Aortic Origin Research Articles

Neo-Ostium Creation for Anomalous Aortic Origin of the Coronary Artery

For the anomalous aortic origin of the coronary artery with interarterial course, surgical repair is recommended. We describe a technique of neo-ostium creation at the correct anatomic position for the right coronary artery arising from a single left coronary ostium without an intramural course and coursing between the ascending aorta and the main pulmonary artery.

Computed tomography in the evaluation of the anomalous origin of the coronary artery: coexistence with other congenital heart disease in an adult population.

The aim of the study is to assess the prevalence of anomalous origin of the coronary artery in cardiac computed tomography (CT) and to evaluate its coexistence with other congenital heart disease. 7,115 patients, who underwent 64-slice or dual source cardiac CT in the years 2005-2011 were screened for the presence of anomalous origin of the coronary artery. Anomalous origin of coronary artery was found in 62 (0.87%) patients (34 males, mean age 57.4 ± 15.1 years). Anomalous aortic and pulmonary origin of coronary artery concerned 59 (0.83%) and 3 (0.04%) cases, respectively. Concomitant heart defects were observed in 5 (0.07%) patients, all with anomalous aortic origin of coronary artery. Malformations included transposition of great arteries (4 patients) and single ventricle in (1 patient). The incidence of anomalous origin of a coronary artery in cardiac CT is similar to invasive coronary angiography. In an adult population the vast majority of those anomalies are isolated abnormalities without concomitant other congenital heart defects.

A minimally invasive, algorithm-based approach for anomalous aortic origin of a coronary artery.

Operative repair for anomalous aortic origin of a coronary artery (AAOCA) has been described using various innovative techniques. Common to each series is the use of a full sternotomy. As demand for minimally invasive approaches to adult cardiac surgery has increased, the upper hemisternotomy has emerged as a safe and effective technique for aortic valve and root replacement. This report reviews our results and describes the application of an upper hemisternotomy to an algorithm-based surgical approach for AAOCA. From January 2012 to March 2013, the aortic root was approached via a 7-cm skin incision and upper hemisternotomy for all patients undergoing repair of an AAOCA. The type of repair performed was in accordance with a predefined surgical algorithm. The anomalous vessel had a slit-like ostium and followed a supracommissural intramural course in three patients with symptomatic anomalous right coronary artery. These patients underwent coronary unroofing. In contrast, a patient with an anomalous left coronary artery presented without an intramural segment and underwent vessel translocation and reimplantation. All patients underwent AAOCA repair according to our surgical algorithm and via an upper hemisternotomy. The median length of stay was 4 days. All patients had resolution of symptoms, and there were no reported complications at a median follow-up of 16.5 months. This series describes a minimally invasive approach to AAOCA repair. When used in conjunction with a defined surgical algorithm, this technique enables a safe and effective repair in all forms of AAOCA without concomitant coronary artery disease.

STANDARDIZED APPROACH TO PATIENTS WITH ANOMALOUS AORTIC ORIGIN OF A CORONARY ARTERY: RESULTS FROM THE CORONARY ANOMALIES PROGRAM AT TEXAS CHILDREN’S HOSPITAL

Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data is lacking. Prospective, observational study of patients (pts) seen with AAOCA from 12/2012 to 04/2014. The

A Minimally Invasive, Algorithm-Based Approach for Anomalous Aortic Origin of a Coronary Artery

A Minimally Invasive, Algorithm-Based Approach for Anomalous Aortic Origin of a Coronary Artery

Surgical treatment of aberrant aortic origin of coronary arteries.

