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.     

Detection of malignant right coronary artery anomaly by multi-slice CT coronary angiography

Coronary artery anomalies occur in 0.3-0.8% of the population and infer a high risk for sudden cardiac death in young adults. Diagnosis is usually established during coronary angiography, which is hampered by poor spatial visualization. Magnetic resonance imaging is an alternative, but it is not feasible in the presence of metal objects or claustrophobia. In this report, a 15-year-old boy experienced ventricular fibrillation and was successfully resuscitated. Cardiac catheterization was inconclusive, and pacemaker implantation prohibited the use of MR imaging. Multi-slice CT coronary angiography revealed a malignant anomalous right coronary artery.     

An anomalous origin of left coronary artery and sudden death in a soccer player: a case report

Coronary artery anomalies, after hypertrophic cardiomyopathy, are the second most common cause of exercise-related sudden cardiac deaths. These anomalies have been associated with myocardial ischaemia, arrhythmia and sudden death during exercise. Anomalous origin of the left coronary artery from the right sinus of Valsalva with anterior or posterior courses is not always thought to be a benign anomaly. A 22-year-old man died suddenly on a football field. At autopsy, there was an abnormal origin of the left coronary artery from the right sinus of Valsalva. The abnormal course of the artery between the aorta and pulmonary trunk had limited victim's functional capacity. The history, cause of death and patho-physiology are discussed, and some preventive strategies suggested.     

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.     

Coronary artery anomalies overview: The normal and the abnormal

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.     

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.     

Congenital anomalies of coronary artery origin in adults: 64-MDCT appearance

OBJECTIVE: The purpose of this pictorial essay is to review the 64-MDCT appearance of congenital anomalies of the origins of the coronary arteries in adults. CONCLUSION: Increasing use of MDCT for cardiac imaging of adults requires familiarity with the cross-sectional appearance of congenital coronary artery anomalies visualized with noninvasive imaging techniques. Many of these anomalies are benign, but a small number are associated with myocardial ischemia and sudden death. Increasing use of MDCT in cardiac imaging may yield diagnostic information not obtained with coronary angiography. Axial, multiplanar, and 3D volume-rendered reconstructions should aid in detection and improve interpretation of such anomalies.     

Sudden death in a case of recurrent Takotsubo syndrome

Herein we report an unusual case of sudden death occurring in a 65 year old woman during a minor oral surgery. The subject, who had a medically treated anxiety, had a history of two reversible left ventricle dysfunction episodes consistent with recurrent Takotsubo Syndrome that had occurred seven and six years before, respectively. She also suffered from moderate, well treated post-menopausal systemic hypertension. Post-mortem examination showed apical biventricular ballooning of the heart with no cardiac rupture, coronary artery lesion or other cardiac/extra-cardiac disease. Toxicological tests and forensic investigations excluded unnatural causes of death, including pharmacological or iatrogenic causes related to medical malpractice. Only non-specific contraction bands and mild hypertrophy were observed by histology in the left ventricle myocytes. Takotsubo syndrome is usually an acute and reversible heart failure syndrome with acute left ventricle apex ballooning, no coronary artery disease or other macroscopic or microscopic cardiac changes; physical or emotional stress are well known triggering factors. Nevertheless, recurrent forms, major cardiac adverse events and even sudden death may occur in a minority of cases, meaning that a diagnosis of Takotsubo syndrome must be considered in cases of sudden death and in forensic investigations.

     

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.     

冠状动脉解剖异常及临床意义(二)——冠状动脉走行、终止、结构异常及发育不良

原发性冠状动脉解剖异常较为少见,但有部分冠状动脉解剖异常所导致的后果较为严重,比如猝死、晕厥、其他先天性心脏病、心绞痛综合征等.由于无创检查手段的广泛应用,特别是多层螺旋CT的成功应用,冠状动脉异常的报道日益增多.本文就近年来关于冠状动脉数日及起源异常报道作简介,以便临床更加深入地认识本病及在临床工作中调整治疗方案.     

Coronary artery anomalies diagnosed by magnetic resonance angiography

Coronary artery anomalies are uncommon entities that may be associated with sudden death. Because of its 2-D projection imaging nature, conventional X-ray coronary angiography may not accurately delineate the origins and course of aberrant coronary arteries with respect to the great vessels. Non-invasive, cross-sectional imaging techniques such as coronary CT angiography and magnetic resonance angiography are increasingly used in clinical practice to diagnose coronary artery anomalies. Although this study reviews coronary artery anatomy and selected anomalies as seen with true fast imaging with steady-state precession magnetic resonance angiography, the information provided is equally applicable to electrocardiogram-gated coronary CT angiography.     

