Sudden unexpected death as a result of anomalous origin of the right coronary artery from the left sinus of Valsalva

The association of sudden unexpected death with coronary artery anomalies has been limited to cases of anomalous origin of the left main coronary artery, either from the right sinus of Valsalva or from the main pulmonary artery. In contrast, anomalies involving the origin of the right coronary artery have been considered to be benign. Postmortem examination in a patient who died suddenly at 23 years of age disclosed that the right coronary artery originated anomalously from the left sinus of Valsalva. The findings in this patient: (1) constitute the first necropsy documentation of premature sudden unexpected death in a patient whose sole pathologic abnormality was anomalous origin of the right coronary artery; (2) have important implications regarding previously proposed mechanisms of sudden death due to anomalous origin of the left main coronary artery; and (3) support recent suggestions that markedly acute angulation of either coronary artery, even when located in the appropriate sinus of Valsalva, may predispose to sudden unexpected death.     

Frequency in the anomalous origin of the right coronary artery with angiography in a Turkish population

OBJECTIVE: The estimate frequency of anatomic variations in origin of the right coronary artery in a Turkish population. MATERIAL AND METHOD: The angiographic data of 5253 consecutive adults patients undergoing coronary angiography were analysed retrospectively for the diagnosis of anomalous origin of the right coronary artery. RESULTS: Among 5253 adults patients, five (0.09%) patients had anomalous origin of the right coronary. They had an isolated anomalous origin of the right coronary artery. The right coronary arose from the left coronary sinus of Valsalva (there was separate orificium for the right coronary artery and the left coronary artery) in two (0.03%) patients, from above the left coronary sinus of Valsalva in three (0.05%) patients. In all patients, the anomalous origin of right coronary artery from the left sinus of Valsalva and from above the left coronary sinus of Valsalva coursed between the aorta and the pulmonary artery. CONCLUSION: The anomalous origin of the right coronary artery is a rare congenital cardiac malformation. Most patients remain asymptomatic. However, there are cases of sudden cardiac death described in the literature, indicating a potentially malign course of the disease. The angiographic recognition of this vessel may be useful for physicians dealing with diagnosis and treatment of the anomaly of the right coronary artery.     

成人冠状动脉起源异常10例

目的 :了解冠状动脉起源异常 (coronaryarteryoriginanomalies,CAOA)的情况。 方法 :回顾性分析我院从 1998至 2 0 0 3年 838例冠状动脉造影资料。结果 :838例中CAOA 10例 ( 1.19% ) ,其中右冠状动脉起源于左乏氏窦 4例、无冠窦 1例 ,右冠状动脉高位开口 2例 ,左前降支和左回旋支分别独立起源于左乏氏窦 2例 ,单支冠状动脉 1例。结论 :最常见的类型是右冠状动脉起源于左乏氏窦 ,大多数异常类型并不导致严重的临床症状 ,只有少数需要外科手术治疗。     

Morbidity associated with anomalous origin of the right coronary artery from the left sinus of Valsalva

Anomalies of the coronary arteries occur infrequently, but can have major clinical consequences. Many reports have described an association between sudden death and origin of the left coronary artery from the right sinus of Valsalva, but origin of the right coronary artery from the left sinus of Valsalva is thought to be benign. Herein, we describe a patient in whom anomalous origin of the right coronary artery from the left sinus of Valsalva was associated with significant cardiovascular morbidity. A 25-year-old man developed complete heart block and myocardial infarction in the distribution of a dominant anomalous right coronary artery free of atherosclerotic lesions. Systolic compression at the origin of the anomalous artery was demonstrated. The left coronary artery was normal. We conclude that anomalous origin of the right coronary artery from the left sinus of Valsalva may be associated with significant cardiovascular morbidity in the absence of atherosclerosis.     

Anomalous origin of the left circumflex coronary artery: A case report

The anomalous origin of the left circumflex coronary artery from the right sinus of Valsalva is a relatively common anatomical variation. Difficulties may occur in the diagnostic procedure, but recognition and adequate visualization of the anomaly is essential for proper patient management, especially in patients undergoing evaluation for percutaneous coronary intervention, coronary artery surgery or prosthetic valve replacement. In the present report, a patient who had undergone percutaneous coronary intervention for a right coronary artery lesion after inferior myocardial infarction is described. The anomalous origin of the left circumflex coronary artery arising independently from the right sinus of Valsalva was previously undetected.     