Aberrant origin of the coronary arteries is rare but can be life threatening. It is an important cause of sudden death in athletes and other young adults, and may be treated surgically. Consensus exists that interarterial left coronary artery (LCA) should be surgically repaired. For interarterial right coronary artery (RCA), the discussion remains open. The purpose of this study was to analyse our surgical experience. From 2001 until 2014, 31 patients were operated for interarterial RCA, interarterial LCA or intraseptal course of the LCA. Twenty-six patients had interarterial RCA, 4 patients interarterial LCA and 1 patient an intraseptal course of the LCA. Median age at operation was 38 years (range 9-66 years). Twenty-eight patients had previous or current symptoms. The most important were a life-threatening event with resuscitation in 3 and myocardial infarction in 3 others. Surgical repair of interarterial RCA consisted of unroofing of the ostium with or without reimplantation in 25 patients and CABG on the RCA with a venous graft in 1 patient. Reconstruction of interarterial LCA consisted of ostium reconstruction of the LCA with a venous patch in 4 patients. The patient with an intraseptal course had a complete release of the LCA out of the septum and reimplantation in the correct coronary sinus. Follow-up was done by analysis of outpatient records, direct patient contact, echocardiography, electrocardiography, CT-angiography and an exercise test. Median follow-up was 6 years (range 0-11 years). One patient was lost to follow-up. No early or late mortality occurred. Three patients had ischaemia with ventricular fibrillation or ventricular tachycardia shortly after surgery. Two were immediately reoperated, 1 had a stent implantation 1.5 months after release of intraseptal LCA. Two of these patients show a slight dysfunction of the left ventricle at follow-up. All other patients are asymptomatic. Surgery for aberrant origin of coronary arteries is safe. There is a risk of cardiac ischaemia shortly after operation, especially in LCA reconstruction. We strongly believe that a slit-like coronary ostium and an intramural aortic course is an absolute indication for surgical repair, also in asymptomatic aberrant RCA.

Management of acute aortic syndrome.

Acute aortic syndrome (AAS) encompasses a group of severe, life-threatening disorders of the aorta, including acute aortic dissection, intramural haematoma (IMH), and penetrating aortic ulcer (PAU). The concept of AAS was developed to enable the early identification and definitive treatment of patients with chest pain from an aortic origin. Aortic dissection is the most common form of AAS, followed by IMH and PAU. Congenital cardiovascular defects, genetic syndromes, and nonsyndromic genetic variants have all been linked with the development of AAS. The diagnosis of AAS in the clinic can be made using imaging modalities such as CT, echocardiography, and MRI. The initial management of patients with AAS is focussed on the control of blood pressure to reduce aortic wall stress. A multidisciplinary team is required to assess each patient and decide whether endovascular or open surgical treatment, or further medical management is indicated. The optimal treatment of patients with AAS remains a challenging clinical dilemma, and further studies are required to fully characterize conditions within the AAS spectrum and to design individualized, patient-centred treatment plans.

Abstract 15789: Cardiac MRI With Adenosine Identifies Coronary Stenosis, Perfusion Defects, and Delayed Enhancement in Pediatric Patients With Anomalous Aortic Origin of a Coronary Artery Before and After Surgical Repair

Background: Anomalous aortic origin of a coronary artery (AAOCA) with an interarterial course is associated with sudden cardiac death in children. Objectives: Using cardiac MRI with adenosine, we evaluated coronary ostial stenosis, proximal coronary size, and left ventricular (LV) function in children with AAOCA. Methods: We prospectively enrolled children 5-18 years old with interarterial AAOCA. MRIs were reviewed for coronary artery origin, proximal course, dimensions, and cardiac function. Surgery consisted of the modified unroofing procedure. We used descriptive statistics and paired t-tests to evaluate for statistical significance. Results: Between 2/2009 and 5/2014, 24 subjects with AAOCA underwent 29 MRIs. The majority were male (N=19, 79%) with anomalous right coronary artery (AAORCA, N=20, 83%). Mean age was 12.8 years at time of initial MRI. MRI was performed an average of 7 months post-operatively in 8 subjects who underwent surgery. In all, the proximal anomalous coronary arose tangential to the aorta with an elliptical, slit-like ostium. The anomalous coronary measured smaller proximally (0.20 mm) compared to distally (0.31 mm, P=< 0.0001), and after surgical repair, the post-operative origin was significantly larger (0.36 vs. 0.21 mm, P=0.02). Other abnormalities at initial MRI included fixed inferior wall (N=1) and reversible subendocardial septal/inferior wall (N=1) perfusion defects. Post-operatively, the neo-ostium was round in 6 (see Figure), but in 2, the orifice remained elliptical. One patient had a new small mid-myocardial scar and one had dyskinetic septal wall motion. LV function was normal both before and after surgery (mean ejection fraction =68.1% vs. 67.5%, P=0.85). Conclusions: Cardiac MRI with adenosine is an important tool for the evaluation of anomalous anatomy, myocardial function, and ischemia/injury and should be considered for the initial and, when applicable, post-operative assessment of children with AAOCA.

