Wrong Sinus Of Valsalva Research Articles

Surgical treatment of anomalous left main coronary artery with an intraconal course.

Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous aortic origin of a coronary artery (AAOCA) from the wrong sinus of Valsalva. There is currently a paucity of information regarding this entity. The purpose of this article is to review our surgical experience with repair of anomalous LMCA with an intraconal course. This was a retrospective review of 12 patients with an anomalous LMCA and an intraconal course who underwent surgical repair. The median age at surgery was 15years (range 2-47). The seven oldest patients all had preoperative symptoms of exertional chest pain and one also had exertional syncope. The five youngest patients had no preoperative symptoms. One of these patients had a hemodynamically significant ventricular septal defect and one patient was the sibling of a patient who had undergone repair of AAOCA. The 12 patients underwent surgical repair including unroofing of the myocardial bridge overlying the intraconal LMCA and a LeCompte procedure. There was no early or late mortality and there were no significant complications. All 12 patients have resumed normal, unrestricted activities. Twelve patients with an anomalous LMCA and intraconal course presented to our institution for treatment. Surgical repair was performed successfully in all 12, with resolution of symptoms in the 7 patients who were symptomatic preoperatively. These results suggest that the surgical treatment is safe and efficacious in patients with an anomalous LMCA and intraconal course.

Utility of Multimodality Imaging in the Morphologic Characterization of Anomalous Aortic Origin of a Coronary Artery.

Anomalous aortic origin of a coronary artery from the wrong Sinus of Valsalva (AAOCA) is a rare congenital anomaly and is associated with sudden cardiac death. Morphologic features considered to be "high risk" are significant luminal narrowing, acute coronary angulation at its origin, intramural course, and long interarterial course. A consistent approach for characterization of these features is lacking. A retrospective single-center review of all patients diagnosed with AAOCA using echocardiogram and computed tomography (CT)/magnetic resonance imaging (MRI) studies was performed. Twenty-nine patients were identified (25 using CT and 4 using MRI) with subsequent three-dimensional data sets. The MRI data sets lacked adequate resolution and were excluded. Twenty-five patients (median age 15.1, range 10-39.5 years, 72% male) were further analyzed using echocardiogram and CT. Morphologic assessment focused on luminal stenosis, coronary angulation, and interarterial length. Additional morphologic features focusing on cross-sectional area and degree of ellipticity were also assessed. Echocardiography tended to yield smaller measurements compared to CT and had poor interobserver reproducibility for measurements pertaining to the narrowest proximal and distal coronary segments. Computed tomography showed good inter-/intraobserver reproducibility for the same. Agreement between both modalities for coronary angulation at its origin was excellent. There was good agreement for measurements of interarterial length between echocardiography and CT, but echocardiography had superior reproducibility. Assessment of luminal cross-sectional area and elliptical shape by CT had excellent inter-/intraobserver reproducibility. The combination of echocardiography and CT characterizes morphologic features of anomalous origin of the coronary artery more reliably than either modality alone.

Surgical Treatment of Anomalous Aortic Origin of Coronary Arteries: The Reimplantation Technique and Its Modifications

Although anomalous aortic origin of a coronary artery has been described as a benign anomaly in the past, several reports suggest that such an anomaly may be the cause of sudden death in children and younger adults. Increased cardiac output during exercise may result in compression of the anomalous coronary artery when it runs between the aorta and the pulmonary artery or when its aortic origin is narrowed and its proximal course is intramural. It is unlikely that this rare anomaly would be identified during infancy, but once diagnosis has been made, indication to proceed with surgery is controversial. Nevertheless, if surgery is recommended, several operative techniques may be used, depending on the specific anatomy; unroofing, direct reimplantation, patch enlargement, and even coronary artery bypass grafting have been proposed. In this short report, we focus on different techniques of coronary reimplantation. As we already know from the arterial switch operation in neonates and from aortic root repair or replacement in adults, sufficient mobilization is necessary to allow direct reimplantation in the correct sinus to avoid kinking and other distortion.

Anomalous Aortic Origin of a Coronary Artery is Always a Surgical Disease.

Anomalous aortic origin of a coronary artery is a congenital anomaly in which a major coronary artery arises from the wrong sinus of Valsalva (left coronary from right sinus or right coronary from left sinus) and courses between the great arteries before reaching its normal epicardial course. Although the risk of sudden death is clearly established, the indications for surgery remain controversial. The risk of sudden death is increased in symptomatic patients, in anomalous left coronary artery, as well as in the presence of some risk factors (intense physical activity, young age [<35 years], aggravating anatomical features [intramural interarterial course, slit-like ostium, acute angle of take-off]). As far as is currently known, surgical management using an anatomical repair can prevent sudden death, provided that normal coronary anatomy and function are achieved and that extensive return-to-play testing is performed. A precise evaluation of the benefit/risk ratio is mandatory on an individual basis, but surgery may be indicated in the vast majority of patients.

