Aortic Origin Research Articles

Congenital coronary artery anomalies

Abstract Congenital coronary artery anomalies are abnormalities in the origin, course or structure of these arteries, and their incidence varies from 0.2% to 5.6%. Although the majority of anomalies are asymptomatic, they are the second most common cause of sudden cardiac death in young athletes. The aim of this study is to highlight the main anomalies with hemodynamic significance, particularly anomalous aortic origin of coronary arteries and anomalous left coronary artery from the pulmonary artery. Anomalous aortic origins of coronary arteries account for 14–17% of all sudden cardiac deaths that unexpectedly occur in healthy children or young athletes during or immediately after physical exercise. The mechanism responsible for the compression/occlusion of the coronary artery originating from the opposite sinus is still unclear and several mechanisms have been proposed. The clinical presentation of these patients varies and physical examination is normal in most cases. Transthoracic echocardiography is the most commonly used test for diagnosis. The treatment, management and follow-up of these patients are the subject of debate. The anomalous origin of the left coronary artery arising from the pulmonary artery is an even rarer condition and, without corrective surgery, most patients die within the first year of life. Echocardiography is also the method of choice to confirm this condition. The diagnosis of this anomaly in a seriously ill child is an indication for urgent surgery. Due to the hemodynamic abnormalities caused by these abnormalities, early diagnosis and treatment are important.

USE OF MULTIMODALITY IMAGING AND 3-DIMENSIONAL PRINTING TO GUIDE MANAGEMENT OF INTERARTERIAL ANOMALOUS RIGHT CORONARY ARTERY

The risk stratification and management of anomalous aortic origin of a coronary artery with an interarterial ± intramural course remains controversial. A 47-year-old man presented with new onset palpitations. He was previously healthy with an active lifestyle and no exertional complaints. Physical

SUDDEN CARDIAC DEATH AND ABORTED SUDDEN CARDIAC DEATH IN PATIENTS WITH ANOMALOUS AORTIC ORIGIN OF A CORONARY ARTERY (AAOCA) FROM THE INAPPROPRIATE SINUS OF VALSALVA: A SYSTEMATIC REVIEW OF THE LITERATURE

Anomalous aortic origin of a coronary artery (AAOCA) with an interarterial and/or intramural course is a potential cause of sudden cardiac death (SCD). Yet, risk stratification and management of these patients remains controversial. We performed a systematic review of AAOCA patients with SCD or

INTRAMYOCARDIAL CORONARY ARTERIES IN CHILDREN: NOT ALL ARE BENIGN BUT MOST ALLOW RETURN TO EXERCISE

Intramyocardial (IM) coronary arteries are characterized by a segment of a coronary artery (CA) that travels into the myocardium instead of the normal epicardial course. It may occur in isolation or in association with anomalous aortic origin of a CA (AAOCA). They are uncommon lesions in children

Patients with anomalous aortic origin of the coronary artery remain at risk after surgical repair

ObjectivesAnomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare cardiac anomaly associated with sudden cardiac death (SCD). Single-center studies describe surgical repair as safe, although medium- and long-term effects on symptoms and risk of SCD remain unknown. We sought to describe outcomes of surgical repair of AAOCA. MethodsWe reviewed institutional records for patients who underwent AAOCA repair, from 2001 to 2016, at 2 affiliated institutions. Patients with associated heart disease were excluded. ResultsIn total, 60 patients underwent AAOCA repair. Half of the patients (n = 30) had an anomalous left coronary artery arising from the right sinus of Valsalva and half had an anomalous right. Median age at surgery was 15.4 years (interquartile range, 11.9-17.9 years; range, 4 months to 68 years). The most common presenting symptoms were chest pain (n = 38; 63%) and shortness of breath (n = 17; 28%); aborted SCD was the presenting symptom in 4 patients (7%). Follow-up data were available for 54 patients (90%) over a median of 1.6 years. Of 53 patients with symptoms at presentation, 34 (64%) had complete resolution postoperatively. Postoperative mild or greater aortic insufficiency was present in 8 patients (17%) and moderate supravalvar aortic stenosis in 1 (2%). One patient required aortic valve replacement for aortic insufficiency. Two patients required reoperation for coronary stenosis at 3 months and 6 years postoperatively. ConclusionsSurgical repair of AAOCA is generally safe and adverse events are rare. Restenosis, and even sudden cardiac events, can occur and long-term surveillance is critical. Multi-institutional collaboration is vital to identify at-risk subpopulations and refine current recommendations for long-term management.