Repair of anomalous aortic origin of a coronary artery in 113 patients: a Congenital Heart Surgeons' Society report.

Anomalous aortic origin of a coronary artery (AAOCA) encompasses a wide morphologic spectrum, which has impeded consensus regarding indications for the diverse repair strategies. We constructed a profile of current surgical techniques and explore their application to morphologic variants. Patients<30 years old (n=113) with isolated AAOCA who underwent operations at 29 Congenital Heart Surgeons Society (CHSS) institutions from 1998 to 2012 were identified from the CHSS AAOCA Registry. Operative findings were related to surgical techniques at index repairs by cross-tabulation. Anomalous origin of the left main or left anterior descending coronary artery was present in 33 (29%) patients and of the right coronary artery in 78 (69%) patients; 2 arteries originated directly above the commissure between the left and right sinuses. There were 101 (89%) interarterial and intramural (IA/IM) arteries, 10 (9%) were interarterial but not intramural (IA/NIM) and 2 (2%) were neither interarterial nor intramural. Intramural arteries were unroofed in 100 (88%) operations, usually with intimal tacking after incision (n=47) or excision (n=25) of the common wall. Coronary reimplantation (n=11), pulmonary artery relocation (n=7; 5 for IA/NIM), simple ostioplasty (without unroofing; n=3), coronary artery bypass grafting (n=2), and ostial window (n=1) were less common. In 37 (33%) operations, a valvar commissure was taken down; 33 were resuspended. Current surgical repair of AAOCA is individualized to morphology, particularly the presence of intramural and/or interarterial segments. This report is foundational for future planned CHSS studies that will examine interventional and noninterventional outcomes and ultimately guide management of AAOCA.

Abstract LB-146: Investigation of Gas6/Axl signaling in clear cell renal cell carcinoma tumor cells and endothelial cells in presence of the first line treatment drug Sunitinib Malate

Abstract Introductory sentence: The aim is to investigate whether Gas6 dependent Axl activation and downstream signaling can occur in clear cell Renal Cell Carcinoma tumor cells (ccRCCs) and Endothelial cells (ECs) during treatment with Sunitinib Malate. Experimental set up: The ccRCC cell line 786-O and ECs of human aortic origin (HAECs) were grown in vitro, serum-starved over night and pre-incubated with Sunitinib Malate for 2 hours prior 100 ng/mL Gas6 stimulation for 10 minutes. Activation of Axl in terms of induced tyrosine phosphorylation was determined by immunoprecipitation and immunoblotting. Gas6-induced downstream signaling of Axl was analyzed by immunoblotting for phosphorylation of Akt and Erk protein in total cell lysates. Gas6/Axl specific signaling was verified by pre-incubation with an Axl specific kinase inhibitor, R428. Functionality of Sunitinib Malate was verified by inhibition of VEGFR-activation in ECs. In addition, prolonged stimulation of ccRCCs with Gas6 only was analyzed up to 2 days. Longterm effects on induced Axl activation and downstream Akt and Erk signaling in ccRCCs were investigated as described above. Results: In ccRCCs and ECs, Gas6-induced Axl activation and downstream signaling are resistant to physiologically relevant and higher doses of Sunitinib Malate. Gas6/Axl-signaling activates Akt and Erk pathways in presence of Sunitinib Malate. Sunitinib Malate contributes to Gas6 signaling with an increased phospho-Axl, Akt and Erk activation in ccRCCs and ECs. Axl is constitutively activated in ccRCCs upon longterm Gas6 stimulation, with early peak Erk activation and sustained Akt activation. Conclusions and importance: RCC is a lethal cancer with bad prognosis due to development of chemoresistance and recurrence of a more aggressive tumor. The Gas6/Axl system influences many cellular processes, including survival, migration and invasion, contributing to both chemoresistance and metastasis of several tumor types. Axl overexpression is also associated with poor patient outcome. Our data indicates that the Gas6/Axl system can be constitutively activated in ccRCCs with sustained Axl activation and downstream signaling of pathways known to apply survival, growth and proliferative advantage to tumor cells. Sunitinib Malate, first-line anti-angiogenic targeted therapy drug used in advanced RCC, does not inhibit and moreover, increases Gas6/Axl signaling in both ccRCCs and ECs. Importantly this suggests that, Sunitinib Malate, while targeting ECs and tumor-angiogenesis, simultaneously possibly provide a pro-tumorigenic tumor microenvironment where Gas6/Axl signaling, not inhibited by Sunitinib Malate, stimulates ccRCCs and ECs more intensively than in the non-treated tumor microenvironment. Altogether, Gas6/Axl signaling during targeted therapy is possibly contributing to the acquired chemoresistance and recurrence of aggressive tumors in patients treated for advanced RCC. Citation Format: Anna Gustafsson, Björn Dahlbäck. Investigation of Gas6/Axl signaling in clear cell renal cell carcinoma tumor cells and endothelial cells in presence of the first line treatment drug Sunitinib Malate. [abstract]. In: Proceedings of the 105th Annual Meeting of the American Association for Cancer Research; 2014 Apr 5-9; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2014;74(19 Suppl):Abstract nr LB-146. doi:10.1158/1538-7445.AM2014-LB-146