The primary anomalies of coronary artery origin and course: A coronary angiographic analysis of 16,573 patients

BACKGROUND/OBJECTIVES:

Coronary artery anomalies are present at birth, but relatively few are symptomatic. The majority are discovered incidentally. In the present study, coronary angiograms performed in the authors’ centre (Ondokuz Mayis University Hospital, Samsun, Turkey) were analyzed to determine the prevalence and types of coronary artery origin and course anomalies.

METHODS:

Coronary angiographic data of 16,573 patients were analyzed. Anomalous origins and courses of coronary arteries were assessed.

RESULTS:

Anomalous coronary arteries were detected in 48 (0.29%) of 16,573 patients. The origin of the circumflex (Cx) artery from the right coronary artery (RCA) or right sinus of Valsalva was the most common anomaly (28 patients [58.3%]). An anomalous RCA originating from the left anterior descending artery (LAD) or Cx artery was observed in six patients (12.5%). The left coronary artery originated from the right sinus of Valsalva in five patients, and the LAD originated from the RCA or the right sinus of Valsalva in five patients. The RCA originated from the left sinus of Valsalva in three patients and from an ectopic ostium in the ascending aorta in one patient.

CONCLUSIONS:

The most frequent anomaly observed in the present study was related to the Cx artery, which is consistent with previous reports. Although coronary artery anomalies are rare, they may cause difficulties during coronary interventions or cardiac surgery and may occasionally result in sudden cardiac death. Therefore, the recognition and diagnosis of these anomalies is important and requires specialization in coronary angiographic techniques and other imaging modalities.     

冠状动脉起源异常的检出率与冠状动脉狭窄

目的　评价国人冠状动脉(冠脉)起源异常的冠脉造影检出率,并探讨其与冠脉狭窄之间的关系。方法　回顾性分析2001年1月至2004年6月在我院接受选择性冠脉造影的患者,观察并记录冠脉起源异常的检出频度和类型,以及合并冠脉狭窄的情况。结果　4094例患者中,共检出冠脉起源异常者32例,检出率为0 78%,其中右冠脉异常起源最为多见,占总数的65 6%;合并冠脉狭窄者共11例(占34 3% ), 其中仅5例(占15 6% )累及起源异常的冠脉,且无1例冠脉狭窄单独累及起源异常的冠脉。结论　国人冠脉起源异常的检出率与国外文献报道结果相似,其中以右冠脉起源异常最为多见,冠脉起源异常与冠脉狭窄不具有相关性。     

Percutaneous transluminal coronary angioplasty of anomalous left circumflex coronary artery

We present our experience of percutaneous transluminal coronary angioplasty in two patients with an anomalous left circumflex coronary artery with severe stenosis. In the first case, the anomalous vessel originated from the first portion of the right coronary artery, and in the second case it originated from the right sinus of Valsalva. Cannulating the anomalous vessel with the guiding catheter can be difficult. The right Judkins-type catheter, with a posteriorly directed tip, is the most appropriate catheter for cannulating the anomalous circumflex artery when the vessel originates from the first portion of the right coronary artery, and the left Amplatz-type 1 is most appropriate when the vessel originates from the right sinus of Valsalva.     

Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries.

Clinical and angiographic features of 31 patients with anomalous coronpary artery origin are reviewed. Of 17 aberrant circumflex arteries from the right sinus of Valsalva or artery, each was retro-aortic, six atherosclerotic, and seven irrigated a small area of myocardium. Of seven anomalous right coronary arteries from the left sinus of Valsalva or artery, each was ante-aortic and two atherosclerotic. Aberrant origin of the circumflex or right coronary artery was a benign anomaly. The proximal course of seven aberrant left coronary arteries from the right sinus of Valsalva or right coronary artery was related to clinical events. The anomaly was best demonstrated in the lateral view. In five cases, coronary blood flow during exercise and myocardial metabolism during pacing were assessed. Two had had a previous infarction with nonatherosclerotic arteries. Exercise coronary blood flow was normal in four and low in one patient. Pacing resulted in lactate production in two with atherosclerotic and one without atherosclerotic arteries. We conclude that anomalous left coronpary origin from the right sinus of Valsalva can result in significant myocardial ischemia and infarction.     