Diagnosis and treatment of anomalous aortic origin of a coronary artery from the opposite sinus in children and adolescents

Objective To observe the clinical characteristics and improve the diagnosis and treatment of anomalous aortic origin of a coronary artery from the opposite sinus (AAOCA) in children and adolescents. Methods Between January 2012 and December 2016, 11 children/adolescents [8 were boys, 3 were girls, age was (10.1±5.6) years old] diagnosed with AAOCA were retrospectively reviewed at Department Pediatric Cordiology, Guangdong General Hospital.The clinical features, transthoracic echocardiography (TTE), computed tomography images, electrocardiogram (ECG), cardinc troponin I(CTnI) and creatinine kinase-MB(CK-MB) were analyzed. Results The diagnosis of AAOCA was confirmed by TTE and computed tomography images in all 11 cases.Left coronary artery originated from the right sinus in 5 patients, and right artery coronary originated from the left sinus in 6 cases.Five patients with intramural course had the history of exercise-related syncope.Of these 5 cases with exercise-related syncope, 4 cases were diagnosed as left coronary artery originated from the right sinus and 1 case was diagnosed as right artery coronary originated from the left sinus.ECG, CTnI and CK-MB revealed acute myocardial ischemia in these patients with exercise-related syncope.Unroofing procedures were undergone in these 5 patients.The remaining 6 patients had no syncope and symptoms of cardia ischemia, sports activities were restricted and follow-ups were counseled in these 6 patients.There was no sudden death events later, and none of the patients demonstrated any evidence of new myocardial ischemia in the follow-up study. Conclusions AAOCA can be associated with syncope and myocardial ischemia in children and adolescents.The correct diagnosis of AAOCA requires a very high index of suspicion.Sports activities restriction and follow-up should be counseled in cases with AAOCA.Unroofing procedures should be performed in symptomatic cases with AAOCA and an intramural course. Key words: Coronary vessel anomalies; Exercise-related syncope; Sudden cardiac death; Adolescents; Child

Monomorphic Ventricular Tachycardia as the First Manifestation in a Patient with Anomalous Coronary Artery

Introduction Coronary artery anomalies comprise a heterogeneous group of rare congenital heart defects, being classified according to their origin, course and distal bed, with an incidence ranging between 0.3% and 1.5% in the overall population. Dodge-Khatami et al. subdivided them into seven categories, according to their clinical complexity: coronary arteries originating from the pulmonary artery, coronary arteries with anomalous aortic origin, congenital atresia of the left main coronary artery, coronary arteriovenous fistulas, coronary arteries forming myocardial bridges, coronary artery aneurysms [...]

Aortic Origin of Left Vertebral Artery: A Case Report

Aortic Origin of Left Vertebral Artery: A Case Report

Anomalous aortic origin of right and circumflex coronary arteries - procedural risks during combined aortic valve replacement and coronary artery bypass grafting

Introduction. Anomalous aortic origin of the right and the circumflex coronary arteries presents extremely rare and potentially dangerous combination in patients scheduled for combined coronary bypass grafting and aortic valve replacement surgery. We report this illustrative case to emphasize the importance of meticulous diagnostic setup enabling the surgeon to anticipate and avoid numerous possible pitfalls. Case outline. A 74-year-old woman, with anterior-wall myocardial infarction and aortic valve stenosis, underwent successful combined coronary artery bypass grafting and aortic valve replacement. Preoperative coronary angiography revealed unusually high take-off of the right main coronary trunk and anomalous origin and course of the circumflex coronary artery. Anatomy of both anomalous coronary arteries in the light of underlying surgical pathology necessitated a meticulous preparation and caution during successive phases of surgical treatment. Conclusion. Estimating potential procedural risk should be standard practice for each patient with known congenital coronary artery anomalies, regardless of the natural risk imposed by a particular anomaly. Preoperative evaluation of coronary circulation, with high surgical awareness and knowledge of different congenital coronary artery anomalies, should be a standard approach in cardiac surgical practice. This would add a predictive value for an actual procedural risk in cases of previously unrecognized anomalies.