Utility and importance of new echocardiographic screening methods in diagnosis of anomalous coronary origins in the pediatric population: assessment of quality improvement.

Anomalous aortic origin of coronaries from the contralateral sinus (AAOCA) is rare but an important cause of cardiac death in the otherwise healthy young athlete. This necessitates prompt, accurate identification; transthoracic echocardiography (TTE) remains the primary screening tool. Assessment of accuracy is difficult since the true prevalence of the disease is unknown, with estimates at 0.3-1.07%. The incidence by TTE remains much lower, between 0.09 and 0.17% even with sophisticated equipment and a high index of suspicion. Our goal was to incorporate two new screening views to our standard TTE protocol and assess improvement in diagnosis of AAOCA by TTE in our laboratory. Recently (2011), we incorporated two new screening methods to standard protocol. The parasternal short axis sweep is extended to visualize the anomalous segment arising superiorly from the ascending aorta before exiting the root at a site close to a 'normal' origin. Secondly, the anomalous, interarterial coronary demonstrates an anterior, steep-angled course visualized in the parasternal long axis between the aorta and pulmonary artery. The echocardiogram database was searched for patients newly diagnosed with AAOCA in 2010 (prior to incorporation of new methods) and 2012. AAOCA incidence in our patient population improved from 0.02% (2010) to 0.22% (2012), age range from 4 days to 17 years. Teenagers and symptomatic patients with anomalous right coronary origin (6) underwent additional confirmatory imaging, and three underwent surgery. One patient with anomalous left coronary origin underwent surgical repair. Addition of the screening views can significantly increase the sensitivity of TTE in diagnosing AAOCA in the asymptomatic patients. We propose that these views be incorporated into the standard TTE evaluation of coronary arteries.

Direct Reimplantation as an Alternative Approach for Treatment of Anomalous Aortic Origin of the Right Coronary Artery

Anomalous aortic origin of the coronary artery (AAOCA) is a rare cardiac anomaly associated with myocardial ischemia, infarction, and even sudden death. We report 2 patients presenting with AAOCA of the right coronary artery originating from the left coronary sinus with an initial intramural course. In both patients, we performed uncomplicated direct reimplantation of the right coronary artery into the right coronary sinus. For this purpose, the right coronary artery was dissected from the transmural emersion point out of the aortic wall, subsequently as proximal as possible transected and directly reimplanted into the right coronary sinus. In both cases, the postoperative course was uneventful, and cardiac computed tomography confirmed excellent postoperative results after 1 year of follow-up. This technique is a convenient and easily performable approach, characterized by short cross-clamp times and avoidance of aortotomy, and thus is an excellent alternative strategy.

