artery from RCA (n = 2, 0.26%), and myocardial bridging (n = 29, 4%). CONCLUSIONS: An anomalous origin of the coronary anatomy must be present in the interpretations because of its importance for patients, cardiologists, and surgeons. As a conclusion, our study showed that MDCT, especially volume rendering and maximum intensity projection techniques, may be useful for assessment of complex variations, when the conventional angiography may not be sufficient.     

128层CT冠状动脉成像对冠状动脉变异的诊断价值

目的:探讨128层CT对冠状动脉变异的诊断价值。方法:回顾性分析我院行冠状动脉CTA检查后发现的69例冠状动脉变异患者的图像,对所有图像分别行VR、MPR、MIP和CPR等,观察冠状动脉开口、起源、走行、分布及与周围血管的关系。结果:所有受检者中共发现存在冠状动脉变异69例(82处),右冠状动脉高位开口2例,其中起源变异共32例,走行异常42例,终止异常2例,发育不良或未发育6例。结论:128层CT冠状动脉成像可清晰显示冠状动脉起源、行程及终止的异常,从而为临床提供高质量的影像诊断。     

Exercise-related sudden death due to an unusual coronary artery anomaly

Coronary artery anomalies are, after hypertrophic cardiomyopathy, the second most common cause of exercise-related sudden cardiac death in young American athletes. These anomalies have been associated with myocardial ischemia, arrhythmia, and sudden death during exercise. A 14-yr-old male with no previous abnormal medical history collapsed during soccer practice and was successfully resuscitated without defibrillation. An extensive cardiac workup did not reveal any abnormalities. Two weeks later, he experienced a cardiac arrest while running and could not be resuscitated. Autopsy demonstrated an acute angle take-off of the left main coronary artery and a transverse slit-like opening with a fibrous cushion, which created a kink near its origin. This case report illustrates the difficulty in diagnosing coronary artery anomalies in general, and acute angle take-off and ostial ridges in particular.     

Coronary CTA assessment of coronary anomalies

Coronary anomalies occur in <1% of the general population and can range from a benign incidental finding to the cause of sudden cardiac death. The coronary anomalies are classified here according to the traditional grouping into those of origin and course, intrinsic arterial anatomy, and termination. Classic coronary anomalies of origin and course include those in which a coronary artery originates from the contralateral aortic sinus or the pulmonary artery with anomalous course. Single coronary artery anomalies, in which single coronary artery branches to supply the entire coronary tree, are also included in this category. Anomalies of intrinsic arterial anatomy are a broad class that includes myocardial bridges, coronary ectasia and aneurysms, subendocardial coursing arteries, and coronary artery duplication. Coronary anomalies of termination are those in which a coronary artery terminates in a fistulous connection to a great vessel or cardiac chamber. In the case of those anomalies associated with a risk of sudden cardiac death, the relevant imaging features on CT angiography (CTA) associated with poorer prognosis are reviewed. Recent guidelines and appropriateness criteria favor the use of coronary CTA for the evaluation of coronary anomalies. Although invasive angiography has historically been used to diagnose coronary anomalies, multidetector CT imaging techniques have now become an accurate noninvasive alternative. Cardiac CTA provides excellent spatial and temporal resolution, allowing accurate anatomical assessment of these anomalies.     

Single coronary artery from the right sinus of Valsalva: angiography, anatomy, and clinical significance.

A case of single coronary artery from the right sinus of Valsalva with a connecting branch passing between the aorta and right ventricular infundibulum is described. The anomaly was demonstrated at coronary arteriography and verified at surgery. This type of single coronary artery has been associated with sudden exertional death in young persons. The case prompted a review of the classification of single coronary artery with emphasis on clinical significance of the various subtypes and angiographic findings.     