Diagnosis and management of anomalous origin of the right coronary artery from the left coronary sinus

The anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva coursing between the aorta and the pulmonary artery or right ventricular outflow tract, is considered a potentially fatal abnormality which may require surgery. However, diagnosing the correct course with coronary arteriography may be difficult. Fast gradient echo magnetic resonance (MR) imaging can be helpful to identify and confirm the course of aberrant coronary arteries and their relationship to the surrounding tissue. In this study, diagnostic procedures and management are described of four patients in whom the RCA originated from the left sinus of Valsalva. Although reported as investigational by the Task Force document on MR imaging by the European Society of Cardiology we are of the opinion that MR coronary angiography may have an important future role in the assessment of anomalous coronary arteries.     

Anomalous left coronary artery from the right sinus of Valsalva associated with coronary atheromatosis

Anomalous origin of the left coronary artery from the right sinus of Valsalva is an anatomical abnormality that is usually associated with myocardial ischemia and sudden death. Although this abnormality may coexist with obstructive atherosclerotic coronary disease, disease is not usually found in the anomalous course of the artery. When this coronary anomaly and obstructive coronary disease are both present, it is difficult to determine the cause of ischemic symptoms. We report a case in which three different diagnostic techniques were used to find the cause of ischemic symptoms in a patient whose left coronary artery originated anomalously in the right sinus of Valsalva and followed a course between the aorta and the pulmonary trunk and who had obstructive atherosclerotic lesions in the right coronary artery. The techniques were conventional angiography, which was used for the initial diagnosis, multislice computerized tomography, which was used to determine the anomalous course of the artery and its relationship with vascular structures, and exercise echocardiography, which was used to evaluate ischemia in the left coronary artery territory after treatment of the stenoses in the right coronary artery.     

Surgery for Anomalous Origin of the Left Main Coronary Artery from the Right Sinus of Valsalva,in Association with Left Main Stenosis

Most medical literature regarding the anomalous origin of a coronary artery from the opposite sinus of Valsalva pertains to sudden death in the young. The surgical treatment of anomalous origin of the left main coronary artery from the right sinus of Valsalva is not particularly well codified, and when an anomalous left main coronary artery is associated with an extramural stenosis, treatment becomes more technically challenging. Herein, we describe a unique approach toward treating this combination of conditions.A 15-year-old adolescent boy was diagnosed with anomalous origin of the left main coronary artery from the right sinus of Valsalva. The condition was accompanied by a slit ostium and an extramural stenosis of the left main coronary artery. Surgery was offered to the patient in view of his young age and the uncertain prospective course of the disease. Coronary unroofing was not indicated, due to the extramural location of the stenosis. With the patient under cardiopulmonary bypass, the stenotic segment of the left main coronary artery was transected outside the aorta, repaired by vein patch augmentation, and reimplanted directly into the left coronary sinus. The patient recovered uneventfully. A postoperative computed tomographic angiogram showed good patency of the reconstructed artery. We expect excellent longevity of the directly reimplanted coronary artery.Key words: Adolescent, cardiac surgical procedures, chest pain/etiology, coronary angiography, coronary vessel anomalies/classification/diagnosis/surgery, death, sudden/prevention & control, prognosis, replantation, sinus of Valsalva/abnormalitiesMost of the medical literature regarding the anomalous origin of a coronary artery from the opposite sinus of Valsalva pertains to sudden death in young individuals.^1,2 Anomalous origin of the left main coronary artery (LMCA) from the right sinus of Valsalva is observed less frequently than is anomalous origin of the right coronary artery (RCA) from the left sinus,^1,3 and the surgical treatment for the former condition is less clearly codified than that for the latter.⁴ When an anomalous LMCA is associated with an extramural stenosis, treatment becomes more technically challenging. Here, we describe a unique approach for treating these conditions, upon their diagnosis in a 15-year-old patient.     

Primary stenting of an anomalous left main trunk originating from the right coronary artery during acute myocardial infarction

In 1.5 to 2.0% of patients with AMI referred for primary percutaneous coronary intervention, the left main trunk is identified as the culprit vessel. Among the congenital coronary anomalies, an anomalous origin of the left main trunk from the right sinus of Valsalva is very rare. A 73-year-old patient with lateral acute myocardial infarction was referred to primary angioplasty. The initial angiogram showed an anomalous origin of the left main from the right coronary artery, apparently with a septal course, with an angiographic image of intraluminal thrombus; in addition, a severe lesion was present at the distal right coronary artery. The left main and right coronary arteries were successfully treated with direct stenting. The subsequent clinical course during hospitalization was uneventful, and the patient was discharged alive.     