The Congenital Heart Surgeons' Society Registry of Anomalous Aortic Origin of a Coronary Artery: an update.

The anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva with an interarterial, intramural, and/or intraconal course is a relatively rare congenital defect of the heart that may be associated with an increased risk of ischaemia of the myocardium and sudden death, notably in children and young adults. Data are limited regarding stratification of risk and long-term outcomes of these patients. In 2009, the Anomalous Coronary Artery Working Group formed the Congenital Heart Surgeons' Society Registry of Anomalous Aortic Origin of a Coronary Artery to obtain information on large numbers of young patients with anomalous aortic origin of a coronary artery with the goal to better understand the natural and surgical history of this anomaly as well as to develop evidence-based treatment and management guidelines. In this report, we describe the data we have collected from the registry and the current state of the registry.

Left Main Coronary Artery Compression by Right Ventricle-to-Pulmonary Artery Conduit Relieved by Anterior Translocation of the Right Pulmonary Artery

Pulmonary artery translocation has been described as an alternative surgical strategy to treat anomalous aortic origin of the coronary artery from the wrong sinus of Valsalva. We describe another application of this strategy in a patient who underwent complete repair of pulmonary atresia with ventricular septal defect and presented years later with ischemic symptoms due to compression of the left main coronary artery by the right ventricular-to-pulmonary artery conduit. To our knowledge, this is the first application of the pulmonary artery translocation technique to treat such a problem.

Anomalous aortic origin of a coronary artery in siblings with Marfan syndrome

We report a case of two brothers with Marfan syndrome and coexistent anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva. To our knowledge, this is the first ever report of siblings with Marfan syndrome who also have anomalous aortic origin of a coronary artery. The treatment and management options in the context of sudden death risk are discussed.

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.

The registry of anomalous aortic origin of the coronary artery of The Congenital Heart Surgeons’ Society

The anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva with interarterial, intramural, and/or intraconal course is a rare congenital anomaly that is associated with a high risk of sudden death in children. The Congenital Heart Surgeons' Society established the Registry of Anomalous Aortic Origin of the Coronary Artery to help determine the outcome of children and young adults managed with surgical intervention versus observation and to test the hypothesis that subsets of patients with anomalous aortic origin of a coronary artery can be identified in whom the risk of intervention is less than the risk of observation. All institutional members of the Congenital Heart Surgeons' Society were recruited for participation. The registry consists of a retrospective cohort of patients diagnosed between 1 January, 1998 and 20 January, 2009 and a prospective, population-based cohort of patients newly diagnosed from 21 January, 2009 onwards. Baseline demographics, diagnoses, and results of tests will be obtained through a review of the medical records. Annual follow-up data will be collected. Data will be analysed for different factors of risk at diagnosis, different strategies of treatment, and the impact of both on the outcomes of the patients. As of June 2010, 28 institutions had applied for approval from their institutional review board and 16 institutions had received approval from their institutional review board. Seventy-four patients have enrolled to date. We hope to use the established Pediatric Cardiomyopathy Registry as a guide to successful implementation, with a cooperative effort between institutions. The overall purpose of the Registry of Anomalous Aortic Origin of the Coronary Artery is to determine the outcome of surgical intervention versus observation in children and young adults with anomalous aortic origin of a coronary artery, and to describe the natural and "unnatural" history of these patients over the course of their lifetime. In this report, we describe the creation and design of the Registry of Anomalous Aortic Origin of the Coronary Artery. Data from the registry will be published at a later date.

Congenital anomalies of coronary arteries

After hypertrophic cardiomyopathy, coronary artery anomalies of origin from the wrong sinus of Valsalva are the second most common cause of sudden death on the athletic field in the USA. Although the right coronary artery arising from the left coronary sinus (ARCA) is four times as common as the left coronary artery arising from the anterior sinus (ALCA), it is the latter that is by far the more common cause of sudden death with or shortly after vigorous physical activity. Of the four types of ALCA, the interarterial type, where the left coronary artery passes anteriorly between the aorta and the right ventricular outflow tract, is the only type that places the patient at risk of sudden death. Another feature of this syndrome is the fact that sudden death occurs associated with or shortly after vigorous exercise and is very unusual after the patient is > 35 years of age. The mechanism by which there is sudden occlusion of the interarterial coronary artery is at present unknown, although there are a number of hypotheses involving the oblique passage of the vessel as it leaves the aorta. Sudden death is probably rare considering the number of people who have these anomalies. Symptoms premonitory to a fatal event such as exertional syncope, chest pain, or palpitations are probably common in patients at risk, and surgical correction is indicated in symptomatic patients at any age. In older asymptomatic patients, surgery is not recommended, since the incidence of sudden death in this age group is extremely small. In asymptomatic young patients, a stress test, preferably with radioisotope myocardial perfusion imaging or stress echocardiogram, should be done and surgical correction performed in those with ischemia provoked in the appropriate myocardial region. Since there is evidence that in patients who have survived a potentially fatal event, it is rare to be able to provoke ischemia with equal or greater exercise than had precipitated the malignant arrhythmia, the decision to surgically correct an asymptomatic young patient, serendipitously found to have ALCA, who has a negative exercise test, is debatable. Any decision for surgery in such patients should be made only after a full discussion of the risks pro and con surgery with the patient and the patient's family.