Surgical Unroofing of Anomalous Aortic Origin of a Coronary Artery: A Single-Center Experience

Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The optimal management of patients with AAOCA is controversial. We examined our experience with surgical unroofing of AAOCA to determine the midterm effect of surgical repair. From October 1992 through December 2011, 75 patients with AAOCA underwent surgical unroofing. Mean age was 39.6 ± 19.6 years; 23 patients (32%) were aged younger than 30 years. Angina, shortness of breath, or syncope was present in 55 patients (72%); 2 (3%) had history of sudden cardiac arrest. Of 40 patients (53%) who had preoperative stress tests, results were abnormal in 20 (50%). Coronary or computed tomography angiography demonstrated an anomalous right coronary artery (RCA) arising from the left sinus in 69 patients (92%) and the left main coronary artery arising from the right sinus in 6 (8%). Two patents (3%) were referred for recurrent anginal symptoms after previous RCA bypass with the right internal mammary artery. Minimally invasive partial upper sternal split was performed in 17 patients (22%). Two patients (3%) needed right internal mammary artery-to-RCA grafting due to flow acceleration at the RCA ostium. There were no early deaths. One late death (1%) occurred related to noncardiac causes. At follow-up (mean, 18 months; maximum, 7 years), all patients remained free of cardiac symptoms. Surgical unroofing of AAOCA is associated with low morbidity and mortality. At intermediate follow-up, resolution of symptoms and freedom from sudden death can be expected. The threshold for offering intervention should be low.

Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.

In a fundamental 1974 article, Cheitlin et al1 of the Armed Forces Institute of Pathology emphasized the special role of anomalous aortic origin of the coronary arteries and differentiated this condition from the other coronary artery anomalies (CAAs) as being associated with an increased frequency of sudden cardiac death (SCD) in young persons, especially during strenuous exertion. More recently, CAAs have also been considered possible causes of clinically disabling symptoms, including dyspnea, angina pectoris, and syncope, especially in young adults.2–5 Clinicians and epidemiologists have identified the need to prevent not only SCD in young persons, especially athletes or military recruits, but also other CAA-related symptoms in persons of any age.6–12 Much of the available information concerning the incidence of SCD in carriers of CAAs is lacking a denominator (measure of the carriers at risk). The most notable study of SCD incidence is a classic 2004 article by Eckart et al,7 who reported the mortality rate (>25 years) that US military recruits experienced during a 2-month-long boot-camp training period. All the recruits were involved in strenuous exercise and had undergone routine screening based on a history and physical examination performed by general practitioners. Of the 23 million recruits, 64 died of SCD (0.28 per 100 000 per 2 months, or 1.68 per 100 000 per year). Of these deaths, 21 (33%) were attributed to CAAs, specifically anomalous origin of the left coronary artery from the right sinus of Valsalva with an interarterial course. To establish a solid theory and a preventive policy in this field, we must understand the pathophysiology of CAAs, identify the prevalence of individuals living with different kinds of CAAs (the denominator), clarify the individual severity of each case, study the influence of different types of exertion, and identify the …

Anatomical variations in the origins of the celiac axis and the superior mesenteric artery: MDCT angiographic findings and their probable embryological mechanisms.

To identify the spectrum and prevalence of anatomical variations in the origin of the celiac axis (CA), the superior mesenteric artery (SMA) and their major branches by using multidetector computed tomographic (MDCT) angiography. A retrospective evaluation was carried out on 1,500 abdominal MDCT angiography images. The aortic origins of the CA, the SMA and their major branch patterns were investigated. Normal aortic origins of CA and SMA were noted in 1,347 (89.8%) patients. Seven types of CA and SMA origin variants were identified in 153 (10.2%) patients. The three most common variations were hepatomesenteric trunk (67 patients, 4.47%), celiomesenteric trunk (CMT) (51 patients, 3.4%) and splenomesenteric trunk (18 patients, 1.2%). An evaluation of CMT was classified as long (34 patients, 66.7%) or short (17 patients, 33.3%) subtypes, compared with the length of the common trunk. Further CMT classification was based on the origin of the left gastric artery: subtype I, 26 patients (53.1%); subtype II, 5 patients (10.2%); subtype III, 15 patients (30.6%); subtype IV, 3 patients (6.1%). Dislocation interruption, incomplete interruption and persistence of the longitudinal anastomosis could be the embryological mechanisms of the variant origins of the CA, the SMA and their major branches. • Aortic origins of CA, SMA and their major branches were investigated. • Celiomesenteric trunk includes several different subtypes and configurations. • Probable embryological mechanisms of origin variants in these observed arteries were discussed. • Origin variants in these observed arteries have wide-ranging health implications.