Sudden death in competitive athletes

Sudden death in athletes is an extremely rare event yet no less tragic for its infrequency. Up to 90% of these deaths are due to underlying cardiovascular diseases and therefore categorized as sudden cardiac death (SCD). The causes of SCD among athletes are strongly correlated with age. In young athletes (<35 years), the leading causes are congenital cardiac diseases, particularly hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and congenital coronary artery anomalies. By contrast, most of deaths in older athletes (<35 years) are due to coronary artery disease. This review focuses on the cardiac causes of SCD and provides a brief summary of the principal noncardiac causes. Current pre-participation screening strategies are also discussed, with particular emphasis on the Italian experience.     

Acute coronary syndrome as a cause of sudden death in patients with old myocardial infarction: a pathological analysis

Old myocardial infarction (OMI) is one of the most important pathological manifestations in sudden cardiac death. Fatal arrhythmia arising from a fibrotic scar has been determined as the cause of death in most cases with old myocardial infarction. However, the significance of acute plaque disruption/thrombosis of the coronary arteries in those patients has not been investigated. We examined a series of 33 hearts from individuals with OMI who died suddenly during the period from 1998 to 2001. Detailed coronary pathological findings on these hearts indicated fresh or recent rupture of the coronary plaque with thrombosis in 18 cases (55%). As a result of comprehensive analysis, the sudden deaths were explained by acute coronary syndrome in 18 cases (55%), fatal arrhythmia in eight (24%), cardiac pump failure in five (14%), and other causes in two (6%) cases. Our findings revealed that a new coronary plaque rupture independent of the old infarct was a major cause of sudden cardiac death with OMI.     

Detection of malignant right coronary artery anomaly by multi-slice CT coronary angiography

European Radiology - Coronary artery anomalies occur in 0.3–0.8% of the population and infer a high risk for sudden cardiac death in young adults. Diagnosis is usually established during...     

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.     

Plötzlicher Tod bei sexueller Aktivität

A case of sudden cardiac death in a 37-year-old man during sexual activity is reported. The only morphological findings which could explain death were determined with the histological investigation of the cardiac conduction system. The heart exhibited fibromuscular dysplasia of the atrioventricular node artery and individual small coronary arteries as well as fiber residues of the left bundle branch. The potential death mechanisms as well as the predisposing factors are discussed. This case is reported to supplement the causes described so far for sudden natural death during sexual activities.     

冠状动脉解剖异常及临床意义(一)——冠状动脉解剖、数目及起源异常

原发性冠状动脉解剖异常较为少见,但有部分冠状动脉解剖异常所导致的后果较为严重,比如猝死、晕厥、其他先天性心脏病、心绞痛综合征等.由于无创检查手段的广泛应用,特别是多层螺旋CT的成功应用,冠状动脉异常的报道日益增多.本文就近年来关于冠状动脉数日及起源异常报道作简介,以便临床更加深入地认识本病及在临床工作中调整治疗方案.     

Anomalies of coronary artery origin: Evaluation on multidetector CT angiography

Anomalies of origin of coronary arteries are an uncommon occurrence and found in approximately 1–2% of the general population. While a large proportion of these anomalies are clinically silent, a few might be hemodynamically significant and may even result in sudden cardiac death. Comprehensive knowledge of the normal as well as variant anatomies of the coronary artery origin and familiarity with imaging appearances and clinical significance of these anomalies is imperative for precise diagnosis and subsequent planning of treatment, whenever required. Multidetector computed tomography angiography, on account of its non-invasiveness, faster scan times and multiplanar reconstruction capabilities, is increasingly being utilized for characterization of coronary artery origin anomalies and their three-dimensional spatial relations. It shows a superior rate of detection of these anomalies compared to conventional angiography, providing more accurate delineation of the ostium as well as course. With the advent of newer generation CT scanners and use of advanced dose reduction techniques, images can be obtained rapidly having excellent spatial resolution and with minimal radiation dose. In this review article, we present the multidetector CT angiography imaging findings of the spectrum of anomalous coronary artery origin, using a third-generation dual-source CT scanner.     

Bland-White-Garland-Syndrom

This is a report on the sudden death of a 4-week-old female infant without external signs of violence. At autopsy the anomalous origin of the left coronary artery from the pulmonary trunk (Bland-White-Garland syndrome) was identified as the possible cause of death. Immunohistochemical investigations revealed fresh cardiac necrosis indicating an acute fatal event. Toxicological analyses were negative.     

冠状动脉粥样硬化斑块的影像学评价

冠状动脉硬化斑块易发和腐蚀和破裂,导致急性冠状动脉综合征,引起急性心肌梗塞.有效的评价斑块的结构及成分特点,监测其演变过程,对选择治疗时机和治疗方案有重要意义.本文综述了冠状动脉粥样硬化斑块的病理特点和演变规律,评价了各种影像技术包括血管内超声,多层螺旋CT和磁共振,冠状动脉造影对其诊断的价值以及应用的限度.