64MSCTA对冠状动脉变异的诊断及临床评价

目的评价64层螺旋CT冠状动脉血管成像（CTCA）对冠状动脉变异的诊断价值及临床评价。方法回顾性分析3529例临床疑为心肌缺血患者及常规体查者冠状动脉CTA影像检查结果并对发现的变异冠状动脉进行分类。结果3529例冠状动脉CTA受检者中,共检出冠状动脉变异42例（1．19％,42／3529）,MIP、VRT、CPR重建图像均明确显示其开口及走行;其中右冠状动脉起自主动脉左冠状窦者9例,左冠状动脉起自主动脉右冠状窦者2例,左回旋支异位开口于右冠窦1例,冠状动脉高位开口于升主动脉壁者6例．并行左主干3例（前降支、旋支分别开口于左冠窦）,左主干开口于肺动脉伴右冠状动脉瘤1例,左旋支-冠状静脉瘘者2例,副冠状动脉9例,右冠状动脉-左心室瘘合并右冠状动脉瘤l例。冠状动脉发育不良8例。结论冠状动脉CTA能准确显示各种类型冠状动脉变异,可作为无创性诊断冠状动脉变异的首选方法。     

Sudden infant death with anomalous origin of the left coronary artery from the pulmonary artery

Autopsy of a 2-month-old boy revealed an anomalous origin of the left coronary artery from the pulmonary trunk. The endomyocardial fibrosis associated with myocardial ischemia was noted in the left ventricular wall of the enlarged heart. Death was attributed to silent heart failure due to the anomalous left coronary artery. Significant clinical symptoms are generally observed in patients with such anomalies, however, they might cause sudden death in childhood.     

Coronary artery anomalies: assessment with free-breathing three-dimensional coronary MR angiography

PURPOSE: To evaluate a simplified protocol by using free-breathing three-dimensional (3D) coronary magnetic resonance (MR) angiography to determine the anatomy of anomalous coronary arteries, in particular the relationship of the vessels to the aortic root. MATERIALS AND METHODS: Twenty-six patients (18 men, eight women; mean age, 50 years; age range, 18-77 years) who had a history of chest pain, palpitations, or syncope and who were suspected of having coronary artery anomalies were examined with free-breathing MR angiography. Multiple 3D volume slabs were acquired at the level of the sinuses of Valsalva by using diaphragmatic navigators for respiratory artifact suppression. The proximal anatomy of the coronary arteries was determined. RESULTS: Six anomalous circumflex arteries originated from the right sinus of Valsalva and passed behind the aortic root. Six right coronary arteries arose from the left sinus of Valsalva and coursed between the aortic root and the right ventricular outflow tract (RVOT). Nine left coronary arteries arose from the right sinus of Valsalva; seven of nine coursed between the aortic root and the RVOT. Five patients had minor anomalies. Overall, in eight patients with anomalous arteries that coursed between the aortic root and the RVOT, conventional coronary angiography could not be used confidently to identify the proximal course. CONCLUSION: Free-breathing 3D coronary MR angiography can be used to identify the proximal anatomy of anomalous coronary arteries.     

致猝死的冠状动脉起源异常及其临床意义

目的 了解起自对侧冠状动脉窦并可能引发青少年运动员猝死的冠状动脉畸形在国人中的发病率,结合文献资料比较其影像学特点和临床意义.方法 应用PACS系统顺序回顾14 343例不伴有其他心脏大血管畸形的国人CT冠状动脉成像所见,选取左、右冠状动脉起自对侧冠状动脉窦及左、右单冠状动脉的病例,对冠状动脉开口位置、走行路径、非粥样硬化狭窄截面形态、径线、狭窄程度及冠状动脉近段与主动脉壁夹角进行分析统计.结果 检出冠状动脉发自对侧冠状动脉窦(包括单冠状动脉)74例,其近段走行于主、肺动脉之间者59例.右冠状动脉起自左冠状动脉窦且走行于主、肺动脉之间55例,左冠状动脉起自右冠状动脉窦且走行于主、肺动脉间2例,左、右单冠状动脉且走行于大动脉间者各1例.国人此类冠状动脉近段走行于两大动脉之间的起源异常发病率为4.1‰(59/14 343).右冠状动脉起自左冠状动脉窦,起始段常伴非粥样硬化性狭窄,狭窄面积≥50%者29例(52.7%),≥70%者3例(5.4%).近段走行于主、肺动脉之间的右冠状动脉起自左窦是左冠状动脉起自右窦的18.7倍(56/3).结论 CT冠状动脉成像是诊断冠状动脉起源异常的最佳影像学手段.国人中该畸形的解剖类型分布有其特点,这一特点对引发我国青少年运动性猝死的影响值得探讨.     