Anomalous left coronary artery with single ostium (Shared with Right Coronary Artery) detected by echocardiography: Two case reports

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva has received increasing attention due to its association with malignant cardiac events in otherwise healthy and asymptomatic individuals. Despite this, we still face tremendous challenges in detecting the anomalies in a timely fashion to prevent sudden cardiac death. Here, we present two cases of anomalous left coronary artery with single ostium (shared with right coronary artery); in patients with normal cardiac function. The diagnosis of AAOCA in both cases was first suspected on transthoracic echocardiography and was later established using either coronary computed tomography angiography or invasive coronary angiography. Although angiography is the gold standard for diagnosing AAOCA, echocardiography is a more frequently used imaging modality in the workup of cardiac patients.

Double Aortic Arch Presenting with Neonatal Respiratory Distress But not Due to Airway Obstruction: Anatomic and Embryologic Explanation

Double aortic arch resulting from embryonic persistence of bilateral fourth branchial arches often leads to neonatal stridor due to tracheal compression. Two neonates presented with dyspnea without evidence of tracheal compression. An aneurysmal ductal aneurysm from the left-sided arch in the first neonate led to symptoms. Spontaneous duct closure and regression of ductal aneurysm led to relief of symptoms. Second neonate had severe neonatal pulmonary hypertension due to aortic origin of left pulmonary artery (LPA) that was disconnected from main PA. Surgical reanastomosis of the LPA and division of the smaller right arch corrected the anatomy. These two associations show that airway obstruction alone is not the cause of respiratory symptoms in neonates with double aortic arch.

Repair of Intramural Coronary Artery in Anomalous Aortic Origin of a Coronary Artery

It has become increasingly evident that anomalous aortic origin of a coronary artery is considered as an important anatomic anomaly in adolescents and adults. The question is whether or not surgical repair should be performed in asymptomatic patients. Previous reports showed that the numbers of asymptomatic patients who result in a sudden cardiac death are not negligible. Another reason to proceed to surgical intervention is the improvement in understanding this unique anatomy and the refinement of surgical techniques that brought the satisfied outcomes. Physiologic repair, that is, coronary artery bypass grafting was used to be the first choice of surgery; however, anatomic repair, such as coronary implantation and repair of intramural coronary artery is considered favorable in adolescents as "radical" oppose to "palliative" repair. We report our technique to relieve the intramural coronary artery based on the experience in arterial switch operation performed in neonates.

Surgical unroofing of intramural anomalous aortic origin of a coronary artery in pediatric patients: Single-center perspective

BackgroundIntramural anomalous aortic origin of a coronary artery (AAOCA) is associated with an increased risk of sudden cardiac death. This is amenable to surgical coronary unroofing, but outcomes studies are lacking. ObjectiveTo perform a comprehensive review of our institutional experience with pediatric patients with AAOCA who underwent surgical repair with unroofing of the intramural segment, focusing on preoperative and postoperative course and testing as well as intraoperative findings. MethodsA retrospective cohort study was conducted to evaluate patients with AAOCA status post–coronary unroofing at Children's Hospital of Wisconsin. Data extraction included symptoms, preoperative and postoperative imaging and testing, surgical findings, and postoperative clinical course. ResultsFrom January 1999 to December 12, 2015, 63 patients underwent unroofing at a median age of 13 years (0.5-18 years). The majority underwent unroofing of an intramural right coronary (79%); 21% had an intramural left AAOCA. Symptoms suggestive of possible ischemia were present in about 50%. Additional structural cardiac anomalies were present in 33%. Transthoracic echocardiography was diagnostic in 60 of 63 (95%) and correlated with surgical findings in all cases. There was no surgical mortality associated with the unroofing, and no additional coronary reinterventions were performed. The median duration of postoperative follow-up was 3.1 years (7 days to 13.6 years). Symptoms either persisted or developed in 46% postoperatively. Postoperative exercise stress testing, stress echocardiography, and cardiac magnetic resonance imaging were performed in 76%, 8%, and 20%, respectively, of the cohort. None identified findings consistent with reversible coronary ischemia. Three patients had sudden cardiac arrest (1 death) after surgery without an identified residual coronary abnormality. ConclusionsTransthoracic echocardiography, with carefully designed coronary imaging protocols, can be diagnostic in accurately identifying intramural AAOCA in pediatric patients. Unroofing can be performed safely with no early morbidity, but symptoms can persist (including rare life-threatening events) without evidence of ischemia by postoperative provocative testing.