Anomalous origin of the right coronary artery from the ascending aorta: case history

A patient is described with an anomalous right coronary artery arising high above the left sinus of Valsalva. This patient is unique because the other 2 cases with such an anomalous origin of a right coronary artery had bicuspid aortic with such an anomalous origin of a right coronary artery had bicuspid aortic valves; this patient had a normal tricuspid aortic valve.     

Myocardial ischemia caused by anomaly at the origin of the left coronary artery

The case of a 68 years old man admitted because of angina "de novo" and submitted to coronariography is reported. The rest ECG showed abnormalities of the ST-T suggesting myocardial ischemia. The coronariography showed: No significant atherosclerotic lesions. The left coronary artery had an anomalous origin; the left descending coronary artery originated from an independent ostium located at the right Valsalva sinus, the circumflex artery had its origin at the same ostium as the right coronary artery.     

Symptomatic anomalous origination of the left coronary artery from the opposite sinus of valsalva. Clinical presentations, diagnosis, and surgical repair

Anomalous origination of a coronary artery can have serious, even fatal, consequences. Intravascular ultrasonography has recently provided new insights into anomalous coronary artery origination from the opposite sinus of Valsalva. On the basis of these insights, we describe 3 typical forms of this anomaly with left coronary artery involvement, including clinical presentations, diagnostic methods (particularly intravascular ultrasonography), and details of surgical treatment. In this case series, the left coronary artery originated from the noncoronary sinus in 1 patient and from the right sinus in another patient. In the 3rd patient, both the left and right coronary arteries originated from the ascending aorta above the sinotubular junction. Baseline areas of stenosis ranged from 48.6% to 70.1%. Intravascular ultrasonography was the only method that enabled us to clarify the mechanisms and the severity of the anomaly. Pharmacologic challenge was useful to predict worsening that might have occurred under physiologic conditions. We found that, in cases of symptomatic left anomalous coronary artery origination from the opposite sinus of Valsalva, the proximal segment of the left coronary artery consistently has (1) an intramural course inside the aortic wall; (2) hypoplasia, as determined by its circumference; and (3) a cross-sectional ovaloid deformity (lateral compression) with phasic and exercise-induced worsening of the deformity With regard to surgical treatment, ostioplasty is preferable to coronary bypass. To establish sound guidelines for managing these anomalies, a larger series should be studied prospectively with quantitative parameters and long-term follow-up.     

Anomalous aortic origin of a coronary artery: a universally applicable surgical strategy

The most commonly reported coronary arterial malformation, in accounts of sudden deaths, is anomalous aortic origin of a coronary artery. Anomalous coronary arteries may arise from the left, right, or non-coronary sinuses of Valsalva. Importantly, although the left coronary artery from the right sinus has the worst prognosis, sudden death has been reported in all variants of origin from the various sinuses of Valsalva. This paper describes a technique that addresses all of the problems relating to anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva. This technique includes the transection of the ascending aorta and pulmonary trunk, coronary arterial enlargement with a pericardial patch, and lateral translocation of the pulmonary trunk to the left pulmonary artery. Anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva is a potentially lethal cardiac anomaly that can be corrected in all cases using this simplified surgical technique that addresses the major anatomic and physiological problems.     

成人冠状动脉起源异常分析

目的分析成人冠状动脉起源异常的发生情况。方法回顾性分析大连市金州区第一人民医院2003年3月至2006年12月接受冠状动脉造影的690例患者。结果完成冠状动脉造影690例,检出冠状动脉起源异常10例,检出率为1.4%,冠状动脉起源异常的10例中右冠状动脉起源异常7例,3例起源于无冠窦,1例起源于左冠窦,3例高位开口(2例开口于右冠窦上方,1例开口于无冠窦的上方);左冠状动脉起源异常3例,占30%,其中1例左回旋支起源于右冠窦,1例起源于右冠状动脉,1例左主干高位开口。结论冠状动脉造影是发现冠状动脉起源异常及确定其类型的重要手段。     

Stenting of anomalous left main coronary artery arising from the right sinus of Valsalva: a case report

Anomalous origin of left and right coronary arteries from a single coronary ostium in the right sinus of Valsalva is rare. Accordingly, few reports have described percutaneous coronary interventions in this anomaly. We report a case of a 75-year-old woman with a severe lesion in the anomalous left main coronary artery arising from a single ostium in the right sinus of Valsalva. The patient was successfully treated with direct stenting.