Linking the Congenital Heart Surgery Databases of the Society of Thoracic Surgeons and the Congenital Heart Surgeons’ Society

A link has been created between the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the Congenital Heart Surgeons' Society Database (CHSS-D). Five matrices have been created that facilitate the automated identification of patients who are potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices are now used to (1) estimate the denominator of patients eligible for CHSS studies and (2) compare "eligible and enrolled patients" to "potentially eligible and not enrolled patients" to assess the generalizability of CHSS studies. The matrices were applied to 40 consenting institutions that participate in both the STS-CHSD and the CHSS to (1) estimate the denominator of patients that are potentially eligible for CHSS studies, (2) estimate the completeness of enrollment of patients eligible for CHSS studies among all CHSS sites, (3) estimate the completeness of enrollment of patients eligible for CHSS studies among those CHSS institutions participating in each CHSS cohort study, and (4) compare "eligible and enrolled patients" to "potentially eligible and not enrolled patients" to assess the generalizability of CHSS studies. The matrices were applied to all participants in the STS-CHSD to identify patients who underwent frequently performed operations and compare "eligible and enrolled patients" to "potentially eligible and not enrolled patients" in following five domains: (1) age at surgery, (2) gender, (3) race, (4) discharge mortality, and (5) postoperative length of stay. Completeness of enrollment was defined as the number of actually enrolled patients divided by the number of patients identified as being potentially eligible for enrollment. For the CHSS Critical Left Ventricular Outflow Tract Study (LVOTO) study, for the Norwood procedure, completeness of enrollment at centers actively participating in the LVOTO study was 34%. For the Norwood operation, discharge mortality was 15% among 227 enrolled patients and 16% among 1768 nonenrolled potentially eligible patients from the 40 consenting institutions. Median postoperative length of stay was 31 days and 26 days for these enrolled and nonenrolled patients. For the CHSS anomalous aortic origin of a coronary artery (AAOCA) study, for AAOCA repair, completeness of enrollment at centers actively participating in the AAOCA study was 40%. Determination of the denominator of patients eligible for CHSS studies and comparison of "eligible and enrolled patients" to "potentially eligible and not enrolled patients" provides an estimate of the extent to which patients in CHSS studies are representative of the overall population of eligible patients; however, opportunities exist to improve enrollment.

Evaluation of effects of bronchiectasis on bronchial artery diameter with multidetector computed tomography

Among patients with increased bronchial artery diameter there is a significant association between hemoptysis and bronchiectasis score. The higher score of bronchiectasis, the higher risk of hemoptysis development. To investigate the association of stages of bronchiectasis based on a computed tomography (CT) grading system, with bronchial artery diameter and hemoptysis in patients with bronchiectasis. Patients with lung pathologies other than bronchiectasis, which may cause hemoptysis, were excluded from the study. One hundred and forty-five patients who underwent contrast-enhanced thorax CT by a 64-detector CT for various indications, and who were diagnosed with bronchiectasis, were evaluated retrospectively. CT examinations were carried out by two radiologists with 9 and 4 years of experience with chest radiology, respectively. The diameters of the right and left bronchial arteries were measured 1 cm from the aortic origin and perpendicular to the vessel axis. Cases were assessed based on the Bhalla CT scoring system. The hemoptysis history of every patient was taken. The diameters of the right and left bronchial arteries were significantly greater in patients with scores of 2 and 3 bronchiectasis than in patients with a score of 1. This was significantly greater in patients with a score of 3 than in patients with a score of 2 (P < 0.05). In patients with a score of 1, the right bronchial artery diameter was significantly greater than that of the left bronchial artery (P < 0.05). Right bronchial artery diameters were significantly greater than left bronchial artery diameters in score 3 patients (P < 0.05). A significant association was observed between hemoptysis and bronchiectasis in patients with increased bronchial artery diameter (P < 0.05). In patients with bronchiectasis, as the stage of bronchiectasis increases, the bronchial artery diameters and the risk of hemoptysis increase. We think that in patients who are diagnosed with bronchiectasis via multidetector CT (MDCT), based on scoring with bronchial artery diameters, the risk of hemoptysis can be estimated, and early management plans can be implemented.