Outcomes of surgical intervention for anomalous aortic origin of a coronary artery: A large contemporary prospective cohort study

ObjectiveThe purpose of this study was to prospectively analyze the outcomes of patients with anomalous aortic origin of a coronary artery undergoing surgical intervention according to a standardized management algorithm. MethodsAll patients aged 2 to 18 years undergoing surgical intervention for anomalous aortic origin of a coronary artery between December 2012 and April 2017 were prospectively included. Patients underwent stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and retrospectively electrocardiogram-gated computed tomography angiography preoperatively. Patients were cleared for exercise at 3 months postoperatively if asymptomatic and repeat stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and computed tomography angiography showed normal results. ResultsA total of 44 patients, with a median age of 14 years (8-18 years), underwent surgical intervention: 9 (20%) for the anomalous left coronary artery and 35 (80%) for the anomalous right coronary artery. Surgical procedures included unroofing in 35 patients (80%), translocation in 7 patients (16%), ostioplasty in 1 patient (2%), and side-side-anastomosis in 1 patient (2%). One patient who presented with aborted sudden cardiac death from an anomalous left coronary and underwent unroofing presented 1 year later with a recurrent episode and was found to have an unrecognized myocardial bridge and persistent compression of the coronary requiring reintervention. At last follow-up, 40 patients (91%) are asymptomatic and 4 patients have nonspecific chest pain; 42 patients (95%) have returned to full activity, and 2 patients are awaiting clearance. ConclusionsSurgical treatment for anomalous aortic origin of a coronary artery is safe and should aim to associate the coronary ostium with the correct sinus, away from the intercoronary pillar. After surgery, the majority of patients are cleared for exercise and remain asymptomatic. Longer follow-up is needed to assess the true efficacy of surgery in the prevention of sudden cardiac death.

Are the anomalous vertebral arteries more hypoplastic?: retrospective linear mixed model approach

BackgroundSmall or hypoplastic vertebral artery (VA) is one of the risk factor for posterior circulation stroke. We assess whether various types of VA anomaly contribute to its diameter.MethodsWe screened 238 patients who underwent neck CT and MR angiography within 1 month. A V1 anomaly was defined as the abnormal origin of the VA on a three-dimensional MR angiography and a V2 anomaly was defined as the VA not passing through the 6th cervical transverse foramen (TF) on an axial CT image. A linear mixed model was used to evaluate the determinants of VA size.ResultsAmong participants, 24 (10.1%) subjects exhibited an anomalous VA and, of the 476 VAs examined, 11 (2.3%) had an aortic origin and 27 (5.7%) had an abnormal entrance into the C6 TF. Presence of the V1 anomaly was positively associated with the V2 anomaly (P for chi-square < 0.001) and a linear mixed model revealed that being male (0.2 mm larger, P = 0.015), having a right VA anomaly (0.3 mm smaller, P < 0.001), having a V1 anomaly (0.9 mm smaller, P < 0.001), and having a V2 anomaly (0.7 mm smaller, P < 0.001) were significant predictor of VA diameter.ConclusionThe diameters of VAs with an anomalous aortic origin or an abnormal entrance of the TF were significantly smaller than those of normal VAs. These findings suggest that anomalies of the VA detected in 3-dimensional CTA or MRA may be clues for vertebral artery hypoplasia.

Cardiac stress MRI evaluation of anomalous aortic origin of a coronary artery.

Myocardial ischemia is an insult that is primarily thought of in an adult population. However, there are several congenital and acquired cardiac lesions that may lead to myocardial ischemia in a pediatric population. One of the prominent congenital lesions is anomalous aortic origin of a coronary artery (AAOCA). Anomalous aortic origin of a coronary artery is one of the leading causes sudden cardiac death in pediatric and young adult patients, and thus the assessment of myocardial perfusion is of the utmost importance. Over the past decade, pharmacologic stress MRI has proven to be a highly sensitive and accurate diagnostic examination for qualifying myocardial perfusion in adults with coronary artery disease. This noninvasive imaging modality may be a useful tool in assessing the function impact of AAOCA on myocardial perfusion.

Anatomic types of anomalous aortic origin of a coronary artery: A pictorial summary.

Anomalous aortic origin of a coronary artery is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. As commonly seen in congenital heart disease, a wide spectrum of anatomic variability is seen and hence, it is important for clinicians to document the precise anatomy and course of the anomalous vessel. This article describes coronary artery nomenclature using computerized tomography angiography and virtual angioscopy. These details are important for decision making, useful for surgical planning, and may have prognostic implications.