Unexpected death in a young child associated with anomalous aortic origin of the left main coronary artery without physical exertion: A case of an anomalous coronary artery with highly abundant elastic fibers

Sudden death due to anomalous aortic origin of a coronary artery is far less common among young children in the absence of exercise stress. This report describes the case of a 2-year-old boy with a lower respiratory tract infection who suffered sudden cardiac arrest in his bed at home. The autopsy revealed that the left coronary artery (LCA) originated from the right sinus of Valsalva with an acute angle takeoff and traveled between the aorta and the pulmonary trunk (an interarterial course). Upon histological examination, the LCA, before reaching its major branches, was located adjacent to the outside of the aortic wall without an intramural passage, and the arterial wall was composed almost exclusively of elastic fibers without media containing smooth muscle cells throughout the entire length of the abnormal running. Screening tests for respiratory virus infection detected enterovirus in the lung tissue. In association with an acute angle takeoff and interarterial course, the wall structure with highly abundant elastic fibers that are more flexible tissues among blood vessel components might suggest their vulnerability to compression during the great vessels’ systolic expansion in the sympathetic activation induced by the viral infection, leading to fatal myocardial ischemia without physical exertion.     

Unusual Malignant Coronary Artery Anomaly: Results of Coronary Angiography, MR Imaging, and Multislice CT

We report the case of a man with an uncommon anomaly of the origin and course of the left coronary artery. Clinical, coronary angiography, magnetic resonance imaging, and multislice computed tomography findings of this intermittently symptomatic 49 year-old patient with the rare anomaly of his left coronary artery stemming from the right sinus of Valsalva and taking an interarterial and intraseptal course are presented. The diagnostic value of the different imaging modalities is discussed.     

MDCT coronary angiography for diagnosis of anomalous origin right coronary artery: a case report

This is a case report of anomalous origin of the right coronary artery, which arises from the left sinus of Valsalva depicted with multi-detector row tomography (MDCT) coronary angiography performed in emergency. In symptomatic patient, this anomalous origin is considered a malignant anomaly and needs urgent surgical treatment. Some studies demonstrated that MDCT is a reliable non-invasive technique to identify and define anomalous coronary arteries (ACAs) and their course.     

Anomalous origin of the coronary arteries in children: diagnostic role of three-dimensional coronary MR angiography

When the anomalous origin of coronary arteries (AOCA) is suspected in children (especially athletes), due to signs and symptoms of myocardial ischemia or on the basis of echocardiographic assessment, three-dimensional coronary magnetic resonance angiography (3D-CMRA) can be proposed for the fine morphological evaluation of coronary branches anatomy and course.We tested the diagnostic potential of CMRA angiography in a prospective study on AOCA in young patients. Between July 2005 and June 2008, 15 patients aged 6–29 years (mean age, 13.5 years±5.6 S.D.; median, 14) with clinical and echocardiographic suspicion of AOCA underwent CMRA (1.5 T), 3D whole-heart, free-breathing technique, without the use of contrast medium and β-blockers, with a mean examination time of 30 min. We acquired a second scan of all patients to ameliorate the quality of the acquisition and to improve our experience.AOCA was confirmed by 3D-CMRA in 8 out of 15 cases (53%) and three different anatomical variants were demonstrated, that is, ectopic origin of the left circumflex artery arising from the right coronary artery with retro-aortic course in four cases, single coronary artery arising from the right sinus of Valsalva with interarterial course in one case, ectopic right coronary artery arising from the left sinus of Valsalva with interarterial course in one case; in two patients without anomalies of origin of the coronary arteries, elongated LMCA with angulation of the proximal segment of the left circumflex artery was present.When AOCA is suspected particularly in children (especially athletes), CMRA without the use of contrast medium is an effective diagnostic technique, which is useful to clarify the spatial position of the anomalous course of the main coronary branches in order to suggest the most convenient management of the disease. CMRA does not need contrast medium, needles, and β-blockers; is repeatable in the same examination without the exposure to X-rays; allows a parent to stay near the child; and needs low collaboration in low-stress conditions.     

Ursprungsanomalie der rechten Koronararterie und Roemheld-Syndrom

Anomalies of the origin of the coronary arteries are rare congenital cardiac malformations, which are frequently slot-narrowed and run between the pulmonary trunk and the aorta predisposing to myocardial ischemia. The case of a 42-year-old man who died suddenly is presented where a slot-narrowed anomalous origin of the right coronary artery in the left aortic sinus and an overfull stomach resulted in a higher level of the diaphragm. These two findings can be considered the morphologic equivalent of sudden cardiac death.