Les anomalies de naissance des artères coronaires à partir de l’aorte : une cause fréquente et évitable de mort subite

Anomalous aortic origin of coronary arteries is a congenital heart defect in which one coronary artery arises from the opposite sinus (the left coronary artery from the right coronary sinus, or the right coronary artery from the left sinus). The initial segment of the abnormal artery courses between the great arteries and is usually located within the aortic wall. These anomalies are far from rare, affecting 0.1% to 0.3% of the population, and carry a high risk of sudden cardiac death. It is thought that the main mechanism of cardiac death is external compression of the abnormal coronary artery between the great arteries as they expand during strenuous exercise. The risk of sudden death is particularly high in patients with an anomalous left coronary artery or associated anatomic lesions (stenotic intramural segment, slit-like deformation of the ostium), and also in young athletes. A common presentation is sudden unexplained death. The diagnosis can be made by echocardiographic examination. Silent myocardial ischemia must be sought, with stress echocardiography and stress nuclear imaging. Various surgical techniques have been described. Our own technique involves the creation of a neo-ostium in the appropriate sinus. Surgical treatment is relatively safe and provides satisfactory results, most patients having no residual myocardial ischemia under stress. Surgery is recommended for all patients with an anomalous left coronary artery, regardless of symptom status, as well as for symptomatic patients with an anomalous right coronary artery (aborted sudden death, symptoms under stress, myocardial ischemia under stress). Because of the incidence and severity of these anomalies, careful echocardiographic evaluation should be performed routinely, at least in young athletes.

Anomalous aortic origin of coronary arteries: 'anatomical' surgical repair

Anomalous aortic origin of coronary arteries (left coronary from right sinus or right coronary from left sinus) is a rare congenital defect, which carries a high risk of sudden cardiac death. The risk is particularly high when the interarterial course between the great arteries has an intramural segment, or is hypoplastic/stenotic, or has an abnormal orifice. Various surgical techniques have been used, including coronary artery bypass grafting, pulmonary artery translocation, partial or complete unroofing of the intramural course and patch enlargement of the interarterial course. We favour 'anatomical' repair that creates an enlarged neo-ostium into the appropriate sinus, eliminates completely the intramural segment and restores a normal angle of take-off. Reimplantation of the anomalous coronary artery may be indicated in variants without an intramural course. Surgical correction is mandatory for symptomatic and asymptomatic patients with evidence of myocardial ischaemia under stress; it is recommended in asymptomatic patients with high-risk variants (anomalous left coronary artery with the intramural course), particularly in young patients with strenuous activities.

Molecular identification of venous progenitors in the dorsal aorta reveals an aortic origin for the cardinal vein in mammals

Coordinated arterial-venous differentiation is crucial for vascular development and function. The origin of the cardinal vein (CV) in mammals is unknown, while conflicting theories have been reported in chick and zebrafish. Here, we provide the first molecular characterization of endothelial cells (ECs) expressing venous molecular markers, or venous-fated ECs, within the emergent dorsal aorta (DA). These ECs, expressing the venous molecular markers Coup-TFII and EphB4, cohabited the early DA with ECs expressing the arterial molecular markers ephrin B2, Notch and connexin 40. These mixed ECs in the early DA expressed either the arterial or venous molecular marker, but rarely both. Subsequently, the DA exhibited uniform arterial markers. Real-time imaging of mouse embryos revealed EC movement from the DA to the CV during the stage when venous-fated ECs occupied the DA. We analyzed mutants for EphB4, which encodes a receptor tyrosine kinase for the ephrin B2 ligand, as we hypothesized that ephrin B2/EphB4 signaling may mediate the repulsion of venous-fated ECs from the DA to the CV. Using an EC quantification approach, we discovered that venous-fated ECs increased in the DA and decreased in the CV in the mutants, whereas the rest of the ECs in each vessel were unaffected. This result suggests that the venous-fated ECs were retained in the DA and missing in the CV in the EphB4 mutant, and thus that ephrin B2/EphB4 signaling normally functions to clear venous-fated ECs from the DA to the CV by cell repulsion. Therefore, our cellular and molecular evidence suggests that the DA harbors venous progenitors that move to participate in CV formation, and that ephrin B2/EphB4 signaling regulates this aortic contribution to the mammalian CV.