Current practices are variable in the evaluation and management of patients with anomalous aortic origin of a coronary artery: Results of a survey.

Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long-term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner. An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed. A total of 91 providers (46%) completed the survey including pediatric cardiology subspecialists (40%), general pediatric cardiologists (24%), cardiovascular (CV) surgeons (22%), adult cardiologists (10%), nurse practitioners (8%), cardiology fellows (3%) and CV anesthesiologist (1%). Forty-eight percent had been practicing for over 15 years and 28% were in their first 5 years of practice. Fifty-two percent of the providers cared for adults and 93% cared for children/adolescents. Eighty-eight percent were affiliated with an academic institution. All but one provider practiced in the USA, 62% practiced in Texas. Half of participants (50%) were very comfortable managing AAOCA patients and 36% were somewhat comfortable. Providers utilized various imaging tests to confirm the anatomy including computed tomography angiography 88%, cardiac magnetic resonance imaging 70%, cardiac catheterization 60%, echocardiogram 12%, IVUS 2% and myocardial perfusion scan 1%. The majority felt comfortable in counseling the families and felt that depending on the type of lesion these patients should get surgical referral (85%) vs clinical follow up (67%) with exercise restriction (65%). There is heterogeneity in the way AAOCA patients are currently evaluated and managed. A knowledge gap exists even with participants from academic institutions. Long-term data with a defined approach to management of these patients may help to improve outcomes and prevent unnecessary exercise restriction or surgery.

Anomalous Aortic Origin of a Coronary Artery: Symptoms Do Not Correlate With Intramural Length or Ostial Diameter.

Anomalous aortic origin of a coronary artery (AAOCA) is a known cause of sudden death. Our hypothesis was that longer intramural length and smaller ostial diameter correlate with preoperative symptoms. If true, this would assist in the decision for surgical indications. We also assessed the accuracy of preoperative imaging to predict intramural length. Retrospective analysis of patients who underwent AAOCA unroofing from 2006 to 2014. Patients had preoperative computed tomography angiography (CTA) or magnetic resonance imaging (MRI). Intramural length was measured. Intramural lengths and ostial diameters were also measured intraoperatively (operating room [OR]). Symptoms were noted. Intramural lengths and ostial diameters were compared between patients with and without preoperative symptoms. The accuracy of intramural length measured by CTA/MRI versus the length measured in the OR was assessed using a Bland-Altman analysis. Sixty-six patients underwent surgical repair of AAOCA. Fifty-two (79%) patients were symptomatic and 14 (21%) were asymptomatic. Mean age was 12.4 ± 4.0 years. There was no mortality. There was strong agreement between intramural length measured by CTA/MRI and measured in the OR. There was no significant difference in AAOCA intramural length in the symptomatic (8.6 ± 3.5 mm) and asymptomatic (8.9 ± 2.8 mm, P = .77) patients, which were measured both by CTA/MRI and intraoperatively (symptomatic 7.3 ± 2.5 mm, asymptomatic 6.9 ± 2.8 mm; P = .62). There was also no significant difference in AAOCA ostial diameters between groups (symptomatic = 1.9 ± 0.5 mm, asymptomatic = 1.6 ± 0.5 mm; P = .09). Preoperative CTA/MRI was very accurate in predicting the length of surgical unroofing. There was no demonstrable correlation between preoperative symptoms and intramural AAOCA length or AAOCA ostial diameter.

Introduction to anomalous aortic origin of a coronary artery.

Anomalous aortic origin of a coronary artery (AAOCA) occurs when both coronary arteries arise from the same aortic sinus from a single ostium or two separate ostia. While most coronary anomalies are benign, the two most common subtypes that predispose to sudden cardiac death in the young are interarterial anomalous right coronary artery and interarterial anomalous left coronary artery. Practitioners face many challenges with AAOCA. Diagnosing patients may be difficult because children and adolescents are often asymptomatic and first presentation may be sudden death or sudden cardiac arrest. Risk stratification is also challenging as determining which unique characteristics place the child at highest risk of sudden death has not been adequately delineated and ischemic testing may give false negative results. Last, there is significant variability in decision making regarding management of youth with AAOCA. Future research is needed to help determine the best way to identify at-risk children and which treatment is the safest and most efficacious.