A simple correction for anomalous coronary arteries in adults

Although the risk of sudden death in children and young adults with anomalous coronary arteries arising from the wrong sinus of Valsalva is well described, the risk for older adults who present with such anatomy is unclear. Age greater than 30 years has been shown to impart a diminished risk of sudden death, although it can occur in older adults. Although simple coronary artery bypass for these patients has been described, the outcome can be hampered by competitive flow in the absence of concomitant coronary disease, and poor long-term patency can be expected. Other techniques include internal unroofing, either across the commissural post or not, with a possible effect on aortic valve function. We report a simple physiologic approach previously described in children and adolescents and now used in 2 cases of symptomatic adult patients with anomalous coronary arteries arising from the wrong sinus of Valsalva with an intra-arterial course between the pulmonary artery and aorta.

Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy

The most commonly reported coronary artery malformation leading to sudden death in children and young athletes is an anomalous coronary origin from the wrong aortic sinus, with or without a proximal course of the coronary artery between the aorta and the pulmonary artery. Although the incidence is difficult to assess, there is a 0.17% incidence in autopsy series (all variants included) and a 0.1 to 0.3% in catheter-echo diagnostic series. There may be a regional variation. In a prospective study of 1950 consecutive patients undergoing coronary angiography, anomalous origin of the right coronary artery from the left aortic sinus was found in 0.92% of cases, whereas the incidence of left coronary artery arising from the right aortic sinus was 0.15%. 1 In a study of 2388 patients who had transthoracic echocardiographic examination of their proximal coronary anatomy as part of innocent murmur investigation and evaluation of performance status, four patients (0.2%) were found to have an anomalous origin of the coronary artery from the wrong sinus. 2 Whatever the true prevalence, this lesion predisposes to fatal episodes of myocardial ischemia that may occur during or shortly after strenuous exercise. 3-5 Timely diagnosis requires a high index of suspicion but is frequently impossible, as the majority of patients with the anomaly are asymptomatic and thus do not seek medical attention. Therefore, sudden death is frequently the first manifestation, especially in young athletes, with only 30% of patients reporting prodromal symptoms. 5,6 Screening young adults before participation in competitive sports has been unsuccessful. Stress testing is unreliable because of high false-positive and falsenegative results; echocardiogram, echocardiography, and stress testing all lack predictive value. 4,5 Finally, although the major association of sudden death is with young athletes, cases have also been reported in children under 1 year of age.

Anomalous Coronary Artery From the Wrong Sinus of Valsalva: A Physiologic Repair Strategy

A coronary artery arising from the wrong aortic sinus, with or without a proximal course between the aorta and the pulmonary artery, may predispose to myocardial ischemia, myocardial infarction, or sudden death. We repaired this lesion in 5 symptomatic patients, all of whom had experienced exercise-related chest pain, dyspnea, or syncopal episodes. All patients underwent patch angioplasty of the proximal anomalous coronary artery with glutaraldehyde-treated autologous pericardium. When the coronary artery coursed between the aorta and the main pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary artery to create additional space and prevent compression. All patients recovered well and to date have demonstrated no evidence of recurrent myocardial ischemia. The combination of coronary artery angioplasty and translocation of the pulmonary artery seems to effectively address all the mechanisms that can generate ischemia.

ANOMALOUS CORONARY ARTERIES OF AORTIC ORIGIN

SUMMARYAnomalous coronary arteries cause only uncharacteristic symptoms and are therefore often an incidental finding during conventional coronary angiography, with an incidence of 0.3‐0.8%. The commonest anomaly is an aberrant origin of the main left or right coronary artery from the wrong sinus of Valsalva. Rarely there is a fistula draining into one of the cardiac cavities (right ventricle, right atrium, left ventricle or, rarely, superior vena cava) or displaced connection, as seen in anomalous origin of coronary artery from the pulmonary artery, resulting in a left‐to‐right shunt. In congenital heart disease, especially Fallot's tetralogy, the incidence of abnormal coronary arteries may be 2% or more. The proximal course in the former category may be misdiagnosed in up to 50% of cases. Aortic root injection with subtraction angiography, further detailed investigation with transoesophageal echocardiography or magnetic resonance angiography are therefore required as these have potential implications on subsequent surgery. Because of the abnormal course between aorta and pulmonary artery/outflow tract of the right ventricle and acute angulation there is a risk of angina, acute myocardial infarction or sudden death during or after exercise. It is therefore important to identify the exact cardiac anatomy, particularly in patients undergoing angioplasty, stenting or cardiac surgery. (Int J Clin Pract 2000; 54(6): 390‐394)